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Pathology Test 3 THE RESPIRATORY SYSTEM Conditions caused by pulmonary disease • Hypoxia • Deficient oxygenation of arterial blood • May lead to hypoxia: Diminished availability of oxygen caused anywhere in the body • Signs and Symptoms of hypoxemia: • PaO2mmHg 80- 100: normal • 50-60: Malaisa, nausea, vertigo, restlessness, lightheadedness, impaired judgment, incoordination • 35-50: marked confusion, cardiac dysrhythmias, labored respiration • 25-35: Decreased renal blood flow, decreased urine output, loss of consciousness, poor oxygenation, poor CO, lactic acidosis, lethargy • <25: decreased minute ventilation secondary to depression of the respiratory center • Pulmonary Edema • Accumulation of fluid in the tissues and air spaces of the lung • Oxygen transport deficits in systemic disease • Pathologic conditions of every major organ system can have secondary effects on the pulmonary (cardiopulmonary) function and on oxygen transport • important for rehabilitation in that we stress oxygen transport system even more with activity • Pulmonary disease is often classified as: • Acute or chronic • Obstructive or restrictive • Infectious or noninfectious Most common signs/symptoms of pulmonary disease: • Cough • Persistent dry (non-productive) cough • Productive cough with nonpurulent sputum • Productive cough with purulent sputum • Hemoptysis • Dyspnea or shortness of breath • Usually indicates inadequate ventilation or insufficient amounts of oxygen in the circulating blood • Factors contributing to the sensation of dyspnea: • Work of breathing • Respiratory muscle fatigue • Decreased reserve • Emotion • Positional dyspnea: when person in lying down • Orthopnea- redistribution of body fluid • Chest pain (pulmonary pain patterns) • Can originate in the pleurae, airways, or the chest wall • Pulmonary pain patterns • Substernal or chest region over involved lung fields • Can radiate to the neck, upper trapezius, costal margins, thoracic back, scapulae, or shoulder • Shoulder pain may radiate along the medial aspect of the arm mimicking other neuromuscular causes of neck or shoulder pain • Abnormal sputum • Secretions may change in: amount (copious), viscosity (thickness), color (white, yellow, green, or pink), texture (frothy, clumped) • Cyanosis: bluish discoloration of the skin and muscous membraines • May be central or peripheral • Central: • Decreased oxygen saturation of hemoglobin in arterial blood • Best observed in buccal mucous membranes and lips • Peripheral: • With slow blood circulation in fingers and toes • Best observed in nail beds • Potential causes: • Decreased arterial oxygenation • Pulmonary or cardiac right-to-left shunts • Decreased cardiac output • Cold external temperature • Anxiety • Altered breathing patterns • Changes in rate, depth, regularity, effort of breathing • Occurs in response to any condition affecting the pulmonary system • Apneustic: gasping inspiration f/b short expiration • Cheyne-Stokes: repeated cycles of deep breathing f/b shallow breaths or cessation of breathing • Lateral costal breathing: Chest flattened anteriorly with flaring of lower ribs (supine), minimal upper chest expansion or accessory muscle involvement, and person breaths into the lateral plane due to diaphragm and intercostal muscle weakness • Stridor: shrill harsh sound of inspiration • Wheezing: breathing with rasp of whistle due to constriction or obstruction • Clubbing • Thickening or widening o f the terminal phalanges of the finger and toes - loss of the angle between the nail and the nail bed • Cause: • Any condition that interferes with oxygenation • 75% to 85% is due to pulmonary disease and resultant hypoxia • Clubbing does not always indicate lung disease • Assessment of clubbing: Schamrath method • Client places fingernails of opposite fingers together and holds them up to the light. If a diamond shape can be seen between the nails, there is no clubbing • Pulmonary edema: • Accumulation of fluid in the tissues and air spaces of the lung • Most commonly caused by heart disease, especially left ventricular failure • Also can be a complication of pulmonary disease and other systemic conditions. Effects of Aging: • Affects not only the physiologic functions of the lungs (ventilation and gas exchange) but also the ability of the respiratory system to defend itself • Movement of cilia slows • Loss of an effective cough reflex • An overall decrease in the functional ability of the lungs to move air in and out (especially after age 55 years) • Parenchyma • Changes in size and shape, becoming rounder • Loss of alveolar wall tissue and elastic tissue fibers • Reduced alveolar surface area and airways • Lung bases become less ventilated due to closing off of a number of airways Musculoskeletal Aging: • Respiratory muscle strength weakens • Increased stiffness of the chest wall Other aging changes: • Loss of elastic recoil of the lungs Result of aging • All of these changes contribute to the increased work of breathing • The elderly person must have more ventilation for the same oxygenation than the younger person • These changes are influenced by: environmental factors, respiratory disease, body size INFECTIOUS AND INFLAMMATORY DISEASES: PNEUMONIA AND PULMONARY TUBERCULOSIS Pneumonia: • Overview and Incidence • Inflammatory response to an organism or agent affecting the parenchyma of the lungs • May be primary or secondary, often follows influenza • One or both lungs at the level of the lobe (lobar pneumonia) • Distally at the bronchioles and alveoli (bronchopneumonia) • Etiology • Inhalation of toxic or caustic chemicals, smoke, dusts, or gases • Aspiration of food fluid, or vomitus • Bacterial: Prevalent in elderly and occurs during epidemics of viral respiratory infections • Viral: common, is not usually life-threatening except in the immuno-compromised client • Pathogenesis • Multiplying microorganisms release damaging toxins stimulating full-scale inflammatory and immune responses with damaging side effects • Endotoxins: Damage bronchial mucous and alveolo-capillary membranes • Inflammation and edema cause the acini and terminal bronchioles to fill with infectious debris and exudate • Types Of pneumonias • Aspiration is defined as the inspiration into the airways of foreign material • Virus destroys ciliated epithelia cells and invades goblet cells and bronchial mucous glands • Bacteria reaches alveolar surfaces and causes damage • Clinical Manifestations of pneumonias • Preceded by an upper respiratory infection • Signs and Symptoms: • Pleuritic chest pain aggravated by chest movement • Hacking, productive cough with rust-colored of green, purulent sputum • Dyspnea • Tachypnea accompanied by decreased chest excursion on the affected side • Cyanosis • Fatigue • Fever and chills and generalized myalgias • Treatment: • The primary treatment is antibiotic therapy for bacteria • Adequate hydration and pulmonary hygiene: deep breathing, coughing, and chest therapy (postural drainage) • Mechanical ventilation and supplemental oxygen • If severe positive end-expiratory pressure (PEE) is added to the ventilatory support measures • Preventive measures • Early ambulation, Proper positioning, Vaccination, monitoring • Immunization can provide protection for 3-5 years in 80% of vaccinated persons Pulmonary Tuberculosis: (Formerly known as “consumption”) • Infectious, inflammatory systemic disease that affects lungs and may disseminate to involve lymph nodes and other organs • Caused by mycobacterium tuberculosis • Characterized by granulomas, caseous (resembling cheese) necrosis, subsequent cavity formation • May be primary or secondary • • • • • • • Primary Tb: • Infection with T-Bacillus • Usually asymptomatic • Involves middle to lower lung area with lesions consisting of excudation in the lung parenchyma • Lesions quickly becomes caseuos and spread to the bronchopulmonary lymph nodes • Secondary TB: • Most common form of clinical TB • Develops as a result of either endogenous or exogenous reinfection by the tubercle bacillus • Occurs when the primary lesion becomes active as a result of debilitation or lowered resistance Multidrug-Resistant TB • An iatrogenic disease now emerging as a major infectious disease problem throughout the world • Some of the factors contributing to the increased incidence of MDRTB include • AIDS pandemic • Increased incidence of TB in populations without easy access to anti-TB medications • Deterioration of the public health infrastructure • Inadequate training of health-care providers in the epridemiology of TB Etiology • Transmitted by inhalation of infected airborne particles: droplet nuclei,or produced when the infected persons sneeze, laugh, speak, sign, or cough • Casual contact or brief exposure to a few bacilli typically will not result in transmission • Prolonged, frequent or intense contact of many months places you at high risk for transmission • Tubercle bacillius is capable of surviving for months in sputum that is not exposed to sunlight • Within the body it can lie dormant for decades and then become reactivated • Secondary TB infection (endogenous reinfection) can occur at any time the person’s resistance is lowered • Antigen concentrations at initial site of infection causes necrosis, fibrosis and calcification, and renders the disease inactive or latent • Residual lesions are often visible on chest radiograph • Sites remain potential lesions for reactivation years later Miliary Tuberculosis • Produced when tubercle bacilli spread to other parts of the body by way of the blood • Common in 50 years or older or very young children with unstable or under-developed immune systems • Erosions of blood vessels by the primary lesion • Causes a large number of bacilli to enter the circulatory system and lymphatics • May lodge in any organ (kidneys, bone growth plates, lymph nodes, and meninges) Clinical manifestations • Most symptoms associated with TB do not appear in the early, most curable stage of disease • May be delayed > a year after initial exposure • Long before symptoms appear, TB may be detected by a simple skin test • Symptoms suggestive of TB • Productive cough of >3 weeks duration and when accompanied by weight loss, fever, night sweats, fatigue, malaise, and anorexia Diagnosis • includes: History, PE, Tuberculin skin test, chest radiograph, and microscopic examination and culture of sputum • Bronchoscopy or biopsy may be indicated • Tuberculin skin test determines whether the body’s immune response has been activated • A positive reaction • Causes a swelling or hardness at the site of injection • Develops 3 to 10 weeks after the initial infection • Indicated the presence of a TB infection Treatment • All cases of active disease are treated and certain cases of inactive disease are treated prophylactically • Treatment may be initiated with only a positive skin test even if a chest film and sputum analysis show no evidence of disease (pharmaceuticals) • Preventing Transmission • Covering mouth and nose • Adequate room ventilation and preventing overcrowding such as in homeless shelters or prisons • Preventive drug therapy is under investigation • Vaccination with BCG, a freeze-dried preparation of a live, attenuated strain is used for children (countries with high prevalence OBSTRUCTIVE DISEASE: Chronic Obstructive Pulmonary Disease (COPD) • A number of disorders that affect the movement of air in and out of the lungs • Most clinically important: obstructive bronchitis, emphysema, obstructive bronchiolitis, chronic unremitting asthma (obstructive but typically not considered part of COPD) • These 3 disease share a obstructive component, can occur independently by can coexist • Incidence and RIsk • COPD is almost always caused by exposure to environmental irritants, especially smoking • Prevalence associated with age, more men • Medical Management • DX: Physical exam, PFT, x-ray, labs • Treatment: multifaceted approach • Smoking cessation, Pharmacologic, Pulmonary hygiene, Exercise, Avoid irritants, Psychological, Diet • Prognosis • For chronic bronchitis and emphysema is poor because they are chronic progressive and debilitating Chronic Obstructive Bronchitis • A condition with productive cough lasting for at least 3 months per year for 2 consecutive years • FEV1/FVC ratio < 75% combined with chronic cough • Characterized by: • Chronic inflammation, scarring of lining, thus obstructing to and from lungs and increase mucous production • Wheezing and cough, impaired ciliary function = risk of infection • Infection results in more mucous, inflammation and thickening • Chronic spiral continues • Reduction in alveolar ventilation, hypoxia and acidosis • Poor tissue oxygenation and abnormal ventilation perfusion ratios • Manifestations: persistent cough with expectoration, SOB, prolonged expiration and recurrent infection Emphysema • Pathologic accumulation of air in tissues of the lung and is found in most people with COPD • 3 types: • Centrilobar: Destruction of bronchioles, usually in upper lung, often alveolar sac intact • Panlobar: Destroys air spaces of entire acinus and most commonly in lower lung • Paraseptal: destroys alveoli in lower lobes of lungs resulting in isolated blebs along the lung periphery • Pathogenesis • Destruction of elastic protein in the lung causing destruction of airways • Loss of elasticity causes narrowing and collapse, loss of recoil, making breathing difficult especially with expiration (increased work) • Permanat overdistension of airspace and destruction of septal walls • Blebs - air pocket between alveolar space • Bullae - air pocket in parenchyma • Clinical manifestation • High rates of anxiety, Dyspnea, Often thin client, Tachypnea with prolonged expiration, Forward lean with arms braced on knees supporting shoulders and chest, Barrel chest, Increased expiratory effect Asthma • A reversible obstructive lung disease caused by increased reaction of the airway to various stimuli • Chronic inflammatory with acute exacerbations • Causative factors • Extrinsic (allergies) • Intrinsic (non allergies) • Can be adult onset, exercise induced, aspirin sensitive and occupational • Incidence and Risk • Air pollution, Increase of aging population, Airtight building • Occurs at any age but more likely in those less than 5 • Childhood > in boys than girls • Urban industrialized setting and in colder climates • Pathogenesis • Airways are site of inflammation: Epithelial disruption, edema, mucous plugging • Inflammatory mediators produce muscle spasm, vascular congestion, vascular permeability edema, thick mucous • Results: • Ventilation profusion mismatch • Hypoxemia • Obstructed expiratory flow • Increased work of breathing • Clinical Manifestation • Differ in presentation, degree and frequency among clients • Sensation of chest constriction, Cough, SOB, prolonged expiration, Wheeze (ina nd out), loud cheat sounds, Anxious, Fatigue • Rehabilitation Implications • Chest therapy: breathing exercises (pursed lips), postural drainage • Physical training to improve posture and strengthen respiratory muscles and efficiency of cough and LE training • Monitor vital signs • Use medications optimally PARENCHYMAL DISORDERS Atelectasis • Collapse of normally expanded and aerated lung tissue at any structural level • Most cases are categorized as either obstructive- absorptive or compressive • Always secondary to another lesion such as obstruction of bronchus, decreased motion, or loss of ability for pulmonary expansion • Causes • Primary cause is obstruction of the bronchus • Develops when there is interference with natural forces that promote lung expansion • Tumors, mucus, or foreign material obstructing bronchi • Failure to breath deeply post-operatively • Neuromuscular disease • Insufficient pulmonary surfactant, as occurs in respiratory distress syndrome • Inhalation of anethesia • High concentrations of oxygen • Lung contusion • Aspiration of gastric contents or smoke inhalation • • • • • • Increased elastic recoil as a result of interstitial fibrosis can also interfere with lung distention • The compressive type due to: • Air (pneumo-thorax) • Blood (hemothorax) • Fluid (hydrotherax) filling the pleural space Clinical Manifestations • When sudden obstruction of the bronchus occurs, there may be: • Dyspnea, tachypnea, Cyanosis, Elevation of temp, Drop in blood pressure, Substernal retrations, Shock • In chronic form, the client may be asymptomatic with gradual onset of dyspnea and weakness Medical Management • X-ray examination may show a shadow in the area of collapse • If an entire lobe is collapsed, the radiograph will show the trachea, heart, and mediastinum deviated toward the collapsed area, with the diaphragm elevated on that side • Blood gas measurements may show decreased oxygen saturation Treatment and Prognosis • Directed toward removing the cause by suctioning or bronchoscopy may be employed to remove airway obstruction • Re-expansion of the lung is often possible • Becomes increasingly resistant to re-inflation within a few hours • Chronic atelectasis may require surgical removal of the affected segment or lobe of lung • Antibiotics to combat infection accompanying secondary atelectasis Prevention • Frequent position changes, deep breathing, coughing • Early ambulation help promote drainage of all lung segments • Deep breathing and effective coughing enhance lung expansion and prevent airway obstruction Summary: Gas exchange is import to exercise dose and patient tolerance. Rehabilitation professionals identify patient signs and symptoms as they improve function. THE INTEGUMENTARY SYSTEM: • Two major components of skin: • Cutaneous membrane: epidermis, dermis • Accessory structures: hair, nails, multicellular exocrine glands • Subcutaneous layer: loose connective tissue (hypo dermis), and extensive interconnections with dermis • General Functions of the skin: Protection, excretion, maintenance of normal body temp, synthesis of Vit D3, storage, and detection • Epidermis (Stratified epithelium): outmost layer • Mechanical protection/ keeps organisms out • Keratinocytes: water-proof, helps keep organisms out • Basal cells: Stem cells that produce keratinized cells • Merkel cells: Sensory rolls • Langerhans cells: antigen presenting cells • Skin color: carotene (orange coloring from vit A) and melanin (darker skin tone) and dermal circulation (decrease in O2 on Hb can lead to a color change to blue) • Sun exposure converts steroid to vit D (important for absorption of calcium) • Dermis (middle layer of skin) • Capillaries, sensory neurons, accessory organs • Collagen: • skin turgor: Test to measure dehydration (decrease turgor when skin remains elevated when picked up) • wrinkles and stretch marks • Retin-A (form of vit A) • Lines of Cleavage • Lines of pull for normal movement • Parallel incisions with lines of cleavage leads to a decrease in scarring • Dermal blood supply: cutaneous and papillary plexus • Innervation of skin • Nn fivers control blood flow, secretion, monitor sensory receptors (merkels discs, meissner’s and pacinian corpuscles) • Skin Lesions • Causes: cancer, trauma, genetic changes • Primary (on skin) and secondary (have pathology that causes lesions) • First lesion to appear (vesicle), when changes occur called secondary (crust) • Birthmarks • Pigment cell (Cafe au lait) - coffee stain • Vascular (strawberry hemangioma - red • Epidermal (epidermal nevus) - wart • Connective tissue (collagenoma) • Signs and symptoms of Skin disease • Pruritus (itching) - could also be a reaction to a medicine or antigen (allergy) • Urticaria (hives) • Rash (eruption on the skin) - virus • Xeroderma (dry skin) • Unusual spots, moles, nodules, cysts - looking to see if new or changed • Edema or swelling • Other (lack of sweating, pain, increase sweating) • Aging and integument • Epidermis thining • Langerhans cells decrease about 50% (greater risk of invasion) • Vit D3 production declines by 75% (decrease in calcium absorption so bone can become weaker) • Melanocyte activity declines (More ar risk for skin exposure) • Glandular activity declines )risk of not being able to regulate temp) • Blood supply to dermis is reduced at the same time sweat glands become less active • Hair follicles stop functioning or produce fine hair • Dermis thins and elastic fiber network declines (fragile, more wrinkles) • Changes in secondary sex characteristics (more hair growth in unusual places) • Skin repairs proceeds slowly with recurring infections • Special Implications • Therapist should remain alert to any skin changed that may indicate the onset or progression of a systemic condition • Care must be taken when using electrical or thermal modalities (heat or cold) with elderly people • Decreased sensation can cause thermal burn • Environmental Factors that induce skin disease • Mechanical factors • Friction, Pressure, Vibration, Cuts • Physical factors • Heat, Cold, Humidity, Water, Sunlight, UV light, Ionizing radiation • Chemical agents • Primary irritant, Sensitizers, Photo irritants, Photosensitizers • Biologic agents • Insect and animal parasites, Bacteria, Rickettsiae, Fungi, Viruses, Irritant and sensitizing plants and woods THERMAL INJURIES (cold injuries) • Cold injuries • Result from over-exposure to cold air or water and occur in two major forms • Localized injuries (frostbite) • Systemic injuries (hypothermia) • Untreated or improperly treated frostbite can lead to gangrene and amputation requiring therapy and rehabilitation • Pathogenesis/ clinical manifestations • Localized cold injuries • Ice crystals form in tissues and expand extracellular spaces • With compression, cell membranes rupture, interrupting enzymatic and metabolic activities • Increased capillary permeability accompanies release of histamine, resulting in aggregation of red blood cells and microvascular occlusion • Types of frostbite • Superficial frostbite • Affects skin and subcutaneous tissue • Upon returning to a warm place, frostbite produces burning, tingling, numbness, swelling, and a mottled, blue-gray skin color • As re-warming occurs, the person will feel pain • Deep frostbite • Extends beyond subcutaneous tissue and usually affects the hands or feet • Skin becomes white until it has thawed and then turns purplish blue • Produces pain, blisters, tissue necrosis, and gangrene • Treatment • Localized injury • Warming the injured part without rubbing or massaging the area to avoid further tissue damage • Analgesics for pain • Proper positioning • Debridement of blisters may be required • Bulky dressings may be applied to permit drainage and provide protection • A bed cradle may be needed to keep the weight of bedcovers off the affected part(s) • If a compartment syndrome develops a fasciotomy may be performed to increase circulation by lowering edematous tissue pressure • If gangrene occurs, amputation may be necessary • Smoking causes vasoconstriction and slows healing: the client should be advised to quit smoking, at least during the recovery period • Prognosis • Depends on the extent of localized cold injury and development of any complications • Long term effects: increased sensitivity to cold, burning and tingling upon re-exposure to cold, and increased sweating of the affected area • Prevention: windproof, water-resistant, many-layered clothing, moisture-wicking socks, a head covering, mittens instead of gloves, and heat-generating devices in pockets or battery-operated socks • Implications • Rehab professional may see when complications such as necrosis and gangrene resulting in amputation • Whirlpool • Gentle agitation directed away from the affected are • Can be prescribed as part of re-warming procedure • Water temperature is based on tissue temperature and determined in conjunction with the medical staff • Massage may further tissue damage and is contraindicated until local tissue has healed BURNS • Burn injuries occur when energy from a heat source is transferred to the tissues of the body • The depth of injury: function of temp, source of energy, duration of exposure • Etiology: • Burns are categorized according to mechanism of injury • • • • • • Caused by exposure, tissue contact, ingestion, inhalation • Thermal burns: flames, hot liquids, steam, semisolids (tar), or hot objects • Chemical burns: strong acids, alkalis, or organic compounds • Electrical burns: exposed or faulty electrical wiring, high-voltage power lines, or lightening • Radiation burns: Exposure to a radio-active source Severity • Factors that influence injury severity: depth, age, size (TBSA, rule of nines, lund-Browder method), location, general health, mechanism of injury • Rule of nines: head 9%, upper limb 9% each, trunk 18%,genitalia 1%, lower limb 18% each • Burn depth can be divided into categories based on the elements (depth) of the skin that are damaged • Superficial: 1st degree (epidermis) • Partial thickness: 2nd degree (epidermis and dermis) • Full thickness: 3rd and 4th degree (epidermis, dermis, and subcutaneous tissue) • Burns that require medical intervention are a combination of partial and full thickness burns Partial thickness burns • Large thick-walled blisters covering extensive area • Edema • Appearance: mottled red base, broken epidermis, wet, shiny, and weepy surface • Painful, sensitive to cold air • Superficial P-T burns heals in 14-21 days • Deep P-T burn requires 21-28 days for healing • Healing rate varies Full thickness burns • Appearance: variable (deep red, black, white, or brown) • Edema, fat exposed, epidermal and dermal tissue destruction • Little pain or insensate • F-T dead skin suppurates and liquefies after 2-3 weeks • Spontaneous healing may be impossible but small areas may be left alone to form scarring without grafting • Requires removal of eschar and subsequent split or full thickness skin grafting • Hypertrophic scarring and wound contractures likely to develop without preventive measures Pathophysiologic changes • Depend on the extent or size of the burn • Smaller burns: Body’s response to injury is localized to the injured area • Extensive burns (25% > of the TBSA): Response is systemic, potentially affecting all major systems of the body • Systems more obviously affected: cardiovascular, renal, gastrointestinal, immune, respiratory systems Cardiovascular changes • Occurs immediately (Burn shock) • Vasoactive substances released with cell necrosis are released (catecholamines, histamine, serotonin, leukotrienes, prostaglandins) from injured tissue which increase capillary permeability • Extensive burns • Generalized body edema in both burned and non-burned tissue • Decreased intravascular blood volume • Heart rate increases • Secondary to catecholamine release and hypovolemia • Overall cardiac output falls • If intravascular space is not replenished, hypovolemic (burn) shock and death may result • Capillary permeability decreases and continues to return to normal (over weeks) • Cardiac output returns to normal and then increases about 24 hours after the injury due to hypermetabolic needs of the body • The body begins to reabsorb edema fluid and excretes the excess fluid over the ensuing days and weeks • Renal, GI, immune and respiratory • Initial shunting of blood from the kidneys and intestines leading to oliguria and intestinal dysfunction in clients with burns greater than 25% of TBSA • Immune system function is depressed, increasing the risk of infection and sepsis • Respiratory system leads to pulmonary artery hypertension and decreased lung compliance, even when there has been no inhalation injury • Smoke inhalation • Carbon monoxide: smoke poisoning from the inhalation of byproducts of combustion • Direct thermal burns to the pulmonary airways • Electrical and Chemical burns • Heat is generated as the electricity travels through the body resulting in internal tissue damage • Entrance and exit wounds may be significant • Cutaneous burn injuries • Electrical burns may be negligible, but soft tissue and muscle damage may be extensive (high-voltage) • It is possible for energy burns to ignite the person’s clothes causing thermal burns • Amount and type of damage • The voltage, type of current (direct or alternating), contact sire, and duration of contact are important factors • Alternating current > dangerous than direct • Other significant injuries may occur • Long-bone or vertebral compression fractures • SCI or TBI can occur if the victim falls on electrical contact • Chemical burns are associated with systemic toxicity from cutaneous absorption • Burning process continues until chemical removed • Contact time, concentration and mount • 24 to 74 hours for burn to fully develop • Clinical Manifestations • Burn location influences injury severity • Head, neck, and chest frequently have associated pulmonary complications • Face may have associated corneal abrasions • Hands and joints can result in permanent physical and vocational disability requiring extensive therapy and rehabilitation • Circumferential burns of extremities may produce a tourniquet-like effect and lead to total occlusion of circulation • Full-thickness burn • Nerve endings have been destroyed and no pain should be associated with this type of injury • Most full-thickness burns occur with superficial and partial-thickness burns in which nerve endings are intact and exposed • Excised eschar (dead tissue) and donor sites expose nerve fibers as well • As peripheral nerves regenerate, painful sensation returns • Clinical Course • 1. Emergent or Resuscitation phase • At the time of injury, concludes with the restoration of capillary permeability (48-72 hrs) • Resuscitation: Initiation of fluid measures, ends when capillary integrity returns and large fluid have decreased • 2. Acute phase • Person is hemo-dynamically stable, capillary permeability is restored, and diuresis has begun (>48- 72 hrs since injury) continues until wound closure is achieved • 3. Rehabilitation Phase • Overlaps, last beyond hospital stay, focus on independence and recovery • Treatment: • Minor burns: • Cleansing; removal of any damaging agents; debridement of loose, nonviable tissue, being careful not to break any blisters • Topical antimicrobial creams or ointment and a sterile dressing • Instructions for home care • Observation for clinical manifestations of infection • Active ROM exercises to maintain normal joint function, decrease edema formation, and decrease possible scar formation • Major burns • Lifesaving measures immediately after injury (ABC’s: airway, breathing, circulation) • Restorative care • Ex: infection control, wound care, skin grafts, pain management during the acute phase until wound closure is achieved • Maximize functional recovery and cosmetic outcome • Therapeutic interventions include • Positioning and immobilization following skin grafting to prevent unwanted movement and shearing of grafts • Scar and contracture prevention, wound care • Exercise, ambulation and ADL • Elasticized garments: reduce scar hyper- trophy and may be worn from months to 2 years after hospitalization • Bioengineered temporary skin grafts to minimize fluid and protein loss from the burn surface, prevent infection, and reduce pain • Autograft- self • Allografts- genetically identical • Xenografts- another species • Biosynthetic grafts- synthesized • Full-thickness burn: autograft may be required • Intact graft: over areas where appearance or joint movement is important • Meshed (fenestrated) to cover up the 3X its original size • Test tube skin graft • Developed for burns that cover the entire body surface • Epidermal cells are cultured into smooth sheets of skin and then grafted onto burns • Prognosis • Client’s age affects the severity and outcome of the burn • Obesity, alcoholism, and cardiac disorders affecting general health • Disorders that impair peripheral circulation • Peripheral vascular disease increase the complication and mortality rates for client with burns • Special implications: burns • Client education especially in elderly • Reviewing simple cooking precautions • Do not leave burners in use unattended • Do not use high heat • Do not wear clothing with loose sleeves or belts especially bathrobes when near flame • Use front burners whenever possible and avoid leaning over front burners when using back burners • Hot water higher temp should be lowered • Therapy goals • Encourage deep breathing and facilitate lung expansion • Promote wound healing • Reduce dependent edema formation and promote venous return • Prevent or minimize deformities and hypertrophic scarring • Increase ROM, strength, and function • Increase independence in daily activities and self-care • Precautions • Patients are at high risk of infection due to loss of skin barrier and impaired immune response • The amount of BSA exposed during wound care must be minimized to prevent hypothermia • Hydrotherapy treatment must be limited to 30 mins or less with water temp in the 98-102 degrees F range • Infection control techniques must be practiced carefully • Skin donor sites require the same care and precautions as other partial thickness wounds • Arrange therapy likely to elicit painful responses to coincide with medications • Combining relaxation techniques, music therapy, distraction with would care may be helpful • Burned areas must be maintained in positions of physiologic function within the limits imposed by associated injuries, grafting, and other therapeutic devices PRESSURE ULCERS (bed sores, decubitus ulcer) • Caused by unrelieved pressure resulting in damage to underlying tissue • Usually occur over bony prominences • Sacrum, heels, ischial tuberosities, and greater trochanter • Graded or staged to classify the degree of tissue damage observed • Other types of ulcers, such as arterial, venous, or diabetic ulcers, are staged using partial and full-thickness classifications • Etiology • Interface pressure, friction, shearing forces, maceration • Risk factors • Bed and chair bound clients • Patients with impaired ability to reposition themselves • Decreased mobility, incontinence, obesity, nutritional factors, chronic disease accompanied by anemia, edema, renal failure, or sepsis, altered level of consciousness, contractures, increased muscle tone, and loss of sensation • Stages of pressure ulcers • Stage 1: non blanchable erythema - the heralding lesion of skin ulceration • Stage 2: Partial thickness skin loss involving epidermis, dermis, or both • Ulceration is superficial and appears clinically as an abrasion, blister or shallow crater • Stage 3: Presents clinically as a deep crater with or without undermining of the adjacent tissue • Full thickness skin loss: damage or necrosis of subcutaneous tissue that may extend down to buy not through the underlying fascia • Stage 4: Full thickness skin loss • Extensive destruction, tissue necrosis, or damage to muscle bone or supporting structures (tendon or joint capsule) • Undermining and sinus tracts may also be associated • Pathogenesis • Pressure is the external factor causing ischemia and tissue necrosis • Continuous pressure of the soft tissues between bony prominences and hard or unyielding surfaces • Compresses capillaries and occludes blood flow • Reduces blood supply and lymphatic drainage to affected area • If the pressure is relieved, a brief period of rebound capillary dilation (called reactive hyperemia) occurs and no tissue damage develops • If pressure is not relieved • Endothelial cells lining the capillaries become disrupted by platelet aggregation • Form microthrombic, necrotic tissue • Predisposes to bacterial invasion and subsequent infection preventing granulation tissue • Clinical Manifestations • Superficial sores: common on the sacrum as result of shearing or friction forces (parallel) • • • • • • Deep sores: develop closer to the bone as a result of tissue distortion and vascular occlusion from pressure over heels, trochanters, and ischii • Deep lesions often go undetected until they penetrate the skin and significant subcutaneous damage has occurred • Wounds are generally red, brown, black, or yellow • Infection is usually localized and self-limiting • Proteolytic enzymes from bacteria and macrophages dissolve necrotic tissues and cause a foul-smelling discharge that appears like pus • Painful in individuals who have sensation • Presence of necrotic tissue produces inflammatory response with hyperemia, fever, adn increased WBC • If ulceration is large, toxicity and pain may lead to loss of appetite, debilitation, and renal insufficiency • Individuals who are immunosuppressed or who have diabetes mellitus may develop infection and inflammation of adjacent tissues (cellulitis) or septicemia Diagnosis • Determine the cause of loss of skin integrity • Wound culture and testing of the exudate • Identify infecting organism • Determines the oral antibiotics that may be needed • Topical antibiotics are typically not effective Medical treatment • Pressure ulcer is cleaned thoroughly • Spontaneous healing will occur more quickly when the ulcer is kept moist with an occlusion dressing • Many physicians continue to advocate the initial use of wet-to-dry dressing • Application of open wet dressing, allowing it to dry on ulcer, and mechanical debride • There is a risk of removing viable tissue, bleeding, thus procedure no longer has universal approval • Antiseptics (such as hydrogen peroxide or iodine) • Controversial as these can be damaging to marginal granulation tissue • Successful healing requires adequate relief of pressure and absence of infection • Presence of necrotic tissue • Optimal environment for bacteria to grow • Important to removing necrotic material ASAP • Therapeutic interventions • Hydro-therapy, electrical stimulation, US • Large deep pressure ulcers • Chemical debridement using proteolytic enzyme agents • Surgical debridement of necrotic tissue • Opening of deep pockets for drainage • Skin grafting techniques may be used if the wound is closed • In stage 3 ulcers, undamaged tissue near the wound may be rotated to cover • In stage 4 ulcer, musculoskeletal flaps as well as a variety of other skin grafting techniques may be used effectively to close the wound Prognosis • Most clients have multiple complicating medical factors contribute to the poor healing • Provided good conditions, the wound should heal successfully • The presence of some factors alters the prognosis negatively (infection, poor nutrition or blood supply, medical complications, incontinence) Rehabilitation implications • Therapists can make major contributions essential to the success of the intervention • Delivery of therapeutic modalities • Appropriate positioning, management of tissue load • Good mobility Risk assessment • • • • • • Evaluating both sensation and physiologic risk of pressure ulcers can be made using a validated risk assessment tool (Braden scale) • Acute care, home-health, or long-term health care setting should be evaluated for risk levels and reassessed every 3 months for changes in status • All clients at risk should have a systematic skin inspection at least daily, paying attention to bony prominences Preventing pressure in bed • Reposition at least every 2 hours if consistent with overall treatment goals • Positioning devices: pillows or foam wedges should be used to keep bony prominences from direct contact with one another • Prone, supine, and side-lying positions, avoid positioning directly on the greater trochanters, heels, and bony prominences Preventing pressure • Persons who are completely immobile should be provided with devices that completely relieve pressure on heels • Maintain the head of the bed at the lowest degree of elevation consistent with medical conditions and other restrictions • Limit the amount of time the head of the bed is elevated • Pressure-reducing surface, such as foam, static air, alternating air, gel, or water mattresses may be indicated High risk client • Frequent shifting of body weight prevents ischemia by redistributing the weight and allowing blood to recirculate • Static or dynamic pressure reducing devices are commercially available • Reducing pressure on the skin must be accompanied by adequate fluid and nutrition intake Preventing friction • Use lifting devices • A trapeze bar allows a patient to help lift and prevent shearing injury to the skin • A mechanical lift or slide board can be used to move (rather than drag) persons who cannot assist during transfers • Linen can be used to move persons in bed who cannot assist during position changes Preventing pressure sitting • Avoid uninterrupted sitting in any chair or wheelchair • Repositioned, shifting the points under pressure at least every hour, or return to bed if consistent with overall management goals • Persons who are able should be taught to shift every 15 mins • For a chair-bound person, the use of a pressure-reducing device is indicates • Do not use donut type devices PATHOPHYSIOLOGY OF THE MUSCULOSKELETAL SYSTEM GENETIC & DEVELOPMENT: Cerebral Palsy • Non-hereditary and non-progressive lesion on the cerebral cortex • Results in neuromuscular disorders of posture and voluntary movements • Associated difficulty in: Speech, vision, hearing, perceptual functions, seizures, hydrocephalus, microcephalus, intellectual disability • Classifications: • Muscle Tone: Hypotonia (low tone), hypertonia (high tone), mixture (fluxuations) • Muscle tone grading: mild, moderate, severe • Spastic (majority of cases): monoplegia, diplegia (lower limbs more affected), hemiplegia, quadriplegia • Ataxia • Dyskinesia (choreoathetiosis)- always moving • Incidence: • • • • • • • Causes are multifactorial: prenatal, perinatal, postnatal, result of cerebral anoxia, hemorrhage, cortex damage Pathogenesis: • Neuropathic lesions • Subependymal hemorrhage (below lining of ventricles- early) • Malformation of CNS • Hypoxia • Systemic degeneration of immature areas • Interferes with cell maturation • Brain edema: CO2 accumulation and lactic acid= acidosis • Severity of damage depends on gestational age Clinical Manifestations: • Muscle tone alterations • Abnormal postures (synergist) • Reflex abnormalities • Delayed motor development • Abnormal motor performance • Associated abnormalities: intellectual disability, learning disabilities, seizure disorders, sensory impairment, B&B, risk for UTI, orthopedic disabilities • Abnormal head circumferences: associated with hydrocephalus and microcephaly • Change occurs with growth and maturation • Musculoskeletal problems frequent • Joint restrictions • Muscle length tension relationship • Contracture • Decreased muscle mass, power, endurance Diagnosis: • Observation, history, neurologic exam • EEG, hip radiograph, CBC, UA, CT • Often child may not be diagnosed initially and it could take time to determine diagnosis Treatment: • Multidisciplinary: Family, PT, OT, ST, MD, RN, Special educators, psych • Pharmaceuticals: Baclofen (can be delivered by pump= decreases spasticity), diazepam, anti-seizure, carnitine • Neurosurgery: Motor point block, Botox (decreases spasticity by keeping the muscle from firing), Dorsal Rhizotomy (sever sensory nerves to decrease abnormalities) • Orthopedic surgery: Mm lengthening, Mm transfers, femoral de-rotational osteotomy, orthosis (joint stabilizers) Prognosis: • Mild CP: Resolution with maturity, mild impairment • Moderate CP: limited mobility • Severe CP: poor prognosis, needs full time care • Ambulation potential: Based on motor milestones • Sitting and scooting by 2 yrs (when usually done by 6 months), ambulation by 8 yrs or most likely will not occur Rehabilitation Implications: • Hypotonia: wide based sitting, head lag, decreased reaching and kicking • Postoperative care • Adaptive equipment • ROM • Orthoses Spina Bifida (Occulta, meningocele, myelomeningocele) • Describes common congenital defects of neural tube closure (NTD) • Normally, the spinal cord and cauda equina are encased in a protective sheath of bone and meninges • Failure of neural tube closure produces defects • Entire length or small area • Most often lumbosacral area but may be found in the sacral, thoracic, and cervical • Spina Bifida Occulta: • Incomplete fusion of the posterior vertebral arch • Bony defect with the spinal cord, meninges and spinal fluid intact • May have hairy patch or dimple as a sign of it • Meningocele: • A cerebrospinal fluid-filled sac protruding • Sac contains fluid and the meninges only • The spinal cord or cauda equina (depending on level of lesion) remains intact • Myelomeningocele: • Most severe form • Protrusion of the meninges and spinal cord • Spine is open and the protruding sac contains CSF, the meninges, and the spinal cord or cauda equina • Etiology: • Genetic predisposition • Essential nutrient deficiency: Folic acid, B vit, during periods of rapid growth, such as pregnancy and fetal development, the body’s requirement for this vit increases • Radiation, environmental influences, maternal alcohol intake, maternal treatment with anti-epileptic drugs, and viral causes continue to be investigated. • Pathogenesis • Normally, about 20 days after conception, the embryo develops a neural groove in the dorsal ectoderm which depend as the two edges fuse to form the neural tube • By about day 23 this tube is completely closed except for an opening at each end • When the posterior portion of the neural tube fails to close causes: • Cerebrospinal fluid, the meninges or the spinal cord, or both, to bulge out • Degree of dysfunction is related to the anatomic level of defect • Clinical manifestations • Motor dysfunction depends on the level of the lesion and sparing of sensory and motor innervation • Loss of motor function is unevenly distributed over the limbs and spine • Muscle imbalances • Scoliosis and musculoskeletal deformities • Associated characteristics: Spina Bifida Occulta • Does not protrude visibly • Is often accompanied by: • Depression or dimple in the skin • Tuft of dark hair • Soft fatty deposits (subcutaneous lipomas or dermoid cyst) • Port wine nevi • Combination of above • Associated characteristics: Menengocele • Saclike cyst protrudes outside the spine • Like spina bifida occulta, meningocele rarely causes neurologic deficits • Occassionally bowel and bladder problems or foot weakness may be present • Associated characteristics: Myelomeningocele • 0-2 years • Truncal hypotonia • Delayed autonomic reactions • • • • • 90% have normal intelligence (IQ >80) • Increased incidence of learning disabilities • 90% present with hydrocephalus • 10-30% risk of seizures • obesity • Late childhood and early adolescence • Kyphoscoliosis • 30% demonstrate decreased ambulatory status by age 12 • Increased risk for strabismus (deviation of the eye - cranial N 3,4,5) • Vasomotor insufficiency Motor and Functional Level • Thoracic defect • No LE movement • HKAFOs, RGO (reciprocal gait orthosis) • Wheelchair user, independent in transfer (may require slide board) • Therapeutic standing and ambulation as children • High lumbar defects (L1-L2) • Hip flexion and adduction may be spared • KAFOs • Independence in all transfers • Household and short community ambulator as children • Low Lumbar defect (L4-L5) • Hip flexion and knee extension • KAFOs • Household and short community ambulator as children • Sacral Defects • Foot and ankle weakness • Usually community ambulators (occasionally with some equipment) Myelomeningocele characteristics • Flaccid or spastic paralysis • Bowel and bladder incontinence • Sensory disturbances usually parallel motor dysfunction • Musculoskeletal deformities • Scoliosis, hip dysplasia, hip dislocation, clubfoot (talipes equinovarus) • Hip and knee flexion contractures • Hydrocephalus, and sometimes intellectual disability. Hydrocephalus • Arnold-chiari Malformation • Malformation with enlarged ventricles • Hindbrain is placed through the foramed magnum leading to increased CSF pressure and actual splitting of the tube during fetal development • Brainstem, 4th ventricle, and part of cerebellum are pulled downward through the foramen magnum • Leads to blockage of CSF flow • Pressure may develop on brainstem where the cranial nerves are located • A ventriculoperitoneal shunt (VP) provides drainage of CSF from the ventricles to an extra cranial compartment (usually either the heart or the peritoneal cavity) • Extra tubing is left in the extracranial site to uncoil as the child grows Bowel and Bladder problems • Present in children with myeomeningocele since B&B is controlled at the S2-4 level • Often occur with sacral lesions but they have normal leg movement • Urinary incontinence and infection can occur • Bladder small and spastic (holds little urine) • • • • • • • • • Large and hypotonic (decrease emptying and ureter reflux) • Management of B&B is critical to prevent infection, promote independence and functional tolieting • Complete bladder emptying using manual pressure on the bladder or intermittent catherization helps prevent UTI’s • High fluid intake is critical part of overall program • Stool incontinence is managed by a program to regulate bowel movements using timed enemas or suppositories Prenatal Diagnosis • Frequently, NTDs are detected prenatally by ultrasonic scanning and serum alpha-fetoprotein (AFP) test • Amniocentesis can detect only open NTDs and is recommended women who have previously had children with NTDs • Prenatal diagnosis makes it possible for planned cesarean section or therapeutic abortion Postnatal Diagnosis • Meningocele and myelomeningocele are obvious at exam • Trans-illumination of sac can usually distinguish • Spinal films detect defects • CT scan demonstrated presence of Hydrocephalus • Lab tests: urinalysis, urine cultures, and tests for renal function Surgical repair • Skin closure • Goal is to place the sac and its contents back in the body with good skin coverage • Most authorities advocate early closure (24-48 hrs) • Prevent local infection, avoid trauma to exposed tissues, and avoid stretching of other nerve roots • Delayed closure can increase the ability of infant to tolerate procedure • A variety of orthopedic surgical interventions may be required throughout the child’s growing yrs • Hip varus or derotation osteotomies for help dislocation, spinal fusion for scoliosis, and tendon transfers and muscle releases Prognosis • Early, aggressive care of NTDs had improved the overall prognosis associated with this condition • Varies with degree of accompanying neurologic deficit • Most deaths occur before age 4 • Survival to adulthood is approximately 85% Latex Rubber allergy • Children with spina bifida have been identified as having a greater risk of becoming allergic to latex • Symptoms • Watery eyes, wheezing, hives, rash, swelling, and in severe cases, anaphylaxis, a life-threatening reaction • Responses occur when items containing latex touch skin, mucous membranes or open areas • Avoid using toys, feeding utensils, or other items the infant or child may put its mouth that are made of latex Signs and symptoms of tethered cord syndrome • Scoliosis, increased spasticity, increased asymmetrical postures or movement, altered gait pattern, decreased upper extremity coordination, changes in muscle strength (at or below the lesion), back pain Bracing contraindications • Contraindications to HKAFO or RGO include spinal deformity, spasticity, decreased upper extremity strength, moderate obesity, plantar flexion contractures greater than 15-20 degrees, and hip flexion contracture greater than 35 degrees. Prevention • Folic acid can be found in multivitamins, fortified breakfast cereals, dark green leafy vegetables such as broccoli and spinach, egg yolks, and some fruits and fruit juices • Average American diet does not supply recommended level of folic acid • If all women of childbearing age were to consume 0.4mg of folic acid prior to becoming pregnant and during the first trimester, the incidence of folic acid preventable spina bifida, and anencephaly could be reduced by 75-85% CONGENITAL HIP DYSPLASIA Aka developmental dysplasia of the hip (DDH) Common hip disorder affecting children < 3yrs Can be unilateral or bilateral Occurs in 3 forms of varying severity: o Unstable hip dysplasia – hip is positioned normally but can be dislocated by manipulation o Subluxation or incomplete dislocation – femoral head remains in contact with the acetabulum but the head of the femur is partially displaced or uncovered o Complete dislocation – femoral head is totally outside the acetabulum Incidence and risk factors o 85% of affected infants are females o Risk increases in the presences of certain obstetric conditions Breech delivery, large neonates, twin or multiple births Idiopathic scoliosis, myelomeningocele, arthrogryposis, and CP o Other risk factors include Family history, first pregnancies, and oligohydramnios (low ambionic fluid) Etiology o Result of mechanical, physiologic, or environmental factors o Hormonally derived laxity of the ligaments o In utero positioning o Spasticity o Spinal instability o How babies are carried or positioned may affect formation of the acetabular cup and hip stability (double diapering – holds baby in abduction and ER) Pathogenesis o The femur, acetabulum, and hip joint capsule are usually well developed by approximately 10 weeks gestation o Most dislocations occur in the perinatal period, at which time there is maximal joint distention and elasticity o Breech position in utero – movement limited but this presentation places the hips in a position of acute flexion and adduction o If the dislocation is not diagnosed and treated early, secondary changes in both soft tissues and bony structures will occur o The longer the dislocation has been present, the great the secondary changes: Stretching of the hip capsule Contracture and shortening of the structures of the hip joint Changes in the blood supply to the hip Flattening of the femoral head Acetabular dysplasia Clinical Manifestations o Vary with age o In the newborn and nonambulatory up to 12 months, there may be one or more positive signs Physical asymmetries in ROM (even as little as 10 degrees is considered significant, especially limitation of hip abduction) Asymmetry in the buttock or gluteal fold (higher on the affected side) Extra thigh skin folds Leg length discrepancy requires medical evaluation In the ambulating child, uncorrected bilateral dysplasia may cause a characteristic gait pattern known as a “duck waddle” as the child sways from side to side Unilateral dysplasia is usually characterized by a limp with a positive Trendelenburg sign during the stance phase of gait on the involved side A flexion contracture on the involved side(s) develops as a results of posterior displacement of the hips, which then contributes to marked lumbar lordosis Diagnosis o In the newborn period, clinical examination is the most important diagnostic o Radiographic examination is unreliable until about 6 weeks of age and is used more commonly in older infants and children o Ultrasonography allows visualization of the cartilaginous structures of the hip and is especially accurate during the first 6 months of life Treatment o Treatment depends on the age of the child, and the severity and duration of the dysplasia o The most common treatment: Position the infant’s hips in a position of flexion and abduction until the joint capsule tightens and acetabulum is molded to assume a cup shape o This can be accomplished in some children simply by double diapering but in others requires the use of a hip harness such as the Pavlik harness o Treatment in older children who have been walking and for children with spasticity-induced dysplasia is usually surgical Skin or skeletal traction Closed reduction Open reduction Tenotomy of contracted muscles Or osteotomy depending on the clinical presentation Prognosis o If the dislocation is corrected in the first few days or weeks of life, the dysplasia is completely reversible and a normal hip will develop o As the child becomes older (prior to the developmental stage of walking) the deformity will worsen and become permanent o When untreated, long-term problems can include degenerative joint disease, hip pain, antalgic gait, scoliosis, back pain, or the need for total hip replacement Muscular Dystrophy The word dystrophy come from Latin and Greek roots meaning “faulty nutrition” When doctors first began describing muscle diseases in the 19th century, they had few tools other than their own eyes Muscles in may diseases appeared to be wasting away, and the doctors theorized that they somehow weren’t being properly nourished Muscular dystrophy – a group of genetic diseases marked by progressive weakness and degeneration of the skeletal or voluntary muscles, which control movement Most common group of progressive neuromuscular disorders of childhood, although signs of MD can occur at any point in the life span Genetic origin Characterized by ongoing symmetrical muscle wasting without neural or sensory deficits but with increasing deformity and disability Initially and paradoxically – the wasted muscles tend to hypertrophy because of connective tissue and fat deposits, giving the visual appearance of muscle strength Types of Muscular Dystrophy o Duchenne’s (pseudohypertrophic) muscular dystrophy (DMD, 50% of all cases o Becker’s (benign pseudohypertrophic) muscular dystrophy (BMD) o Facioscapulohumeral (Landouzy-Dejerine) dystrophy (FSH) o Limb-girdle dystrophy Incidence and Etiology o The overall incidence of MD is approximately 20 to 30 per 100,000 live births DMD occurs in approximately 1 in 3,500 live male births BMD occurs in approximately 1-3 in 100,000 live male births All Dystrophies Are Inherited o DMD and BMD are X-linked recessive caused by mutations in the dystrophin gene (muscle protein) Males are affected clinically and females are the carrier o FSH is an autosomal dominant disorder with onset in the early adolescence The son or daughter of a person affected with FSH is at 50% risk of inheriting the defective gene o LGD is usually an autosomal recessive disorder of late childhood or adolescence Parents will not exhibit disorder, a 1-in-4 chance of every pregnancy producing a child with the disorder FSH and LGD affect both sexes equally Clinical Manifestations o Muscular weakness, wasting, and hypotonia o Degree of severity and age of onset vary with type Duchenne’s o Usually identified when child has difficulty getting up off the floor Falls frequently, has difficulty climbing stairs Starts to walk with a waddling gait (proximal muscle weakness) and increased lumbar lordosis (compensation for hip extensor weakness) o Child walks on the toes because of weakness and contractures posterior tibial and peroneal muscles o Hip abductor weakness produces a positive Trendelenburg’s sign which will eventually change to a compensated gluteus medius gait as hip abductor weakness progresses o Gower Sign – using environment to assist with getting up off floor o Ambulation continues to deteriorate up to the ages of 10 to 12 years at which time the majority of persons with DMD are no longer able to walk o Within 3 to 5 years, muscles of e shoulder girdle become involved Excessive scapular winging and muscle hypertrophy (especially the upper arms, calved, thighs) Shoulder girdle weakness often prevents the use of crutches to support the body weight Weakness causes difficulty in performing overhead activities related to hygiene and work o Later, other complications develop such as altered mentation, depressed deep tendon reflexes, contractures, kyphoscoliosis, respiratory involvement, and chronic heart failure o These changes occur as a result of the loss of the protein dystrophin normally present in skeletal muscle as well as cardiac muscle and brain tissue Becker’s o S & S resembles Duchenne’s with a later onset and a milder course o Ambulation is preserved into mid-adolescence or later Marked by the tiptoe pattern with bilateral calf muscle hypertrophy o Proximal muscles are affected more Primary involvement in neck, trunk, pelvis, and shoulder girdle o Muscle cramps are a common complaint in late childhood and early adolescence o Scoliosis and contractures (elbow flexors, forearm pronators, and wrist flexors) are common when the child becomes wheelchair dependent Facioscapulohumeral o Mild form beginning with weakness and atrophy of the facial muscles and shoulder girdle Inability to close the eyes may the earliest sign Face is expressionless even when laughing or crying Diffuse facial flattening, a pouting lower lip Inability to pucker to whistle or suckle o Forward shoulders and scapular winging develop Difficulty raising arms overhead o Weakness of the LE is delayed for many years o Contractures, skeletal deformities, and muscular hypertrophy are uncommon Limb-girdle Dystrophy o Slow course with mild impairment o Early symptoms develop as a result of muscle weakness in upper arm (biceps and deltoid) and pelvic muscles o Noticed in the late adolescence or early adulthood but as late as the person’s 40s o Lack of consistent clinical features make this type more difficult to diagnose o Winging of scapulae, lumbar lordosis, abdominal protrusion, waddling gait, poor balance, and inability to raise the arms o Transitional movements are difficult Diagnosis o Based on clinical presentation, family history, and diagnostic testing Electromyography (EMG) Short, weak bursts of electrical activity in affected muscles Serum enzymes Creatine kinase is very high Muscle biopsy Shows variation in the size of muscle fibers and in later stages shows fat and connective tissue deposits Testing o Newer molecular techniques, including linkage studies, are now able to accurately diagnose DMD and BMD, as well as predict carrier status o Chorion biopsy is a prenatal diagnostic technique in which DNA is removed as early as 10 weeks gestation to determine the presence or absences of the defective gene o Currently, laboratory testing can detect a deletion of the dystrophin gene in approximately 70% of fetuses with DMD Pathogenesis o The affected gene encodes mRNA for a protein called dystrophin (helps muscles keep their shape and length) o Dystrophin links the skeletal and cardian muscle sarcolemma to the actin (contractile muscle protein) o Lack of dystrophin makes the sarcolemma susceptible to damage during contraction an relaxation cycles; thus muscle fiber necrosis is initiated by muscle contraction Especially during eccentric contraction o Males with low levels of dystrophin have the Duchenne-type MD o Males with nearly normal level but dystrophin of an abnormal size have the Becker-type MD o The absence or altered state of dystrophin causes Slowing of muscle protein synthesis Failure of muscle growth maintenance Results in higher loads of calcium entering the muscle cell Muscle cells are destroyed and replaced by fatty and connective tissues, often producing contractures Treatment o At present there is no known treatment to halt the progression of MD Corticosteriods may help improve muscle strength but long term use has negative effects o Treatment is directed toward maintaining function in unaffected muscle groups for as long as possible Utilizing supportive measures such as PT, OT, orthopedic appliances, surgery, and pharmaceuticals o Children who remain active as long as possible avoid complications (e.g. contractures, pressure ulcers, infections) and deconditioning that are common once they are wheelchair bound Prognosis o All forms of MD are progressive, but the prognosis varies with the type of MD present o As a general rule, the earlier the clinical signs appear, the more rapid, progressive, and disabling the dystrophy o Duchenne’s patients typically die in their 20s Precautions o When persons become ill or injured and are on bed rest (at home or in hospital) even for a few days they may lose many of their functional abilities o Although activity helps the client maintain functional abilities, strenuous exercise may facilitate the breakdown of muscle fibers so that exercise must be approached cautiously Low-repetition maximum weightlifting or strengthening is contraindicated o Respiratory involvement Requires careful monitoring of breathing techniques, respiratory movements, and oxygen saturation levels Deep-breathing and diaphragmatic breathing exercises and encouraged to cough Inspiratory muscles are similar to other skeletal muscle groups in that they can be trained for both force and endurance in this population Essential to maintain chest mobility Introduction to the Central Nervous System Sensory Disturbances o Dorsal column (DCML) Synapse at the level of the brainstem nuclei where they cross over to the contralateral (opposite) hemisphere of the brain Fine touch, vibration, pressure, proprioception o Anterolateral Spinothalamic tract Anterior – crude touch Lateral – pain and temperature o Disorders of: Afferent nerve Dorsal columns of the spinal cord Brainstem o Result in changes in the sensory input available Lack of tactile or cutaneous sensation Numbness, tingling, paresthesias, pain o Lesions of the midbrain areas that modulate and interpret sensory input Can cause exaggeration of sensory stimuli Somatosensation o Aka proprioception: sensory input from joints and muscles o When lost or disturbed Difficulty maintaining the body in the appropriate position for voluntary and involuntary movements Movement Disorders o Motor control is accomplished by the cooperative effort of many brain structures o A hierarchical organization Plans and strategies for movement Established by the higher centers (cortex) Execution of the task, making the modifications due to environmental changes Lower centers (brainstem and spinal cord) Cranial Nerve Involvement o Peripheral nerves relating to sensation and motor control of the head and neck o Often reflect both sensory and motor impairments because the nuclei for the nerves lie within the brainstem and midbrain Cerebellar Disorders o Cerebellum is involved in the coordination of all skeletal movement, and disorders affecting the cerebellum result in uncoordinated movement Adjust postural muscles and programs and tunes ongoing movements Cerebellar Ataxia o Incoordination of movement, a cardinal sign of cerebellar lesions o Dysmetria the under estimation or overestimation of a necessary movement toward a target o Dysdiadochokinesis Inability to perform rapidly alternating movement The movement is slow, without rhythm or consistency Cerebellar Dysfunction cont o Scanning speech Word selection is not affected, but the words are pronounced slowly, without melody, tone, or rhythm Incoordination or hypotonicity of the muscles of the larynx in controlling the voice o Eye Movements Disrupted, in both a static head and eye position and with movement of the head Gaze evoked nystagmus (inability to hold gaze on an object) o Decomposition of Movement Instead of performing a movement in one smooth motion, the person will move in distinct sequences to accomplish the motion o Gait disturbances The gait becomes wide-based and staggering Proprioception may give inaccurate cues because the cerebellar relays become disrupted Alterations of Consciousness and Disorders of Arousal (alterness) o Range from coma to confusion o Hyper-arousal states Restlessness, agitation, or delirium o Hypo-arousal Stupor – state of unresponsiveness, requires vigorous stimulation to arouse Coma – state of unresponsiveness; unable to arouse Consciousness and Arousal o Reticular activating system o Complex interaction between the brainstem and cerebral cortex Attention Deficits o Acute confusional state o Frontal and prefrontal areas of the brain Mental control, concentration, vigilance, and performance of meaningful activity Temporal relationships (what happened 1st?) Damage to these connections result in removal of frustrations, tensions, and anxieties Often unconcerned with tact, decorum Higher brain function disturbances o Behavioral disturbances that mimic disturbances of mental friction in psych disorders o Delusions Fixed false beliefs, paranoid delusions Associated with the limbic system o Hallucinations Sensory experiences without external stimulation Visual hallucinations generally suggest neurologic involvement Auditory hallucination imply psychiatric disease Memory (hippocampus) o Working memory Ability to hold information in short-term storage while permitting other cognitive operations to take place Amnesia – disorders of recent memory Confabulation – fabrication of information in response to questioning o Declarative memory Retention of experience of the memory of what has occurred (recalled facts and knowledge) o Procedural memory Describes the learning of skills and habits, or how something is done (unconscious memories like riding a bike) Language o Higher function of the brain that is affected in many disorders of the CNS Expressive aphasia – deficit in speech production or language output, accompanied by a deficit in communication in which speech comes out as garbled or inappropriate words Receptive aphasia – inability to understand language Speech o Mechanical act of uttering words using the neuromuscular structures responsible for articulation o Dysarthria – a disturbance in articulation o Anarthria – lack of ability to produce speech Alexia o Acquired inability to read Agraphia o Inability to write Apraxia o Acquire disorder of skilled purposeful movement that is not a result of paresis, akinesia, ataxia, sensory loss, or comprehension o Ideomotor apraxia is the most common and represents the inability to carry out a motor act on verbal command Agnosia o Inability to recognize an object o The previously acquired meaning is no longer attached to it Lobar disorders o Lesions of the hemispheres or lobes may cause the loss of the functions that each hemisphere controls Right hemisphere syndrome o Analyzes sensory information o Relates body to sensory environment (touch, facial recognition, 3-D) o Responsible for spatial relationships Inability to orient the body within external space and generate the appropriate motor responses Left neglect – loss of spatial awareness of the left side The ability to draw in two and three dimensions is lost Perspective and accurate copying Left hemisphere o Contains the general interpretive and speech centers and is responsible for language based skills Essential role in your personality by integrating sensory information and coordinating access to complex visual and auditory memories Loss of the ability to interpret what is seen or heard Aphasia – loss of speech Temporal and limbic lobe syndromes o Temporal Auditory sensation and perception Visual perception Language comprehension o Limbic Involves the primary emotions, those associated with pain, pleasure, anger, and fear o Limbic system (lobe) The hippocampus, amygdala, and cingulate gyrus Establishes emotional states and related drives Memory and storage Hippocampus plays a major role in memory The frontal lobe o Responsible for the highest levels of cognitive processing, control of emotion, and behavior Change from the premorbid personality Character and temperament are changed Slow processing of information Lack of judgment Disinhibition and apathy May lack insight into the deficits Physiologic basis for the recovery of function o Inherent in the recovery of function is the process of motor relearning Defined as the process of acquisition or modification of movement o Studies suggest that motor relearning and recovery of function are accomplished through the same neural mechanisms o Neural modifiability Change in the organization of connections among neurons and is often referred to as plasticity Mechanisms for recovery o Neuronal shock (Diaschisis) Injury to a nerve, and disruption of the neural pathway that extends a distance from the site of injury When the neurons distal to the injury regain function partial function may return o Injury may be secondary to either swelling of the axon or edema in the surrounding tissue May block synaptic activity in the injured neurons as well as that in the surrounding area With reduction of the edema, function may return o Denervation supersensititvity Loss of presynaptic function in one area, the postsynaptic target cells for that area may become more sensitive to neurotransmitters produced in lower concentrations o Regenerative synaptogenesis Injured axons begin sprouting o Collateral sprouting Neighboring axons sprouting to connect with sites that were previously innervated by the injured axon Degenerative Diseases of the Central Nervous System Degenerative disease o Can affect gray matter, white matter, or both o Involvement Diffuse but usually affects one area or system more than another o Etiology Most cases the causes are questionable or unknown o Diagnosis Observing the progression of the neurologic changes Identifying a general pattern consistent with the known disease process o Most common factor slow deterioration of bodily functions controlled by the brain and spinal cord Degenerations changes in o Gray matter – predominant disorder is dementia o Basal ganaglia – involuntary movements o Cerebellum and brainstem - disruption of smooth coordination of muscles o White matter Increasing the excitability of the muscles = spasticity Decreasing the neuronal input = weakness of the muscles Sensory changes Medical Management o Broad base of practitioners o Prognosis Steady decline in function because there is no cure for many of them o Prevention of secondary complications o Therapeutic intervention is intermittent depending on the client’s medical or functional status Amayotrophic Lateral Sclerosis (ALS aka Lou Gehrig’s Disease) ALS overview and definition Most common form of adult-onset progressive motor neuron disease affecting not only the anterior horn cells but also corticospinal tracts Progressive with unknown etiology Degeneration and scarring of the motor neurons in the lateral aspect of the spinal cord, brainstem, and cerebral cortex (hence lateral and sclerosis) Peripheral nerve changes result in muscle fiber atrophy or amyotrophy Resulting weakness cause profound limitation of movement Pathogenesis Affects the UMN of the cerebral cortex Corticospinal and corticobulbar tracts and interneurons Can also directly affect LMN Anterior horn cells in spinal cord and brainstem Affected cells shrink with excessive accumulation of the pigmented lipid (lipofuscin) which normally does not develop until advanced age Affects the giant pyramidal cells of the cortex and premotor area, sensory cortex, and temporal cortex Critical neurons are sparse, dendrites shortened, fragmented and disorganized Demyelination occurs Leads to denervation and consequent atrophy of the corresponding muscle fibers Incidence, etiology, and risk 1 per 100,000 Typically leads to mortality within 5 years Three classifications of ALS have been described: Sporadic – the most common form of ALS in the United States – 90 to 95% of all cases Familial – occurring more than once in a family lineage (genetic dominant inheritance) accounts for a very small number of cases in the United States – 5 to 10% of all cases Guamanian – an extremely high incidence of ALS was observed in Guam and the Trust Territories of the Pacific in the 1950s Clinical manifestations Variable Depending on UMN or LMN are those predominately involved LMN cell death and early denervation Asymmetrical weakness, usually of the distal aspect of one limb Cramping with volitional movement Progressive wasting and atrophy of muscles Fasciculations or spontaneous twitching of muscle fibers Extensor muscles become weaker then flexor muscles, especially in the hands Babinski’s and Hoffmann’s signs are present or the tendon jerks are disproportionately active Four categories of symptoms 1. Pseudobulbar palsy Damage in cranial nerves (bulbar) Dysarthria and exaggeration of the expression of emotion 2. Progressive bulbar palsy Cranial nerve nuclei involvement Weakness of muscles involved with swallowing, chewing, and facial gestures Fasciculations of the tongue are usually prominent 3. Primary lateral sclerosis Results from neuronal loss in the cortex Hyperactivity of tendon reflexes with spasticity causing difficulty with active movement Weakness and spasticity of specific muscles No muscle atrophy and fasciculations are not present This form is rare 4. In progressive spinal muscular atrophy Progressive loss of motor neurons in anterior horns of the spinal cord Progressive weakness, wasting, and fasciculation involving the small muscles of the hands Spinal cord can be the site of the initial disease process with symptoms reflecting the level involved Diagnosis Diagnosis is made by the clinical presentation and EMG EMG criteria for the diagnosis Fibrillations, fasciculation, and motor unit potential changes in multiple nerve root distribution in at least three limbs and the paraspinal muscles Treatment There is no known method of arresting the course of ALS, so therapy is symptomatic therapy There intellect of the client is not affected Health care providers should emphasize the value of maintaining the highest level of function throughout the course of the disease Education and supporting the client and family for the rapid decline in degenerative disease processes Alzheimer’s Disease (AD) Introduction o AD is a chronic, progressive, degenerative condition Dementia and cognitive problems including decline in memory, language, visuospatial skills, and personality changes o Specific cause is unknown Genetic predisposition, the abnormal processing of a normal cellular substance, amyloid, and advanced age Genetic, viral, and metabolic sources continue to be explored o 5.4 million Americans currently have AD which is expected to more than triple by the middle of the century Presentation o The main areas of the brain affected include the cerebral cortex, hippocampus, and amygdala Atrophy Progressive accumulation of insoluble fibrous material known as amyloid beta peptides (tau protein) Neurofibrillary tangles Senile plaques o Although a small number of plaques and tangles are indeed a normal consequence of the aging process, their numbers dramatically increase in persons with AD Clinical manifestations o Early symptoms Psychological changes – mood changes and sometimes depression Subtle personality changes – indifference, egocentricity, impulsivity, and irritability Often the first symptom is loss of the ability to learn new information Disorders of higher function are due to cortical lobe dysfunction o In the early stages of AD, losses of learning and memory are most apparent. Both storage (new learning) and retrieval (memory) are impaired. Clues do not help the person with AD to remember AD causes loss of older memories and recall of events from early life disappears o Language declines in a characteristic progression Word-finding difficulty is first Inability to remember names (anomia) Finally diminished comprehension o Eventually all learning is lost o Other dysfunctions that occur Apraxia Alexia – can’t read Agraphia – can’t write Changes in perception o Visuospatial deficits are an early clinical finding o The loss of ability to solve mathematical problems and handle money is typical in the early stages o Judgment is impaired and safety in driving is diminished o Areas close to the limbic systems also seem to be affected o Broca’s area – the motor speech area, and Wernicke’s area are frequently effected causing either expressive or receptive aphasia (or both) o A person with AD may lose smell and taste sensitivity leading to a decrease in appetite o As more brain cells die, the symptoms of AD become progressively worse. The progressive stage of AD is marked by a loss of function Musculoskeletal impairments such as ataxia, or the shuffling of the feet cause by a lack of muscular coordination, appear o Other systems such as the genitourinary system resulting in incontinence o As AD progresses, delusions, agitation, and even violence may occur o Disorders of sleep, eating, and sexual behavior are common o In the terminal stages of AD, the afflicted person may sleep a great deal and stare vacantly into space when awake o May be impossible to feed o Inevitably mortality often results due to dehydration, infection, or pneumonia or other similar causes Friedreich’s Ataxia Aka: FA, spinocerebellar degeneration The most common of the early-onset ataxias Characterized by degeneration of the ascending and descending fibers of the spinal cord, including the spinocerebellar tract Pathogenesis o Principle changes are cell loss in dorsal root ganglia and loss of large myelinated axons in peripheral nerves o Degeneration of the corticospinal tracts is evident in many cases, with sparing of the corticobulbar tracts o The cerebellum can be affected to varying degrees, with loss of Purkinje cells in the most advanced cases o Cardiomyopathic changes resulting in muscle fiber loss and fibrosis in approximately 60% of persons Clinical manifestations o Disorder of movement with ataxia gait the most common presenting symptom o The person begins to stagger and lurch; often a wide, based gait results o Early in the course of the disease, particularly in children, there may be motor restlessness that may look like chorea Clumsiness, ataxia, and tremor appear in the limbs o Muscle tone is normal at rest, although flexor spasm is common o Unusual combination/total absence of tendon reflexes with increased extensor activity o Weakness of the limbs follows o Dysarthria and scanning speech o Nystagmus is seen in about 20% of clients o Loss of vibration and position sense in the extremities o Pain, temperature, and light touch may be diminished in a distal and symmetrical distribution o Musculoskeletal deformities are a secondary complication of FA Scoliosis is common Clawing of the toes, appears in approximately 50% of clients o Mentation is usually preserved o Diabetes mellitus occurs in 10% of clients, and further 20% have impaired glucose tolerance Huntington’s Disease Progressive and hereditary disorder o Abnormalities of movement, personality disturbances, and dementia o Most often associated with choreic movement = brief, purposeless, involuntary, and random Pathogenesis o Changes occur in the cortex and cerebellum Both loss of neurons and production of glial cells which inhibit neural transmission o Selective vulnerability of neurons in a particular region with preservation of others o The amount of neurotransmitters, including GABA, acetylcholine are reduced Leaves relatively higher concentrations of other neurotransmitters o The normal balance of inhibition and excitation responses in the basal ganglia and thalamus that allows for smooth controlled movement is disrupted This may explain the excessive abnormal involuntary movements described and chorea o In the later stages Loss of the direct inhibitory substance Resultant rigidity and bradykinesia Clinical manifestations o Movement disorders (early stages) No neurologic abnormality on routine examination other than minor choreic movements Movements may be integrated into a purposeful movement such as raising the hand to the head as if to smooth the hair Involuntary movements may appear to be no more than an exaggeration of normal restlessness Chorea is increased by mental concentration, emotional stimuli, performance of complex motor tasks, and walking o Muscle strength Normal in early cases but may be affected by any significant bradykinesia or general motor disturbance o Tone Normal initially, but rigidity will become part of the clinical picture in many cases as the disease progresses o Tendon reflexes Normal. The “hung-up” knee jerk sometimes seen is produced by interposition of a choreiform contraction of knee extensors upon the normal course of the reflex o Dysarthria o Some persons become mute at a stage before motor disability is severe o Abnormalities of swallowing, or dysphagia, can cause choking and asphyxia o Cachexia Wasting of muscle with weight loss, is found despite an adequate diet o Sleep disorders become a progressive problem o Urinary incontinence Chorea o Term is derived from the Greek work for dance, and gait abnormalities are common o When chorea is predominant sign, persons walk with a wide-based staggering gait o Those persons with bradykinesia and hypertonicity may walk with a slow, stiff, unsteady gait Abnormalities in eye movement o Saccades (simultaneous movements of both eyes in same direction) The ability to move the eyes rapidly from one target to another is disturbed o Gaze fixation abnormalities Inability to fix on a light source without the intrusion of small saccadic movement o Smooth pursuit (following moving object) Is interrupted by the same small jerky saccadic movement Summary o Disorders of the central nervous system often reflect the area of involvement and the manifestations are indicative of the brain location o It is important to know what part of the CNS is responsible for specific functions in order to understand the lesion location Acute Stroke/TBI/SCI A sudden loss of neurologic function due to impaired blood flow to the brain, nonconvulsive and symptoms last greater than 24 hours Transient ischemic attack – TIA – a focal nonconvulsive episode of neurologic dysfunction that lasts less than 24 hours Who does it strike? o 3:2 males to females o African Americans ˃ Caucasians o Age 75-84 o Increase in prevalence with each decade o Unmodifiable risk factors Advanced age, male gender, race, heredity o Modifiable risk factors Smoking, HTN, diabetes, alcohol, obesity, abnormal lipid serum, A-fib, cardiac disease, hematologic disorders Types of stroke o Ischemic Lack of blood supply to an area of the brain without bleeding Thrombolytic Stuttering or progressive course Embolytic Abrupt onset (cardiogenic), max deficit at onset Lacunar (occlusion of single deep artery) Rapid, TIA o Hemorrhagic Bleeding into the brain parenchyma resulting from rupture of a cerebral artery Subarachnoid Intercerebral Traumatic Spontaneous Other causes Hypercoagulative states Vasculitis Arterial dissection Drugs Infections Pathophysiology of ischemic stroke o Neuronal injury results from a complex series of metabolic events o Duration, severity and location determine extent of infarct and thus the severity o Impaired energy metabolism, accumulation of Ca+ ions, acidosis and free radicals o Ischemic penumbra, area of misery perfusion may be salvageable Neurons that are functionally silent but structurally intact Pathophysiology of hemorrhagic stroke o Rupture of vessel long exposed to HTN o Aneurism o Acute or subacute increases in BP or flow in regions of previously normal small arteries o Hemorrhagic transformation Secondary bleeding due to alteration of vessel wall permeability and integrity, restoration of flow to injury site Dissects, compresses, and displaces adjacent brain = increased intracranial pressure and edema = vasospasm and resulting cerebral infarction Clinical manifestations o Neurologic symptoms occurring gradually o Headache and seizure may be present o Symptoms worsen over time Syndromes o Reflects dysfunction related to the area of the brain damage o Named according to the arteries that feed areas o Proximal arterial damage can cause greater damage depending if flow is provided collateraly as in the Circle of Willis Middle cerebral artery syndrome o If entire MCA is blocked Hemiplegia Hemiesthesia Global aphasia (dominant hemisphere) o Partial occlusions Combination of weakness, sensory disturbance, motor aphasia depending on branch Anterior cerebral artery syndrome o Infarction of ACA uncommon o Collateral flow is able to compensate so dysfunction is minimal Traumatic Brain Injury Epidemiology o 2 M/yr o Trauma causes 150,000 deaths o 90,000 are left with disability o Young males are preferentially affected o The most common cause of severe TBI is Motor vehicle accidents Assaults, falls, penetrating injuries, and self-inflicted injuries comprise the majority of other injuries o Ethanol intoxication o Illegal drug intoxication Pathogenesis o Epidural hematoma – blood clot forms between the dura and the skull o Subdural hematoma – tearing of bridging veins between the dura and the brain o Intracerebral hematoma – occurs with direct trauma to intracranial vessels o Contusion – a hemorrhagic transformation is characterized by venous injury, edema, and ICP o Diffuse axonal injury (parenchyma) – a distinct pattern of white matter damage. The corpus callosum is typically involved o Direct vascular injury- vascular injury after closed head injury Biochemical alterations in traumatic brain injury o Neuronal injury from trauma is similar in most respects to injury from other forms of ischemia o Pharmacological interventions o There is a degree of primary damage from the injury hat is refractory to any means of neuroprotection o However, a penumbra of tissue may be responsive to neuroprotective agents Clinical manifestations o The clinical examination of a patient with severe brain injury is similar to the evaluation of a comatose patient o Following evaluation and stabilization of airway, breathing, and circulation, attention is focused on the neurologic examination o The Glasgow Coma Scale score is a quick and reliable test of neurologic function Scores eye opening, motor response, and verbal output to obtain a score between 3 and 15 o Intubated patients are assigned a verbal score of 3 Glasgow Coma Scale – Eye opening o Spontaneous = 4 o To speech = 3 o To pain = 2 o Nil (no response) = 1 Glasgow Coma Scale – Motor response o Obeys = 6 o Localizes = 5 o Withdraws = 4 o Abnormal flexion = 3 o Extensor response = 2 o Nil = 1 Glasgow Coma Scale – Verbal response o Oriented = 5 o Confused conversation = 4 o Inappropriate words = 3 o Incomprehension sounds = 2 o Nil = 1 Neurologic testing o Observation for signs Basilar skull fracture Battle’s sign (blood over the mastoid process) Raccoon eyes Palpation and observation for open, depressed skull fracture o Pupillary examination Presence of afferent pupillary defect indicating trauma to the optic nerve or third nerve palsy with interruption in parasympathetic efferents leading to a dilated pupil o Corneal reflex The corneal reflex tests the trigeminal nerve (sensory limb) and facial nerve (motor limb) o Cold calorics In the head injured patient, the neck will also be immobilized Doll’s eye maneuvers, therefore, are contraindicated o In comatose patients Infusion of cold saline into the ear will cause a chronic deviation of the eye to the opposite side with a corrective beat towards the midline if the pontine gaze centers are intact Absent cold calorics indicate damage below the mid pons o Gag reflex Gag reflex test the ninth and tenth cranial nerves o Extremities Each extremity must be assessed Focal hemiparesis may be either contralateral or ipsilateral to an intracranial mass lesion Isolated lower extremity weakness could indicate a spinal cord injury When quadriplegia is present, a cervical spine injury must be suspected o Rectal tone Rectal tone will be diminished in cases of spinal cord injury Cognitive and behavioral impairments o Disorders of learning, memory, and complex information processing o Confusion, disorientation, difficulty problem solving, delayed processing, motor and verbal disinhibition, and lack of initation o Combativeness, agitation, poor social behavior, erratic wondering, motor and sensory perseveration, restlessness o Rancho Los Amigos Cognitive Scale Brain stem damage o CN I – anosmia o CN II – monocular blindness, absent pupillary response, visual defects o CN III – strabismus, diplopia, ptosis, dilated pupil, loss of accommodation reflexes o CN IV – vertical diplopia o CN V – anesthesia to nose, eyebrow, forehead, cheek, upper lip, gums, teeth o CN VI – loss of eye abduction o CN VII – loss of tear production, saliva secretion, taste anterior 2/3 tongue, lack of facial expressions o CN VIII – hearing and vestibular dysfunction, dizziness, vertigo o CN IX, X, XI, XII – cardiac abnormalities, excessive salivation, loss of sensation and gag reflex, taste posterior 1/3 tongue, hoarse voice, dysphasia, and deviation of tongue Motor deficits o Flaccidity, spasticity, rigidity o Decorticate posturing – hyperactive flexor reflexes in the UE and hyperactive extensor reflexes in the LE (loss cortical control) o Decerebrate rigidity – hyperactivity extensor in both UE and LE (loss of inhibitory control of the cortex and basal ganglia) o Hemiparesis Loss of selective motor control Abnormal balance reactions Sensory loss o Cerebellar and basal ganglia dysfunction Heterotopic ossification o Abnormal bone growth around a joint o Cause unknown o Onset 4 to 12 weeks post injury o Presents Loss of ROM Local tenderness and palpable mass Erythema, swelling, pain with movement Medical management o ABC (airway, breathing, circulation) o Complete physical examination o Level of consciousness (Glasgow) o Radiography (MRI, CT) o Indications for surgery o Indications for ICP monitoring o Intercranial hypertension monitoring o Hydrocephalus and ventriculitis o Cerebral perfusion pressure monitoring o Hyperglycemia: Cortisol release increases blood sugar o Fever o anticonvulsants Prognosis o Poorer < 7 (Glasgow Coma Scale) Subdural hematoma (CT scan) Old age Pupil remain dilated (pupillary light reflex) Eyes do not deviate (caloric testing with ice water) Decerebrate rigidity (motor response to noxious stimuli) Deficient (brainstem auditory evoked response) o Better ˃ 7 (Glasgow Coma Scale) Normal (CT scan) Youth Pupil constricts (pupillary light reflex) Eyes deviate to irrigated side (caloric testing with ice water) Localizes (defensive gesture) (motor response to noxious stimuli) Normal (brainstem auditory evoked response) Spinal Cord Injury (SCI) Categorized o Traumatic Most common cause of adult SCI o Nontraumatic injuries Catastrophic event of low incidence and high cost It is most often highly active persons that incur the types of accidents that cause severe SCI Current research o The belief that SCI is irreparable and that there cannot be nerve regeneration in the spinal cord is being replaced by improving care Pharmacologic intervention Hemodynamic factors Compression management Surgical timin o Critical care management Incidence and rick factors o Over the last 2 decades, tetraplegic injuries have become less common than paraplegic injuries o Incomplete SCI is now more common than complete SCI Causes of SCI o Motor vehicle accidents, Ballistic violence, Falls, Sports o An alarming increase in the incidence of gunshot wounds Pathogenesis o Result of vertebral injuries Due to acceleration, deceleration, or deformation forces o Result of violent displacement, stretching, compression, shearing, or penetration o If these occur only momentarily, there may be no neurological sequelae o The mechanism of injury has an influence on the type and degree of the spinal cord lesion (Hyper) Extension injury o Fracture dislocation of posterior elements o Usually in cervical area (Hyper) Flexion injury o Results in a fracture/dislocation of the vertebral elements o Approximately 50% of injuries come from excessive flexion Complete spinal cord lesions occur in ~1/3 of flexion injuries Dislocation in a flexion injury causes a complete lesion 50% of the time Common in the cervical area Compression injury o Common from T12-L2 o With these shattering crush fractures of the vertebrae, there is a 75% change of a complete spinal cord lesion (Flexion) Rotation injury o Ruptures support ligaments in addition to producing fractures Types of injuries o Cord concussion – caused by blow or violent shaking and results in temporary loss of function o Cord contusion – bruises with hemorrhage and swelling with temporary loss of cord mediated functions; associated with fractured bone or osteophytes segments striking the spinal cord o Cord compression – pressure on the cord causes ischemia. Must be decompressed to prevent permanent damage. o Laceration (tearing & disruption of tissue) – may be reversible if only slight damage; may result in permanent loss o Transection (complete or incomplete) – severing of the spinal cord, tracts are disrupted; permanent loss of cord-mediated functions o Hemorrhage – extradural – common, although rarely become large enough to compromise the spinal cord; subarachnoid – due to contusion and laceration of the cord, are frequent, and can cause further compression of the cord o Ischemia – caused by damage to or obstruction of spinal blood supply; local ischemia may cause permanent or temporary damage Neuronal damage o Secondary neuropathologic changes begin immediately after injury o Neural damage to the spinal cord extends beyond the initial contusion Spinal shock, the loss of sensory, voluntary motor, and automatic control below the level of the lesion, occurs after the trauma The mechanism is poorly understood, and resolved within a few weeks after injury Blood flow changes o Changes in blood flow Small petechial hemorrhages After several hours gross hemorrhages present Preceded by endothelial breakdown and pathologic coagulation in vessels o Ischemia and necrosis occur primarily in the gray matter, presumably because of the richer blood supply o Macrophages enter the lesion and begin to digest the necrotic debris, converting the complex myelin lipids to neutral fat o Axonal swelling and increased permeability of blood vessels result in a visibly swollen spinal cord o Glial cells Activated after about 6 days Astrocytic fibers form scar like tissue that lines that cavities created by the necrosis Demyelination o Demyelination is probably due to direct trauma to oligodendroglial cells The myelin sheath becomes this between the nodes, and this is responsible for a decrease in the peak currents along the axon Synaptic dyfunction o Calcium ion rush into the cell, disrupting mitochondrial electron transport and activating phospholipases o Injured mitochondria Generate oxygen free radicals which break down the membranes and cause lipid peroxides and free arachidonic acid to be released Diagnosis o Lateral film studies with plain radiographs o CT scans & myelography o Magnetic resonance imaging (MRI) o Neurophysiologic studies Proper emergent care is critical o Assessment includes understanding the mechanisms of the trauma and obtaining vital signs Paradoxical respiration or abdominal breathing May be present and immediate immobilization should in instituted Use of a rigid collar and spinal board Helps to prevent movement of the spinal column Movement of the distal components of the body Reflects the intactness of the spinal cord Oxygen and medication Given to control the hyperperfusion and swelling of the spinal cord Transport Swift, avoiding physical jarring caused by an uneven road surface and sudden stops Emergency room care o Immediate immobilization of spine To prevent further injury o Orthopedic management Closed or open reduction of the vertebrae and decompression of the spinal cord (laminectomy) o Immobilization in beds or frames, with and without traction When upright, body jackets, braces, or traction devices will be required Monitoring in the critical care phase includes cardiac and neurologic status Examples of immobilization o A body jacket or brace will be used to protect thoracic and lumbar injuries o The Halo apparatus provides immobilization for cervical injuries Cooling of spinal cord o A surgical procedure which when accomplished within 8 hours following trauma has shown promising potential for preserving function Acute respiratory management o High cervical injuries require immediate placement on ventilation equipment and maintenance of pulmonary hygiene Therapy consists of intermittent positive pressure breathing (IPPB), bronchodilators, and mucolytics Prevention of pulmonary infection is critical Autonomic regulation o Acute management of blood pressure is critical o Loss of autonomic nervous system control affects the function of the cardiovascular system Autonomic lesion predisposes persons with high spinal cord lesions to abnormal cardiovascular responses o Management of autonomic dysreflexia Involves determination of the site and nature of the initiating stimulus and alleviation of the precipitating cause o Drugs may be used to lower the blood pressure Clinical manifestations o SCIs are named according of the level of neurologic impairment The level of injury reflects the most caudal level of the SC that exhibits intact sensory and motor functioning o Lesions are reported as Complete No sensory or motor function can be demonstrated below the level of the lesion Incomplete lesions Partial loss of sensory and motor function below the level of the injury Determining level of injury o The American Spinal Injury Association (ASIA) has created standards for assessment and classification, which are widely used The sensory examination consists of testing 28 dermatomes on each side of the body using pinprick and light touch, which are then scored as 0 = absent; 1= impaired; 2 = normal o Function of the external anal sphincter is recorded as “yes or no”: o ASIA has also created standards for assessment and classification of motor injury o Key muscles are used to identify function at each level of the cervical and lumbar roots Motor innervation guidelines from the American Spinal Injury Association o C5 – elbow flexors o C6 – wrist extensors o C7 – elbow extensors o C8 – finger flexors (distal phalanx of middle finger) o L2 – hip flexors o L3 – knee extensors o L4 – ankle dorsiflexors o L5 – long toe extensors o S1 – ankle plantar flexors American Spinal Injury Association Impairment Scale o A = Complete – No sensory or motor function is preserved in the segment S4-S5 o B = Incomplete – Sensory but no motor function is preserved below the neurologic level. Sensory function extends through segments S4-S5. o C = Incomplete – Motor function is preserved below the neurologic level. Sensory function extends through segments S4-S5. o D = Incomplete – Motor function is preserved below the neurologic level, and the majority of key muscles below the neurologic level have a muscle grade ≥ 3 o E = Normal – Sensory and major functions are normal Spinal cord injury syndromes o Within the category of incomplete spinal cord lesions are some recognizable syndrome Changes in muscle o Paralysis of the voluntary musculature Descending motor tracts Anterior horn cells Spinal nerve Combinations of tracts Damage to descending tracts Results in immediate flaccidity and absent reflexes called “spinal shock” May be followed by autonomic symptoms Sweating and reflex, incontinence of bladder and rectum Spasticity o Basic spasticity May be of some benefit to the individual when emptying the bladder or flexing the hip and knee o Excess spasticity is thought to be due to imposed afferent stimuli o Drug therapy is used to treat the spasticity o Can be exacerbated by a sudden change in temperature or by physical or emotional stress o Can also be made worse by the presence of constipation, infection, fracture, or a pressure sore Respiratory complications o Lesions above C4 result in paralysis of inspiratory muscles and generally require artificial ventilation due to loss of the phrenic nerve Can be life-threatening o Pulmonary complications in lesions at C5 through T12 arise due to loss of innervation of the muscle of expiration (abdominals) and intercostals Bowel and bladder control o The spinal center for urination is the conus medullaris Sacral cord segment (in an adult, located between L1 and L2 vertebra) o During spinal shock, the urinary bladder is flaccid. All muscle tone and bladder reflexes are absent Sexual response o Sexual response is directly related to the level and completeness of injury o There are two types of responses Reflexogenic or response to external stimulation Psychogenic which occur through cognitive activity such as fantasy o Men Higher level lesions can often achieve a reflexive erection, but typically do not ejaculate Lower lesions can more easily ejaculate, but the ability to achieve an erection is more difficult Cauda equine lesions, erections, and ejaculation are not usually possible Female sexual responses o Menses are typically interrupted for approximately 3 months after injury o Sensory loss alter perception of orgasm but performance is usually not impaired o Fertility and pregnancy are unchanged Pregnancy must be followed closely, especially in the last trimester and most babies are delivered by C-section Labor can begin without the woman’s awareness due to loss of sensation, and labor may initiate autonomic dysreflexia ANS changes o Loss of thermoregulation below the level of the spinal cord lesion Result of the disruption of the autonomic pathways form the hypothalamus o Vasoconstriction, the ability to shiver, and the ability to sweat are lost o Body temperature then is greatly influenced by the external environment and the sensory feedback from the head and neck must be used to assist in the regulating body temperature o The higher the lesion, the more severe the problem Musculoskeletal changes o Joint ankylosis due to heterotopic ossification, or ectopic bone formation in the extra-articular soft tissue such as the tendons and connective tissue, can limit range of motion and cause pain Sensory changes o Dysesthesia Impairement of sensation usually perceived as pain, can occur in areas that have no sensation Described as “burning”, “pins and needles”, or “tingling” o Disturbances of proprioception Person feels that a limb is in a different position than it really is o Pain due to irritation of the nerve root is common, especially in cauda equine injury Spasticity o Treatment of spasticity Includes muscle relaxants and spasmolytic agents o Peripheral nerve blocks Provide a temporary reduction of spasticity o If there is long-term severe spasticity, the contractile potential of the muscle can be modified by surgery • Sexual Responses • Reflexogenic: response to external stimulation • Psychogenic: Occur through cognitive activity such as fantasy • Women lose of menses, no sensory response Neurogenic pain management o Systemic or local therapy Nonsteroidal analgesics, opiods, and antidepressants and anticon-vulsants o Neuro-augmentative procedures Transcutaneous electrical nerve stimulation (TENS), epidural spinal stimulation, and central thalamic stimulation (primarily in experimental studies to date) o Neuro-destructive procedures Chemical and surgical destruction of nervous structures Deafferentation, interruption of ascending pain systems, or destruction of cells in the dorsal horn Secondary complications o Contractures o Deep vein thrombosis o Pressure sores o Postural hypotension o Osteoporosis below the level of the lesion o Kidney stones due to increased concentration of calcium in the urinary system o Musculoskeletal pain can result from faulty posture and over-use of limbs Summary o Stroke, traumatic brain injury, and spinal cord injury are major pathologies treated in rehabilitation o Physical and occupational therapists are important team members in the recovery and return to function process
