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Cerebrovascular disease A 68-year-old woman with a one-month history of nondescript aching and stiffness in her shoulders and intermittent fever, both responsive to Extra Strength Anacin, comes to your office concerned that she has developed a severe, right frontal throbbing headache and while reading this morning’s newspaper became aware that something was wrong with the vision in her right eye. On examination, she is blind in her right eye. 1. The most likely diagnosis is: 2. How would you confirm your diagnosis? ______________________________________________________________________ A 65-year-old previously healthy housekeeper awakens from sleep unable to stand or walk. On exam, she has moderate-to-severe left leg and foot weakness, mild left arm weakness, is unable to identify numbers drawn over the dorsum of her left foot, but can identify them when drawn over the dorsum of her right foot or palm of either hand. There is no language deficit or visual field loss. The left plantar response is extensor. 1. What is your diagnosis? 2. Where is the lesion? ______________________________________________________________________ A 65–year-old man drove himself to the emergency room after experiencing a suddenonset ten-minute episode of right hand weakness and word-finding difficulty. He had experienced similar episodes over the last month but this one had lasted considerably longer. In the ER he tells you he has had a long history of hypertension and diabetes for which he takes medications regularly; he takes no other medications. He had a cardiac stent implanted a year ago following an evaluation for unstable angina. His neurological examination is normal except for a mid-calf stocking neuropathy with diminished deep tendon reflexes at the ankles. 1) Where would you localize the brain lesion that caused this man’s symptoms? What vascular territory? 2) What are the risk factors for stroke? 3) What studies would you suggest are appropriate in evaluating this individual’s problem? 4) How (or) would you treat this man’s presenting problem? ______________________________________________________________________ Case Study #1: Patient BF A 52-year-old, right-handed woman with medical history of hypertension awakened from sleep at 2330 hours with the urge to urinate. She arose from bed and walked to the bathroom adjoining the bedroom in her home. As she was walking, patient BF spoke to her husband who remained in the bed that she left. At 12 midnight (0000 hours), her husband was startled to hear the sound of the patient collapsing behind the closed door of the bathroom. He found his wife lying on the floor of the bathroom, unable to move 886484622 Printed: 6/21/2017 her left side but still capable of speaking. Patient BF was transported by a paramedic ambulance unit to the Emergency Department of a local community hospital and arrived at 0030 hours. Upon arrival, the patient weighed 100 kg and had blood pressure of 190/110. The pulse rate was regular at 98 beats per minute. She was afebrile. Patient BF breathed room air comfortably with oxygen saturation measured at 98% from a cutaneous monitor. In addition to hypertension and moderate tachycardia, the physical examination showed absence of cervical bruits or cardiac murmurs, rightward gaze preference, a left homonymous hemianopsia, left hemiplegia, left hypesthesia to pinprick, and an extensor plantar sign in the left foot. The patient was able to correctly identify her left hand when it was placed by the examiner within her intact right visual field. Computerized tomography (CT) of the brain was performed without infusion of iodinated contrast and is shown in the Figure displayed below: Figure: CT scan of the brain performed without contrast enhancement in a 52-year-old woman with acute onset of left hemiplegia. This scan was performed approximately 75 minutes after the onset of symptoms. 886484622 Printed: 6/21/2017 Questions 1. Which of the following statements regarding the clinical presentation is correct? A. The neurological deficit sustained by patient BF began at 2330 hours. B. The neurological deficit sustained by patient BF began at 12 midnight. C. Because of the patient’s body weight of 100 kg, she is not a candidate for administration of recombinant tissue plasminogen activator (rt-PA) to treat acute ischemic stroke. D. Because the blood pressure is elevated to 190/110, the intravenous dose of rtPA should be reduced by 50%. 2. Which of the following interpretations of the CT scan is correct? A. Subarachnoid hemorrhage B. Normal calcification of an intracranial artery C. “False falx sign” D. “Hyperdense MCA sign” 3. Which of the following is the most appropriate choice for treatment of hypertension experienced by this patient? A. The blood pressure of 190/110 should not be treated with any anti-hypertensive agent to avoid hypoperfusion of ischemic brain and further expansion of stroke. B. The blood pressure of 190/110 is supportive of the clinical diagnosis of hypertensive encephalopathy and should be treated aggressively to achieve systolic blood pressure of 160 mm Hg. C. The blood pressure of 190/110 should be treated with application of one inch of nitroglycerin paste to the skin surface and with serial boluses of labetalol given intravenously to lower the systolic blood pressure below 185 mm Hg. D. None of the preceding choices A, B, and C ______________________________________________________________________ Case Study #2: Patient AG Patient AG, a 21-year-old, right-handed man with Marfan’s syndrome, underwent replacement of the aortic and mitral valves and surgical repair of an aneurysm of the aortic root at age 19 years. He was placed on warfarin for anticoagulation after the surgery. Three days before admission to a local community hospital, the patient developed headache, fever to 102oF, and left-sided weakness. He was then transferred to an academic medical center. Physical examination upon admission revealed Marfanoid body habitus, normal mental status, no cervical bruits, mechanical “clicks” representing opening or closure of the aortic valvular prosthesis, left visual neglect, and a spastic left hemiparesis of modest severity. Direct funduscopy was unremarkable. A CT scan of the brain obtained upon admission to the local community hospital is shown in Figure 1. Selected views from a cerebral angiogram performed after transfer to the academic medical center are shown in Figure 2. Figure 1: CT scan of the brain performed without contrast enhancement in a 21year-old man with an infected aortic valvular prosthesis who was taking warfarin at the time of onset of left 886484622 hemiparesis and headache. Printed: 6/21/2017 Questions 1. Select the only correct statement from the following options: A. Examination of the fingers will show white, linear discolorations oriented transversely across the nail bed. B. Examination of the blood smear will show a microcytic, hypochromic anemia with fragmented red cells. C. Examination of the anterior chamber of the eye by slit lamp will reveal goldenbrown discoloration of the peripheral cornea. D. None of the preceding choices A, B, and C 886484622 Printed: 6/21/2017 Figure 2: Views of selective right carotid injection (upper panel: lateral projection; lower panel: anterior-posterior projection) during cerebral angiogram performed in a 21-year-old man who experienced the acute onset of left hemiparesis and headache in association with the lesion observed on CT of the head in Figure 1. 2. Select the most accurate statement from the following options: A. This patient sustained intracerebral hemorrhage (ICH) within a metastatic melanoma deposited in the right cerebral hemisphere. B. This patient sustained ICH in the right cerebral hemisphere due to spontaneous rupture of a congenital aneurysm at the bifurcation of the right middle cerebral artery. C. This patient sustained ICH in the right cerebral hemisphere due to spontaneous rupture of an arteriovenous malformation. D. This patient sustained ICH in the right cerebral hemisphere due to spontaneous rupture of a mycotic aneurysm. ______________________________________________________________________ 886484622 Printed: 6/21/2017 A 65-year-old male went to sleep on a Thursday night in his usual state of health. He is taking medication for high blood pressure and elevated cholesterol. He has smoked more than 2 packs per day for 40 years. Upon awakening Friday morning he cannot move his right side. He is alert and without headache or visual complaints. 1. What happened? 2. Where is the lesion? 3. Your diagnosis can be confirmed by…? ______________________________________________________________________ 886484622 Printed: 6/21/2017 Cord/root disease A 55-year-old male with a 3-week history of back pain radiating into the right leg comes to your office for evaluation. Abnormalities on exam include decreased sensation to pain over the dorsum of the lateral aspect of the right foot. 1. What muscles would you expect to be weak? 2. What deep tendon reflexes would you expect to find diminished or absent? 3. The 5th lumbar nerve root exits between what vertebrae? 4. What are the indications for a lumbar discectomy? ______________________________________________________________________ A 36-year-old construction worker complains of neck pain radiating into the left arm after stacking lumber on a flatbed truck. On examination, there is mild weakness of elbow extension, a depressed triceps deep tendon reflex, and loss of sensation to pinprick over the 3rd (middle) finger – all involving the left arm and hand. 1. What muscles comprise the C-7 myotome? 2. Where is the C-7 dermatome? ______________________________________________________________________ 886484622 Printed: 6/21/2017 Dementia A 40-year-old man with AIDS presents with memory problems. His problems date to six months earlier when he found he had to re-read everything several times as he was unable to recall what he had just read. His family states that he had become socially withdrawn. Examination showed him to be very slow in both response to questions and in movement. He had a masked face. His postural balance to threat was impaired and his gait slow and unsteady. An MRI showed diffuse brain atrophy. No focal weakness or sensory loss was evident. 1. List causes of dementia: 2. How do you distinguish one type of dementia versus another on clinical and radiologic grounds? ______________________________________________________________________ A 78 year-old woman visited her doctor because her husband "thought she needed to be checked." She reported that nothing was wrong, that her husband had always been overly protective. He reminded her that, while driving, she had become lost on several familiar streets and that, at a recent reunion, she had been unable to recall friends. About a year ago she had quit helping him with the bills, whereas they used to sit down once a month and go over finances together. She takes no medications, does not use tobacco, and rarely drinks alcohol. Her general exam is normal. She is very outgoing and speaks fluently, but makes frequent paraphasic errors. She cannot recall any of three simple objects, has significant difficulty drawing a simple clock, and cannot think of the name of the current president. When told the President's name she says, "I knew that. I thought you asked if I had voted for him." 1. Where would you localize a lesion that leads to: a. Paraphasic errors? b. Problems with short-term memory? c. Difficulty constructing simple figures? 2. What are the 3 most common causes of dementia in the US? 3. What is the difference between a cortical and a subcortical dementia? 4. One should screen for what treatable diseases in a person who presents with signs/symptoms of dementia? 5. What is the recommended therapy for Alzheimer’s disease? What is the prognosis? ______________________________________________________________________ NEUROPSYCHOLOGICAL CASE #1 Reason for Referral: A 61-year-old, right-handed, Caucasian male is referred for a neurocognitive evaluation by a neurologist secondary to a two-year progression of slowed cognition, memory difficulty, nocturnal disorientation, difficulty maintaining train of thought, increased distractibility, word-finding difficulty, usage of incorrect words and relatively intact activities of daily living independently. Patient spends his day working on 886484622 Printed: 6/21/2017 his farm or completing projects at his home. Patient’s medical history is significant for a REM-sleep behavior disorder, vivid dreams, chronic headaches, head injuries with brief LOC, back pain, ulcers and orthostatic hypotension with a possible neurological diagnosis. Neuroimaging and EEG have been normal. Collateral Information: Patient’s wife indicates patient is occasionally unable to drive secondary to dizziness but is otherwise able to manage activities of daily living. Wife reports patient has fallen often in the last year with no known cause. Wife indicates patient is disoriented and sluggish after these episodes. Background Information: Patient completed high school and three years of college. He denies a history of special education or learning disability. Patient worked as an electromechanical engineer until retiring in 1995. He has been married for 37 years and has two adult children. His adult son lives at home with he and his wife secondary to a seizure disorder. Current medications include Lipitor, Tricor, Inderal, amitriptyline, Nexium, Paxil, and Klonopin. Family medical history is significant for myocardial infarction, cancer, lung disease, bipolar disorder, and unknown dementing illnesses (grandfather and father). Psychiatric history is unremarkable. Patient describes his mood as easygoing, and denies depression and anxiety. His wife’s description was similar. Patient reports social consumption of alcohol and denies current use of tobacco products. He reports a history of heavy alcohol consumption when he was in the Navy. He describes his sleep quality as consistent. Patient indicates his appetite has increased. Procedures: Clinical Interview, Collateral Interview, Functional Assessments Inventory, Memory Assessment Questionnaire, Mini-Mental Status Examination (MMSE), Wechsler Adult Intelligence Scale-III (WAIS-III, select subtests), Boston Naming Test (BNT), National Adult Reading Test (NART), Sentence Repetition, Token Test, Verbal Fluency (FAS and Animal Naming), California Verbal Learning Test-II (CVLT-II), Wechsler Memory Scales-III (WMS-III, select subtests), Wisconsin Card Sorting Test (WCST), Ruff 2 & 7 Selective Attention Test, Trails A & B, Finger Tapping Test, Test of Memory Malingering (TOMM), Geriatric Depression Scale (GDS). Behavioral Observations: Patient was awake and alert and demonstrated an appropriate level of arousal throughout the examination process. Affect was appropriate. No thought disturbance or bizarre mentation was evident. Gross motor functioning appeared intact. Speech was of normal rate, volume, and prosody; however, several mispronunciations were noted. Patient’s performance on a task of effort was good; therefore, the current evaluation is believed to accurately reflect his neurocognitive functioning. Summary of Test Data: Patient’s effort on testing was good; therefore, test results are believed to provide an accurate indication of his cognition. Global mental status indicated disorientation to time (3/5) and place (4/5), mild memory impairment with 2/3 objects recalled, and mildly impaired repetition (MMSE = 25). His pattern of neurocognitive testing is characterized by slowed upper motor speed, slowed 886484622 Printed: 6/21/2017 processing speed, inefficient working memory, dysfluency, fluctuating divided attention, impaired retrieval of visual and auditory material (relative to recall) superimposed on average intellectual abilities (FSIQ = 94). Testing was not suggestive of global intellectual deterioration. Visuospatial skills, abstraction, problem solving, simple attention, naming, retention and receptive language were within normal limits. Patient is not acknowledging considerable affective distress, but mild worry about his physical symptoms. Question 1: Is neurocognitive testing globally consistent with a cortical or subcortical process? What are characteristics of each? Question 2: Is testing suggestive of a possible Alzheimer’s disease, Seizure disorder, Parkinson’s disease (or Parkinson’s plus dementia), or dementia with Lewy bodies? ______________________________________________________________________ NEUROPSYCHOLOGICAL CASE #2 Reason for Referral and Background Information: Patient is a 75-year-old Caucasian female referred for a neurocognitive evaluation as a component of the Memory Disorder Clinic at Sanders-Brown Center on Aging secondary to cognitive dysfunction. Patient reports a six-month history of forgetfulness and decreased attention when driving. She reports she has been involved in several motor vehicle accidents in the last few months. She denies other change in ability to manage activities of daily living independently. She currently lives alone; however, one son frequently visits overnight. Her daughter reports a three-year history of gradual memory decline, decreased comprehension, disorientation to time and place, and difficulty managing daily activities including remembering dates, managing finances, and driving. She indicates her mother frequently repeats herself and still drives locally despite directional confusion and her involvement in several accidents. Patient has an eighth grade education and denies a history of special education or learning disability. She is a retired nurse’s assistant. She is divorced and has five living adult children. Medical history is significant for hysterectomy, aortic insufficiency, hypertension, asthma, and hypothyroidism. Current medications include Accupril, Synthroid, Theolair, calcium supplement, multivitamin, and vitamin E. Family medical history is significant for cancer and Alzheimer's disease. Psychiatric history is significant for treatment of anxiety and depression. She denies current use of alcohol, tobacco, and illicit drugs. Patient describes herself as mostly happy; however, she reports occasional sadness secondary to financial limitations on her activities. Her daughter indicates patient has always been very emotional. Patient reports consistent appetite and sleep quality. Behavioral Observations: Patient was awake and alert and demonstrated an appropriate level of arousal throughout the examination process. Affect was occasionally tearful and patient expressed concern regarding her performance. She wore corrective lenses. No thought disturbance or bizarre mentation was evident. Gross motor functioning appeared intact. Speech was of normal rate, volume, and prosody. 886484622 Printed: 6/21/2017 Patient’s effort appeared consistent; therefore, the current evaluation is believed to be an accurate reflection of her neurocognitive functioning. Test Results: Testing is characterized by mild to moderate cognitive impairment with prominent dysnomia, receptive language dysfunction, disorientation, memory impairment, constructional dyspraxia, executive dysfunction, and moderate IADL impairment. Global mental status on the MMSE (21) suggests mild to moderate impairment based on disorientation to time (3/5) and place (3/5), and episodic memory changes (3/3 immediate recall, 1/3 delayed recall). She was unable to spell “world” backwards (4/5), could not repeat “no ifs, ands, or buts” (0/1), and was unable to complete an oral three-step command (2/3). Testing is suggestive of a mild to moderate dementing disorder such as possible Alzheimer’s disease with co-morbid depression and anxiety; however, an underlying vascular component cannot be ruled out. Question 1: a) Can the patient’s eighth grade education account for her cognitive impairment? b) Does patient’s limited eighth grade education increase her risk for dementia? ______________________________________________________________________ A 52 year-old male develops expressive dysphasia over several months. He becomes unkempt, disinterested and loses his temper easily. He has previously been healthy. There is no history of neurologic disease in the family. On exam he has several frontal lobe release signs, poor recent memory and a nonfluent dysplasia. His MR scan shows frontal and temporal lobe atrophy. 1. Diagnosis? 2. What are frontal lobe release signs? 3. What are the common causes of dementia? ______________________________________________________________________ 886484622 Printed: 6/21/2017 Demyelinating Disease A previously healthy 28 year-old female develops sudden loss of vision in the left eye. Her visual acuity is 20/400 in the left eye. The left pupil is 2mm larger than the right. Shining a bright light in the right eye causes a brisk, equal constriction of both pupils. Shining the same light in the left eye causes a brisk constriction of the right pupil but a sluggish response on the left. VER (visual evoked response) for the right eye is normal but the VER for the left eye is delayed (a prolonged P100). The fundi are normal. This lady has which one of the following: a. optic neuritis b. papillitis c. papilledema d. increased intracranial pressure e. retrobulbar neuritis 1. What is likely to be her underlying disease? 2. What might her CSF exam show? 3. What abnormalities might her MRI of the brain show? ______________________________________________________________________ A 26-year-old woman presents to the ER following the sudden onset of bilateral lower extremity weakness and numbness. She denies prior neurological symptoms, but examination shows a right Marcus Gunn pupil and inability to read color plates with the right eye. Temporal pallor was observed in both eyes. Her lower extremities were profoundly weak and bilateral Babinskis were elicited. There was sensory loss to pinprick at T10. 1. What is the difference in retrobulbar neuritis and optic neuritis? 2. What are CSF findings in MS? 3. What is your diagnosis? ______________________________________________________________________ A 37 year-old bank teller came to your office complaining of a right arm tremor that worsened with movement. She was also having difficulty counting change with that hand and stated she "couldn't make it do" what she wanted. These symptoms had insidiously progressed over several days and had now been about the same for a week. She had been healthy all of her life and denied any prior neurological problems. She did seem to remember that, as a teenager, she had experienced an episode of decreased visual acuity OD which lasted about a month and then spontaneously cleared over about a week. Additionally, her husband reminded her that after the birth of their first child, she had had difficulty with micturition and had to use a catheter for about 2 - 3 weeks. 1. How well can you neurologically localize each of the highlighted symptoms? 886484622 Printed: 6/21/2017 2. What is the most likely diagnosis for this woman? Why? 3. What additional studies might be helpful at this time? 4. Should this woman be treated? If so, what would you consider appropriate treatment? Why? 5. If I were to tell you this was a case of multiple sclerosis, what could you tell me about underlying disease mechanism? ______________________________________________________________________ 886484622 Printed: 6/21/2017 Epilepsy A 16-year-old female with a history of simple partial seizures is brought to the ER because of stupor. Examination reveals slow responses to simple questions, normal vital signs and no focal neurologic findings. A urine drug screen is positive for tricyclic antidepressants. 1. What are some diagnostic possibilities? 2. When mom arrives in the ER what additional information would you like to have? ______________________________________________________________________ An 8-year-old male is taking phenytoin 100 mg BID for partial complex seizures. He has been seizure-free for 10 months. His general health is good. He is referred to you for temper tantrums, mood swings and outbursts of destructive, combative behavior. You place this child on valproic acid 250 mg BID. One week later the child’s mother calls you because the child is stumbling, clumsy, and sleepy. In addition, he had one 90-second generalized tonic-clonic seizure. 1. What are some diagnostic possibilities? 2. What is the physiologic basis for your diagnosis? ______________________________________________________________________ A 27-year-old woman in the second trimester of her first pregnancy was referred to the Epilepsy Clinic from the High-Risk Obstetric Clinic. As a college student, at the age of 19, she had her first generalized tonic-clonic seizure. She had three more seizures during her early twenties, each beginning by staring and picking at her blouse buttons before progressing into a generalized tonic-clonic seizure. She was the product of a normal pregnancy and delivery with normal developmental milestones. At age 15 months she had experienced two prolonged seizures with high fever, both lasing approximately 15 minutes. She had continued to develop normally and had been successful both athletically and scholastically. 1. What type of seizure does she have? 2. What drug would you use and why? ______________________________________________________________________ A 26 y.o. woman in the second trimester of her first pregnancy was referred to the Epilepsy Specialty Clinic. In her early 20’s she had had the first of three generalized seizures that were ultimately controlled with carbamazepine, which she continues to take. Each of these episodes began with a vague and “scary feeling,” followed by loss of awareness. On one occasion she was also observed to stare and pick at her blouse before progressing into a tonic-clonic seizure. She had been the product of a normal birth and delivery, but at age 10 months she had had two prolonged febrile seizures, both lasting about 15 minutes and associated with transient paralysis of her left arm. She had otherwise developed normally and had been successful both athletically and scholastically. 886484622 Printed: 6/21/2017 1. Does this woman have epilepsy? Based on her symptoms, how would you classify it? 2. How well can you localize the ictus, or does it begin as a generalized phenomenon? 3. What is the significance of complicated febrile seizures? 4. What is the mechanism of action of carbamazepine in the treatment of epilepsy? Are there other anticonvulsants that share this mechanism? 5. How is the potential teratogenicity of anticonvulsants mitigated? Should this woman’s anticonvulsant be changed at this time? ______________________________________________________________________ Sitting in the classroom, 9 year-old Meriem felt "funny" and after several minutes she had the strange feeling that she had never seen the classroom, her class mates or the teacher before. During this time the teacher noticed that Meriem was staring and unresponsive. Meriem then looked confused and fumbled with her papers for another 60 seconds after which she was back to normal. She has had this type of spell once or twice a month for the entire school year. No one in the family has similar episodes. Except for a 30 minute seizure with a febrile illness at age 18 months she has been well. Meriem is an average student. 1. What is the differential diagnosis? 2. What is the most likely diagnosis? 3. What tests would be appropriate and what would they be expected to show? 4. How would you manage this child? ______________________________________________________________________ Four-year-old Joe has had four spells, all shortly after going to bed at night. With each one he has jerking of his left lower face that rapidly becomes generalized and lasts about one minute. He is developmentally normal and has a normal examination. Family history is negative for anyone having similar episodes. Pregnancy, labor and delivery were unremarkable and he has never had a serious illness. 1. What is the differential diagnosis? 2. What is the most likely diagnosis? 3. What tests would be appropriate and what would they be expected to show? 4. How would you manage this child? ______________________________________________________________________ As she starts to answer the teacher's question, 8 year old Allison stops in mid-sentence, stares without moving for 30 seconds and then resumes speaking. She is apparently unaware that she did anything unusual and resumes her previous activity. Mother reports that pregnancy, labor and delivery were unremarkable. Allison did well in school until September when these spells began. Mother has noticed 10 or 12 spells a day. Sometimes Allison will stop in the middle of a sentence, have a spell and then resume 886484622 Printed: 6/21/2017 talking. There is a strong family history of epilepsy on father’s side of the family. 1. What is the differential diagnosis? 2. What is the most likely diagnosis? 3. What tests would be appropriate and what would they be expected to show? 4. How would you manage this child? ______________________________________________________________________ Running through the house, 2 year old Bill trips, falls, cries, suddenly stops crying, seems to stop breathing, and then arches his back for about 10 seconds. He then seems to catch his breath, resumes a normal posture, and falls asleep. He is the product of an uncomplicated pregnancy, labor and delivery. His development has been normal and he has not had any surgery or serious illness. Family history is negative for epilepsy. 1. What is the differential diagnosis? 2. What is the most likely diagnosis? 3. What tests would be appropriate and what would they be expected to show? 4. How would you manage this child? ______________________________________________________________________ Three-year-old George W. has repeated episodes where he wakes up at night disoriented and confused. These spells last 15 to 45 minutes and during them he cries, looks fearful, and is out of contact with his environment. After the episode ends he goes back to sleep. He has never had any sort of spell during the day. These episodes began 6 months ago and occur once or twice a month. He is the product of an uncomplicated pregnancy, labor and delivery. His development has been normal and he has not had any surgery or serious illness. Family history is negative for epilepsy. 1. What is the differential diagnosis? 2. What is the most likely diagnosis 3. What tests would be appropriate and what would they be expected to show? 4. How would you manage this child? ______________________________________________________________________ Five year old Arman was well until this morning when he had a low grade fever and ‘just lay around’. This evening he stopped talking and then began to have jerking movements of his right hand that progressed to a generalized seizure. After a large dose of IV lorazepam followed by phosphenytoin his seizures stopped but he is sleepy, not speaking, and febrile to 101. On examination he has a mild right hemiparesis. His spinal fluid has 400 WBC, 86 RBC, glucose = 52, and protein = 89. He was previously well. He is the product of an uncomplicated pregnancy, labor and delivery. His development has been normal and he has not had any surgery or serious illness. Family history is negative for epilepsy. 1. What is the differential diagnosis? 886484622 Printed: 6/21/2017 2. What is the most likely diagnosis 3. What tests would be appropriate and what would they be expected to show? 4. How would you manage this child? ______________________________________________________________________ Bob was well until the 1st grade when he developed a series of rapid eye-blinks. They resolved but that summer he began to do a rapid & repetitive nose twitch. That also resolved but his second grade teacher complained that he made repetitive sniffing noises. Now at the beginning of 3rd grade, his mother reports that Bob also makes an unusual, brief, rapid head turning movement to the right. Bob seems unaware of any of this. He was previously well. He is the product of an uncomplicated pregnancy, labor and delivery. His development has been normal and he has not had any surgery or serious illness. Family history is negative for epilepsy. 1. 2. 3. 4. What is the differential diagnosis? What is the most likely diagnosis? What tests would be appropriate and what would they be expected to show? How would you manage this child? 886484622 Printed: 6/21/2017 Headache A 50-year-old woman with a history of migraine headache comes to the ER with the chief complaint of headache, which came on very suddenly about two hours earlier. The pain is described as severe and began without the aura she usually experiences with her migraines. She had a similar headache about 3 weeks ago, but it was not as severe, her neck seemed stiff for a few days after the headache. The remainder of her history is unremarkable. Her family history is positive for migraines and two family members have polycystic kidney disease. On examination, she acts sleepy, has a complete right CN III palsy, diffusely but mildly brisk tendon reflexes, and positive Kernig’s and Brudzinski’s signs. A CT scan of the head, done without contrast was ordered before you were told of the patient; you agree with the radiologist that it is normal. 1. What test would confirm your diagnosis? ______________________________________________________________________ A 16 year-old female comes to your office with a chief complaint of progressive headaches for 6 months. She is otherwise healthy with an unremarkable past history. Her exam is normal except for obscure disc margins (optic nerve). 1. What would you include in your differential? 2. What is “pseudopapilledema”? 3. What diagnostic tests would you perform? 4. What are some known causes of pseudotumor? 5. If this patient’s opining pressure was 190 mm of H2O, what would the diagnosis be? 6. Why is Diamox effective in treating pseudotumor cerebri? ______________________________________________________________________ “Brenda” is a 32 year old second grade school teacher who has had bilateral periorbital headaches since the age of 14. She remembers having to leave school early in high school, feeling sick to her stomach and wishing to lie down in a quiet room. The headaches are often worse during her menstrual period, but can occur anytime, particularly after times of stress or with abrupt weather changes. Brenda recalls her mother having had “sick headaches,” when she and her sisters needed to keep quiet and turn off all the lights in the house. 1. What are the primary causes (not due to structural lesions, or metabolic dysfunction) of headache in young women? 2. If you had one question to ask Brenda, to help differentiate this headache from an “ordinary,” or mild headache, what would it be? 3. Brenda would like to avoid taking preventative medicines for headache. What can you tell her about her diet that may minimize or avoid headache; i.e. what are common headache triggers?” 4. Does the location of Brenda’s headache help you identify its cause? 886484622 Printed: 6/21/2017 5. Brenda would like to become pregnant. What will happen to her headaches in pregnancy, and which medications are safe for the fetus? ______________________________________________________________________ “Charles” is 42 years old and has had headaches that occur in a peculiar pattern, for more than twenty years. They have a circannual and circadian rhythm, occurring most commonly at the summer and winter solstice, and awakening him from sleep at 1 AM like clockwork. Charles’ pain is like an “auger” in his eye; he paces in circles holding his head on one side, and ends up thrashing on the floor for nearly an hour. Finally he can sleep. 1. Why should Charles not watch Super Bowl football commercials on TV? 2. What type of primary headache does he characterize? 3. What acute and preventative treatments are available for him? 4. Where do the “rhythm” centers that control sleep/wake cycles and circadian rhythms reside in the brain? 886484622 Printed: 6/21/2017 Inflammatory Disease/Infection A 40-year-old male had fever and headache for 3 days. Over the past 24 hours he has had difficulty getting his words out. His family brought him to the ER following a generalized tonic–clonic seizure. On exam he was lethargic and appeared confused. His neck was supple. The optic discs were sharp. There were no focal findings on neurologic examination. His CT scan of the head was unremarkable. CSF studies showed 46 WBC’s (90% lymphs). 356 RBC’s (nontraumatic tap), and a protein of 66. 1. Probable diagnosis? 2. What tests would confirm your diagnosis 3. Discuss management ______________________________________________________________________ A 62 year old college professor is brought to the ER by fire rescue after suffering a grand mal seizure at home. His wife reports that he has been ill for the past 3 days complaining of increasing headache and malaise. She noted that he acted “strange” the day before the seizure and that his memory seemed to be poor, however, she ascribed it to his systemic illness. He remained in bed through the day until the time of the seizure. On examination, he is lethargic and appears incoherent. His temperature is 102.3 and the other vital signs are normal. His general examination is unremarkable. There is mild nuchal rigidity. No focal weakness is detected. His reflexes are quite brisk but no pathological reflexes can be demonstrated. An MRI is done and shows the following: 886484622 Printed: 6/21/2017 1. What is the likely diagnosis? 2. What will you do next to confirm that diagnosis? 3. How will you treat this patient? ______________________________________________________________________ A 35 year-old, previously healthy male, presents to ER with headache, vomiting, and a temperature of 101○. He has not felt well for 3 days. His exam is unremarkable except for fever and resistance to anterior flexion of his neck. I. For each of the following CSF findings give a probable diagnosis 1. protein 65; glucose 48; 75 WBC’s (75% lymphs) 2. protein 120; glucose 22; 1,220 WBC’s (90% polys) 3. protein 155; glucose 35; 120 WBC’s (90% mononuclear cells) 4. protein 48; glucose 70; 65 WBC’s (90% lymphs) 5. protein 75; glucose 68; 85 WBC’s, 850 RBC’s (90% lymphs) 6. protein 200; glucose 15; 65 “bizarre” mononuclear cells II. What is the most common cause of non epidemic encephalitis? 886484622 Printed: 6/21/2017 Movement Disorders A previously healthy 7-year-old male developed quick, jerky movements of both arms over several days. He gradually became irritable and his parents reported he would cry for no apparent reason. His speech became difficult to understand. Examination revealed quick, random movements of both upper extremities that were more pronounced on the right. The movements were worse distally. Speech was slurred and he would occasionally “fling” his right arm. Mild generalized hypotonia was present, and the child could not sustain a fixed posture. 1. What is your diagnosis? 2. What is the mechanism of action of dopamine? 3. What are the signs and symptoms of dopamine toxicity? ______________________________________________________________________ A 7-year-old girl is taken to the pediatrician by her mother. She notes that her daughter has been “moody” and acting “spacey” for the past few days. She also reveals that she has been “fidgety” and had some unusual, seemingly uncontrollable “jerking” movements for the past week. The child is the product of a normal pregnancy and birth. She has achieved all of her developmental milestones on time. She has been healthy although mom recalls her daughter having a sore throat several weeks ago. 1. What test would you perform to help confirm the diagnosis? What else is would you include in the differential diagnosis? 2. Do the movements require treatment? If so, how would you accomplish this? 3. Is any other treatment required? What about long-term treatment? 4. If untreated, what is the most significant long-term complication? ______________________________________________________________________ A 13-year-old female presents to your office with a one-week history of quick, random jerks of her left shoulder. At age 10 years she had been taken to an ophthalmologist for repetitive eye blinking. She was diagnosed as having allergic conjunctivitis and placed on topical steroid drops. The eye blinking responded to treatment over the next several weeks. The following year the child was taken to an allergist for repetitive “sniffing”. She was diagnosed with allergic rhinitis and treated with an antihistamine plus a nasal spray. She improved over the next few months with resolution of her symptoms. During the several months prior to her visit to your office there had been a decline in school performance. Teachers reported poor attention to task and problems concentrating. 1. The child most likely has? 2. Name 3 movement disorders seen in children: 3. What movement disorders begin in childhood? ______________________________________________________________________ 886484622 Printed: 6/21/2017 A 65-year-old retired lawyer was brought in by his wife because of “shaking”. His wife reported that not only did his hands shake, but there had also been a recent “personality” change: the patient had become very “slow” in his movements and often sat motionless with an expressionless face. Examination showed a 4 Hz resting tremor that improved with movement. There was cogwheel rigidity of the limbs and a shuffling gait. The patient was alert and intelligent, and the remainder of the exam was normal. 1. An appropriate differential diagnosis of this case would include: 2. What are the basal ganglia? 3. What is the striatum? 4. What is the lenticular nucleus? ______________________________________________________________________ A 70-year-old retired rehabilitation professor is referred to a neurologist for a right hand tremor that began about 6 months earlier. He complains about “getting older” and losing a lot of his previous energy and motivation. He no longer attends conferences and has laid down a book that he had been actively writing up until a few months ago. He has noticed that his writing is “shrinking” and that his right hand feels clumsy. He has noticed difficulty arising from deep chairs and feels unsteady but has not fallen. He takes no medications, does not abuse tobacco and rarely drinks wine with dinner. He has been healthy throughout life and has no family history of neurological illness. His general exam is normal but he has a slight stoop with no postural instability. He has a ↓ right arm swing while walking, a resting tremor of the right hand that improves with volitional movement, and cogwheel rigidity on the right. 1. Can you anatomically and pharmacologically localize this man’s lesion? 2. What is the most likely diagnosis? What should be considered in the differential? 3. What medications can typically cause symptoms such as this man has? 4. What is the biochemical deficit that causes the majority of this man’s symptoms? How would you treat this man’s symptoms? What can you tell him about the prognosis? 886484622 Printed: 6/21/2017 Neuromuscular Disease A 30 year-old female comes to your office complaining of not feeling well for 6 months. Recently she has had difficulty rising from a chair and climbing stairs. At times she thought she might have had “a little fever”. 1) Your diagnosis? 2) What blood test will support your diagnosis? 3) What will her EMG show (needle electromyography)? 4) How would you treat this lady? ______________________________________________________________________________ A 73 year old man had enjoyed excellent health until the last 2 months. He first noticed difficulties in his golf game, with his fairway drives loosing approximately 25 yards. Within weeks he experienced difficulty arising from the floor, requiring the use of his hands. Recently, he has had to pull himself up from the seat of his car. On neurologic examination he is an excellent historian with good orientation and recall. His cranial nerve examination is normal. He has no discernible sensory deficits. On motor examination his muscle bulk and tone are normal. He has hesitation of hip and back extension when arising from a chair with his arms folded. He is unable to arise from a complete squat, nor is he able to jump off the ground. When arising from the floor, he leads with his buttocks while pushing off with his hands to extend his back and hips. Forced contraction of the biceps is easily broken. Intrinsic hand muscle strength is normal. His tendon reflexes are easily obtained except for relatively depressed ankle reflexes. He has no ataxia. Dx-Polymyositis Questions 1. 1What laboratory tests will directly aid in the diagnosis of his condition? i. CK ii. ANA iii. EMG/NCS iv. Muscle Biopsy 2. Discuss the expected findings for each of these tests. 3. What portion of the general physical exam is particularly important for his diagnosis? i. Skin for extensor surface and periungual rash 4. What criteria are used in the diagnosis of his condition 5. –List potential appropriate initial treatment options Steroids Methotrexate IvIg ______________________________________________________________________ 886484622 Printed: 6/21/2017 A 60 year-old overweight female complains of numbness and tingling in her toes. Her general health is good except for mild hypertension. Her exam reveals a PB of 135/85, mild obesity, and a question of unsteadiness when asked to stand within a narrow base and her eyes closed. The dorsalis pedis and posterior tibial pulses are difficult to palpate. What readily available laboratory test will confirm your diagnosis? What other abnormalities on neurologic examination does this lady have? _______________________________________________________________________ A 13-year-old girl describes a three-month history of difficulty rising to her feet without using her arms, initially for low chairs, and now from any chair. More recently, she started having difficulty holding up her arms to set her hair. Her weakness is symmetric and has not fluctuated. She has no shortness of breath or weakness of head and neck muscles. She does not have pain or sensory disturbance. There is no family history of neurologic or neuromuscular disorders. She has no relevant past medical history, and is taking no medication. On examination, she has normal mental status and cranial nerve function. Muscle bulk and tone are normal. Neck flexor strength is grade 4 over 5. Shoulder abduction is grade 4+ and hip flexion is grade 3+, in a symmetric distribution. She must be helped to a standing position and cannot perform a deep knee bend. Tendon reflexes, plantar responses, and sensory examination are normal. 1. Compare and contrast the signs and symptoms of muscle versus peripheral nerve disease. 2. What is your diagnosis? ______________________________________________________________________ A 55-year-old man suddenly experienced an excruciating, violent, shooting pain along the left side of his face. The pain was so severe that he was forced to stop work. The attack lasted 15-20 seconds, but recurred several times. That evening while you examined him, he again had another attack. You observed lacrimation of the conjunctiva, excess flow of saliva, and palpated a trigger point along the inferior edge of the zygoma. He describes the pain as radiating to the forehead, the eye, and the root of the nose. During the next month these attacks appeared paroxysmally. 1) Discuss your diagnosis 2) Why would Tegretol help this man? ______________________________________________________________________ A 60-year-old male complains of generalized, symmetrical weakness of 3 months duration. On examination, he demonstrates mild generalized weakness that appears more prominent proximally than distally. He seems to become stronger with repeat testing of strength. There is no facial or bulbar weakness. Tone and coordination are normal. Sensation to the primary modalities is normal. Deep tendon reflexes are 886484622 Printed: 6/21/2017 normal and plantar stimulation elicits a flexor response. Nerve conduction studies show a low amplitude compound motor action potential that with repetitive stimulation at 50 Hz shows an incremental response. EMG needle exam of the right leg and paraspinous muscles is normal. 1) What is the difference in botulism and the Lambert-Eaton syndrome at the neuromuscular junction? 2) What is a paraneoplastic syndrome? ______________________________________________________________________ A 40-year-old female with no significant past medical history and who was an adopted child, complains of numbness and tingling sensations in her feet that have been present for 6 months. On exam, she has decreased pin and light touch sensation in a stocking distribution. Motor strength is normal but deep tendon reflexes are depressed with absent ankle jerks. The response to plantar stimulation was weakly flexor. She is on no medications and does not use alcohol. 1) What are the two most common causes of neuropathy in this country? 2) What causes muscle atrophy in neuropathy? ______________________________________________________________________ A 65-year-old male is referred to you by his primary care physician for a few months history of burning feet. It initially bothered him at night bur more recently, it is present almost constantly. The pain involves the entire feet up to the ankles. It is aggravated by physical activity. He also describes decreased sensation in his feet and finger tips. He has no weakness. He occasionally has back pain with no radiation. There is no difficulty controlling his bowel or bladder. He has experienced some lightheadedness with abrupt change of position (mostly from supine to standing). He has a history of coronary artery disease, diabetes and tobacco abuse. There is family history of diabetes and HTN. His exam is remarkable for trace of weakness in both tibialis anteriors, decreased sensation in his feet up to ankles and absent ankle jerks. 1) Localize the site of lesion. 2) Provide a differential diagnosis for this problem. 3) What is your evaluation and treatment plan? ______________________________________________________________________ A 15-year-old female who has been in good health comes to the UK Emergency Room on a Friday evening with complaints of a three-day history of tingling in her legs. Over the last two days, she has become aware of weakness in her legs, which began with frequent tripping and progressed to difficulty getting out of a chair. Today, she feels weak in her hands, complains of aching in her hips and thighs and feels short of breath. She and her mother deny any history of nausea, vomiting, changes in mental status, or cranial nerve symptoms. Her family doctor diagnosed her with a mild viral upper respiratory infection about two weeks ago. On examination, she is symmetrically weak, lower > upper extremities and distal > proximal muscles. Her reflexes are all 886484622 Printed: 6/21/2017 diminished; absent in the knees and ankles. Her sensory exam is normal to all modalities. 1) The most likely diagnosis is? 2) How would you confirm this diagnosis? ______________________________________________________________________ A 7 year-old boy complains of falling and his legs feeling funny. His mother reports his symptoms began about 5 days earlier and seem to be getting worse. On examination he is irritable and uncooperative. Motor and sensory examinations are unreliable. His Achilles reflexes are absent. Which of the following statements concerning this child could be true? 1. 2. 3. 4. He has elevated CSF protein He had gastroenteritis 1 month ago He will develop significant muscle atrophy He could develop respiratory failure Explain why patients with some forms of peripheral neuropathy have slow nerve conduction velocity measurements. ______________________________________________________________________ A 40-year-old man comes to the ER with a complaint of progressive weakness. He had awakened in the morning and on his way to the bathroom, “stumbled”. He also noted some pain in his lower back. Later that morning, he felt clumsy while typing at his computer and subsequently noted some difficulty getting up from his desk chair. A coworker told him he should see a doctor. In the ER, he recalls that besides a recent gastroenteritis, he had felt well and even played basketball and gone out for pizza and beer with his friends. 1) What, if any, is the significance of the previous gastroenteritis? 2) What, if any, is the significance of eating pizza and drinking beer? 3) While examining the patient, he complains of feeling “weaker”. Is there anything in particular that you need to be worried about? 4) What do expect to find on Neurological exam? 5) The patient is admitted to the Neurology Service. What test(s) do you want to perform to confirm your clinical suspicions? What do you expect them to reveal? 6) You make the diagnosis. How would you treat the patient? ______________________________________________________________________ A 30-year-old male complains of easy fatigability and generalized weakness A 48year old man describes double vision, mild arm weakness and rapid fatigability with routine activities. Double vision was noted transiently several weeks ago, but has recently reappeared along with difficulty climbing stairs. His endurance has decreased markedly: he finds it tiring to even chew his food and he thinks he has become more 886484622 Printed: 6/21/2017 liable to choke. He has no pain or sensory loss, takes no medications, and has no family history of neurological illness. His neurological examination is normal except for right eyelid droop and bilateral eye closure weakness. He has normal strength in his shoulders at first, but prominent fatigue after several repetitions of muscle activation. 1) Where would you localize a lesion that leads to fatigable weakness? 2) What electrophysiologic tests are useful in making the diagnosis of this patient’s illness? 3) What laboratory test, if positive, allows for a definitive diagnosis? 4) Describe the pathophysiology of this autoimmune disease. 5) What are current recommended symptomatic and therapeutic treatments. ______________________________________________________________________ A 30-year-old male complains of easy fatigability and generalized weakness, worse in the afternoon. On examination, he demonstrates mild proximal muscle weakness involving the arms and legs. There is mild bifacial muscle weakness and a nasal quality to his voice. Sensory exam and deep tendon reflexes are normal; plantar response is flexor. 1) What serologic studies are potentially abnormal in myasthenia gravis? 2) What are the “classic” electrodiagnostic findings in myasthenia? 3) What is your differential diagnosis in this case? 4) What is your differential diagnosis in this case? ______________________________________________________________________ A 32 year old woman is seen because of weakness and fatigue. She reports feeling so exhausted that she can “barely get through the day”. ______________________________________________________________________ A previously healthy 35 year-old female comes to your office because of intermittent horizontal diplopia of 6 weeks duration. On examination you find only mild ptosis of the right upper eyelid. 1) What would you do next? 2) How does botulinum toxin cause paralysis? 3) How does cobra venom cause paralysis? ______________________________________________________________________ A 62 year-old male comes to your office with a chief complaint of muscle cramps. For the past several months he has had cramping of his calf muscles, thigh and upper arms. He has also noticed occasional “twitching” of muscles in the same areas as the cramping. His general health is good but he reports that he tires easily. On exam both great toes extend with plantar stimulation. His DTRs are brisk but there are no other definite abnormalities. 1) Diagnosis? 886484622 Printed: 6/21/2017 2) What is McArdle’s disease? 3) Who was the “Iron Horse”? 4) What is a fasciculation? 5) What is a fibrillation? ______________________________________________________________________ A 38-year-old female presents to your office with 5 days history of double vision. She first noticed this while driving. She had to close one eye to be able to continue drive home. The symptom has persisted although it may wax and wane at times. She also has difficulty staying awake and “keeping her eyes open” at night. She has felt a “tension” around her eyes but denies having headache, numbness or focal weakness. She has a history of type I diabetes and hypothyroidism. Family history and social history are unremarkable. On exam, she has a mildly convergent gaze with worsening diplopia when looking to the right. 1) Given the finding on the neurological examination, localize the potential sites of the lesion. 2) What is the differential diagnosis for this patient? 3) What is your evaluation and treatment plan? ______________________________________________________________________ A 43-year-old gentleman has been evaluated extensively for two years due to increased ALT and AST, including two liver biopsies, numerous scans and innumerable blood tests – all normal. His doctor checks a CPK, which is found to be 2198 (normal 40-240). In retrospect, he now recalls that at age 8, his father had to drag him several blocks after he became tired walking to Rupp Arena. In high school, though he was a good athlete, he could never run very far before becoming tired, and had to be removed from basketball games frequently from fatigue. As an adult, he recalls three occasions, after strong exercise, his back hurt for a day and his urine “looked like Coca-Cola®”. He runs the treadmill most days. He notices that he feels very fatigued for a few minutes while running at first and then he feels stronger and faster. PE: –General Exam - Normal –Cranial Nerves - Normal –Motor – Normal bulk, tone, strength –Sensation – normal –Cerebellar – normal –DTR 2 and symmetrical, plantar responses are downgoing. –Gait normal. He is easily able to do repeated squats and arise from a chair with his arms crossed. Questions 1. Where would you localize the neurological lesion? 886484622 Printed: 6/21/2017 2. Does this affect your differential diagnosis? 3. What is your differential diagnosis? Labs NCS - Normal EMG - Normal ______________________________________________________________________________ This 47 year old woman awoke with a mild dull ache behind her right ear. When applying make up to her face shortly afterwards, she noted that her face was asymmetric and the right side seemed to be “ironed out”. Facial sensation was normal and there was no limb weakness or other neurological disturbance. She was very concerned that she had suffered a stroke and was taken immediately to the emergency room by her husband. She had no prior history of significant medical illness. Her only hospitalizations were for childbirth. 1. What is the most likely diagnosis? 2. What diagnostic studies, if any, are warranted? 3. How would you treat this lady? 886484622 Printed: 6/21/2017 Spinal Stenosis This 60 year-old gentleman complains of progressive back pain which occasionally radiates to the bilateral hip areas over several years. This is worsened by walking or prolonged sitting. He notices intermittent numbness in the feet for one year. He denies any trauma to the back or change in bladder or bowel habits. Exam • • Neck: supple, nontender Back – Mild midline lumbar tenderness – Bilateral sacroiliac joint tenderness – Slightly limited anterior and posterior ROM – Nontender paraspinous muscles Exam • • • • • Cranial nerves - normal Motor - Moderate weakness right foot dorsiflexion DTR - 2 symmetrical except decreased left ankle jerk. Plantar responses are flexor. Sensation - decreased pinprick medial right foot Gait - leans forward slightly at the waist. Exam 2 • • • • • Laségue’s sign negative Fajerstein test negative Hip rotation test negative Patrick’s test negative Stoop test positive Stoop Test • The "stoop test" has been devised to assess the relationship between claudicationlike symptoms (pain, paresthesia in the lower extremity) elicited while standing versus walking. The test consists of having the patient walk "briskly", while maintaining an upright posture. When the symptoms of claudication become intense, the patient assumes a stooped posture while continuing to walk. The patient is then asked to stop walking and stand upright, at which time the symptoms usually return. The stoop test is considered positive for neurogenic claudication when flexion while walking or stooping relieves the symptoms in the limb. Testing • NCS - Right leg • EMG - Right leg - Normal Normal 886484622 Printed: 6/21/2017 • • • • • • L Ant tib Fibs L Gastroc Normal L Post Tib Fibs L Peron Long Normal L Vastus Lat Normal L L-S Paraspinous Fibs MRI Spinal Stenosis • • “narrowing of the spinal canal sufficient to produce transient vascular compromise of the the neural elements” Frequency* – 13-14% of LBP patients referred to back specialist – 3-4% of LBP patients seen by PCP Spinal Stenosis • • Congenital – Appears usually 30’s - 50’s Acquired – Appears usually 50s - 60’s – Diabetics at 16x risk Spinal Stenosis Anatomic Features • • • Disc protrusion – Canal narrows, nerve compression – Osteophyte growth – Facet arthrosis Ligamentum flavum hypertrophy Spondylolisthesis Symptoms • • • • Back pain 95% Leg pain 71% Weakness 33% Pseudoclaudication 94% Exam • Laségue’s sign usually negative 886484622 Printed: 6/21/2017 • • Sensory changes in the L5(91%), S1(63%) or L1-4(28%) Stoop test – Walk until symptomatic, bending at waist relieves pain Testing • • • • • • EMG/NCV CT/MRI/CT myelography – Normal AP diameter of spinal canal 15 mm – 10-12 mm relative stenosis – <10 mm absolute stenosis Natural History70% remained stable over 4 years 15% improved 15% worsened Progression more likely if – Smaller AP diameter – Bilateral symptoms initially Treatments • • • • • • Regular exercise – pref nonweightbearing Weight loss Management of osteoporosis PT Pain control Surgery • Laminectomy • Spinal fusion • Diskectomy Treatments • • Conservative therapy (nonsurgical) appropriate for mild and moderate symptoms (no diff in early or late surgical treatment groups*) Surgical therapy for – Intractable pain – Significant neurological deficits – Bladder or bowel dysfunction 886484622 Printed: 6/21/2017 Sydenham’s Chorea In the Age of MRI: A Case Report and Review William C. Robertson, Jr, MD and Charles D. Smith, MD A 9-year-old female with acute chorea was found to have multiple areas of abnormal signal on magnetic resonance imaging of the brain consistent with vasculitis. The child’s serology and clinical course were indicative of Sydenham’s corea, and other causes of vasculopathy were excluded. Although magnetic resonance imaging findings similar to those observed in this patient have been rarely reported in Sydenham’s chorea, their presence alone should not prompt a search for an alternative diagnosis. 886484622 Printed: 6/21/2017