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Seth Woolf, MD Fellow, Pediatric Emergency Medicine July 20, 2016 It’s 0230 on a Saturday morning and the rush has finally slowed down and you want to catch up on some charts A patch comes through to Yale Pediatrics from EMS What’s your patient age/diagnosis? 16yo female with alcohol intoxication, actively vomiting 16yo female with alcohol intoxication, actively vomiting 2yo with croupy cough 16yo female with alcohol intoxication, actively vomiting 2yo with croupy cough 11 month old with “fever” to 1000 F 16yo female with alcohol intoxication, actively vomiting 2yo with croupy cough 11 month old with “fever” to 1000 F 20 month old status-post seizure, now with temperature of 39.20 C …are common! Occur in approximately 2-5% of children Have familial predisposition ◦ 10-20% of patients have a parent or sibling who has had or will have a febrile seizure ◦ Monozygotic twins with much higher concordance rate that dizygotic twins or siblings Large prospective study (Klein et al. Pediatrics. 2010 Jul;126(1):e1-8) and large cohort (MacDonald et al. CMAJ 2014) ◦ Higher risk (twice as high) of febrile seizures for MMRV vs MMR + V 7-10 days post-vaccine Large cohort study (Barlow et al. N Engl J Med. 2001;345(9):656.) showed increased risk of febrile seizures after vaccines ◦ DPT – increased on the day of vaccine (RR = 5.7) DTaP (acellular vs. whole cell) has replaced in US ◦ MMR – increased 8-14 days later (RR = 2.83) “The Rule of 6’s” “The Rule of 6’s” ◦ 6 months – 6 years old Fever Generalized seizure with NO focality ◦ Usually clonic, can be atonic or tonic as well Less than 15 minutes (median duration is 3-4 min.) Only 1 episode per 24 hours Return to normal mental status and neuro exam after postictal period Often the initial sign of infection Those which do not meet simple criteria! Those which do not meet simple criteria! ◦ Focal seizure or focal origin (<5%) ◦ Duration >15 minutes, or multiple seizures in <24 hours (<10%) ◦ Abnormal neuro exam after recovery Todd’s paresis is rare (0.4-2% of cases) ◦ Abnormal mental status after recovery Study by Hesdorffer et al. (Ann Neuro. 2011;70(1):93) – 158 children with 1st FS ◦ Developmental delay and younger age are associated with complex febrile seizures History of afebrile seizures or epilepsy Metabolic abnormalities CNS infection Age <6 months or >6 years (diagnosis of exclusion) CBC? Electolytes with divalents and glucose? CBC? Electrolytes with divalents and glucose? Generally very low yield unless patient has history of vomiting, diarrhea, or abnormal fluid intake AAP recommends to perform LP if: AAP recommends to consider LP if: AAP recommends to perform LP if: ◦ Meningeal signs or other symptoms suggestive of CNS infection AAP recommends to consider LP if: AAP recommends to perform LP if: ◦ Meningeal signs or other symptoms suggestive of CNS infection AAP recommends to consider LP if: ◦ Infants 6-12 months old without immunizations against Hib and strep pneumo ◦ If the patient is on antibiotics Because abx can mask sx of CNS infection …also consider with febrile status epilepticus Continuous seizures or intermittent seizures without neurologic recovery ◦ >30 minutes ◦ Clues: persistently open and deviated eyes if motor activity has stopped ◦ FEBSTAT Prospective multicenter study (Shinnar et al. Neurology. 2008;71(3):170) Clinical setting no different than shorter febrile seizures Higher-than-expected family history of epilepsy In another study by Shinnar, patients found to have a higher prevalence of baseline neuro disease and a personal history of epilepsy when examined prospectively after febrile status epilepticus Not recommended for simple febrile seizure ◦ Not even outpatient MRI Definite indications: ◦ Children with abnormally large heads ◦ A persistently abnormal neurologic examination, particularly with focal features ◦ Signs and symptoms of increased intracranial pressure Teng et al., Pediatrics, February 2006: ◦ Retrospective chart review, 71 patients 51 had a single complex feature (20 focal, 22 multiple, 9 prolonged), 20 had multiple complex features ◦ None had intracranial pathology that required emergent neurosurgical or medical intervention Kimia et al., PEC, April 2012: ◦ Retrospective cohort review, 526 patients with complex febrile seizures, 50.4% had emergent head imaging 4 pts had significant findings (2 ICH, 1 ADEM, 1 focal cerebral edema) 3 out of the 4 had other obvious symptoms (nystagmus, emesis, AMS; hemiparesis; NAT bruises) No patients with multiple sz had intracranial pathology No need for simple febrile seizure Generally not recommended urgently in most circumstances ◦ Some neurologists recommend outpatient if complex Patient with febrile status epilepticus requiring significant medication to stop seizures may need urgent EEG ◦ Usually in ICU, not ED Attending-dependent… Simple febrile seizures almost always do nothing but observe the patient and provide good anticipatory guidance and parental teaching Main goal is to evaluate for source of fever! ◦ Check ears, throat, skin, etc. ◦ Consider urine in patient’s without source Will my child have febrile seizures in the future? ◦ Overall recurrence rate is 30-35% 50-65% if patient was <12 months old 20% in older children Risk Factors: ◦ ◦ ◦ ◦ Young age at onset History of febrile seizures in a first-degree relative Low degree of fever while in the emergency department Brief duration between the onset of fever and the initial seizure Children who had all four factors were much more likely to have a recurrent febrile seizure than were those with none (≥70 versus ≤20 percent). My child had a complex febrile seizure, is he more likely to have another than if it had been a simple febrile seizure? My child had a complex febrile seizure, is he more likely to have another than if it had been a simple febrile seizure? ◦ Nope! If my child has another febrile seizure, when will it happen? If my child has another febrile seizure, when will it happen? ◦ Sooner rather than later… 50-75% within one year Almost all within 2 years Will my child have developmental problems? Will my child have developmental problems? ◦ No greater risk than the general population for either simple or complex febrile seizures No increase in neuro deficits, intellectual impairment or behavioral disorders Longitudinal case-control study (Leaffer et al. Epilepsy Behav. 2013 Jul;28(1):83-7) Population-based studies – National Collaborative Perinatal Project (NCPP) in US and one in UK and Denmark No differences in outcomes in patients with complex febrile seizures, unless they developed afebrile seizures Is my child more likely to develop epilepsy? Is my child more likely to develop epilepsy? ◦ Yes Is my child more likely to develop epilepsy? ◦ Yes Simple febrile seizure doubles risk from 0.5% to 1% Complex febrile seizures can increase risk more depending on how many and which factors As high as 49% if complex with all 3 features Should my child take seizure medications? Should my child take seizure medications? ◦ No. ◦ AEDs may decrease the risk of recurrent febrile seizures, but the prevention of these benign events is generally not considered worth the potential adverse effects of treatment. ◦ The use of AED prophylaxis in children with complex febrile seizures is individualized based upon underlying risk factors – no general consensus. Can giving Tylenol or Motrin to my child anytime he is sick prevent febrile seizures? Can giving Tylenol or Motrin to my child anytime he is sick prevent febrile seizures? ◦ No. ◦ RCT showed that patients mount fevers despite antipyretics Risk of febrile seizure does not change whether or not the fever is treated ◦ Treat fevers as with any child. Definition ◦ Acute neurologic conditions which may lead to death or permanent disability if not recognized and treated Quick neuro exam for signs of herniation syndromes, localization, and nature of injury/disease Pace of symptom evolution “Brain failure” degree: GCS Survey history for clues to the category of disease process Secure ABC(D) Recognize intracranial hypertension, herniation Prevent secondary injury • C-spine in trauma • Sedation effects • Maintain oxygen delivery • Treat/avoid hyperthermia • Treat/prevent seizures “Where is the lesion?” Psyche Brain Spinal Cord Spinal root Nerve plexus Peripheral nerve N-M Junction Muscle What kinds of diseases cause lesions in that location? Outside the nervous system “What caused the lesion?” Trauma Infection Inflammation Intoxication Neoplasm Metabolic (endocrine, nutritional) Vascular, cardiovasc. Epilepsy Congenital malformation Degenerative “Where is the lesion?” Psyche Brain Spinal Cord Spinal root Nerve plexus Peripheral nerve N-M Junction Muscle Outside the nervous system Signs & symptoms Behavior Mood Personality Thought disorder Social function “Where is the lesion?” Psyche Brain Spinal Cord Spinal root Nerve plexus Peripheral nerve N-M Junction Muscle Outside the nervous system Signs & symptoms Supratentorial: seizures, mental status, special senses, language, motor or sensory deficit in cortical distribution, with upper motor neuron features, involuntary movements Infratentorial: Cranial nerve deficits (e.g. diplopia, pupillary defects, oculomotor paresis, nystagmus, vertigo) Cerebellar ataxia “Where is the lesion?” Psyche Brain Spinal Cord Spinal root Nerve plexus Peripheral nerve N-M Junction Muscle Signs & symptoms No symptoms “above the neck” Weakness, bilateral & segmental Sensory level Dropped reflexes at the level of lesion Increased reflexes below lesion Sphincter dysfunction Pain is common Outside the nervous system “Where is the lesion?” Psyche Brain Spinal Cord Spinal root Nerve plexus Peripheral nerve N-M Junction Muscle Signs & symptoms No symptoms “above the neck” Sensory &/or motor deficit is segmental Dropped reflexes at the level of lesion No long track signs No/little sphincter dysfunction Pain is common Outside the nervous system “Where is the lesion?” Psyche Brain Spinal Cord Spinal root Nerve plexus Peripheral nerve N-M Junction Muscle Outside the nervous system Signs & symptoms Sensory &/or motor deficit fits type & location of nerves involved e.g. pure motor (flaccid weakness), mixed sensorimotor, small fiber sensory, length-dependent or stocking glove Dropped reflexes at the level of lesion No long track signs No/little sphincter dysfunction Pain is common “Where is the lesion?” Psyche Brain Spinal Cord Spinal root Nerve plexus Peripheral nerve N-M Junction Muscle Outside the nervous system Signs & symptoms Pure motor deficit (flaccid) in characteristic pattern Reflexes present but hypoactive No long track signs No/little sphincter dysfunction 14 year old male Hit in head by swung baseball bat at practice No helmet +LOC x ~1 min, back to baseline soon after, but now more lethargic Vitals stable On exam ◦ PERRL, GCS 13 (confused, localizes to pain) ◦ Has large temporal hematoma and palpable depression in skull ◦ No neuro deficits appreciated Primary Goal ◦ Determine severity of the injury and identify ciTBI Epidemiology ◦ >600,000 annual visits per year (birth to 19) ◦ ciTBI incidence ranges from 0.02-4.4% ◦ TBI is leading cause of acquired disability in children TBI is a spectrum of illness ◦ Minor head injury concussion skull fracture pneumocephalus intracranial hematoma cerebral edema DAI cerebral herniation death Identification of children at very low risk of clinically-important brain injuries after head trauma: a prospective cohort study Kuppermann et al (PECARN study) Lancet 2009; 374: 1160-70 Obtaining CT exams must balance the importance of identifying significant injuries and illnesses with the risks of CT Cancer risk ◦ 10-fold increase in neoplastic potential in children compared to adults ◦ Children have longer lifetime during which radiation-related cancers may evolve ◦ ~1/1000 children who have had a CT scan will develop a radiation-induced fatal cancer in their lifetime 0.35% increase over expected baseline lifetime-risk Age, Years <2 Clinical Criteria Normal mental status Normal behavior per routine caregiver No LOC (or <5 sec with low-risk mech.) No severe mechanism of injury No nonfrontal scalp hematoma No evidence of skull fracture 2-18 Normal mental status No LOC No severe mechanism of injury No vomiting No severe headache No signs of basilar skull fracture Children <2 years old Children 2-18 years old Altered mental status or abnormal behavior per caregiver Altered mental status Nonfrontal location of scalp hematoma Depressed or basilar skull fracture LOC >5 seconds Post-traumatic seizure Depressed or basilar skull fracture LOC Bulging anterior fontanelle Focal neurologic findings Persistent vomiting Worsening severe headache Post-traumatic seizure Focal neurologic findings Suspicion of non-accidental trauma Diffuse axonal injury (DAI) ◦ Acceleration/deceleration or rotational forces ◦ Shear injuries of axons and blood vessels involving white matter Cerebral edema ◦ Direct insult to neurons Local release of inflammatory mediators Vascular leakage Hypoxemic changes Bone involvement: ◦ Parietal > Occipital > Temporal Most common causes: ◦ Falls – 35% (most common <2yo) ◦ Recreational Activities – 29% ◦ MVC – 24% Unilateral, linear (account for 75% of pediatric skull fractures) Incidence of underlying intracranial injury ranges from 15-30% ◦ More common in complicated fractures Predictive of skull fractures in infants <1yo Clinical significance of scalp abnormalities in asymptomatic head-injured infants. Greenes & Schutzman, PEC 2001; 17:88 Prospective study, 422 asymptomatic infants Hematomas in parietal (OR 38) or temporal (OR 16) regions increased likelihood of underlying skull fracture Hematomas in frontal region did not (OR 0.6) Risk of skull fracture increases with size of hematoma No underlying intracranial injury No distracting traumatic injury Normal neurologic exam No concern for NAT Reliable caretakers Neurologically intact children with an isolated skull fracture may be safely discharged after brief observation Rollins et al., J Pediatr Surg 2011; 46:1342. Retrospective review of 235 previously healthy children, median age 11mos 0/58 patients d/c’d from ED after obs (avg 3 hours) returned for further care 177 hospitalized – avg LOS 18 hours ◦ 5 returned for persistence of symptoms ◦ 1 had delayed onset of an extraaxial intracranial hemorrhage Management of uncomplicated skull fractures in children: is hospital admission necessary? Vogelbaum et al, Pediatr Neurosurg 1998; 29:96 Prospective study of 44 pediatric patients Average age 1.8yo No clinical complications in any admitted patients Infants with isolated skull fracture: what are their clinical characteristics, and do they require hospitalization? Greenes & Schutzman, Ann Emerg Med 1997; 30:253 Retrospective study of 101 patients <2yo None had a decline in clinical status during hospitalization Complicated fractures Basilar skull fractures Open skull fractures Fractures associated with intracranial injury 80% contain at least one of 6 classic physical examination findings ◦ Subcutaneous bleeding over mastoid process Battle Sign ◦ Subcutaneous bleeding around the orbit ◦ ◦ ◦ ◦ Raccoon Eyes Hemotympanum CSF rhinorrhea CSF otorrhea Cranial nerve deficits Facial paralysis, anosmia, nausea, vomiting, vertigo, nystagmus, tinnitus, hearing loss Not recommended initially for basilar skull fractures with non-penetrating trauma ◦ ASHP guidelines ◦ Einhorn and Mizrahi (Am J Dis Child. 1978;132(11):1121) Risk of meningitis is significantly increased if CSF leak >7 days ◦ Observational studies in children and adults Use is generally recommended in patients with open skull fractures to prevent osteomyelitis (anti-Staph) 8 year old male previously healthy Found seizing at home in bed No reports of previous headache, fever, or focal neuro deficits Presented to OSH ◦ ◦ ◦ ◦ ◦ Stabilized, intubated Transport – pupils fixed and dilated On arrival here, GCS 3, pupils same Admitted to PICU, OR for repair Declared brain dead, care withdrawn POD#1 Subfalcine ◦ Cingulate Central Uncal ◦ Transtenorial Tonsillar Localized mass effect Compression of: - other structures - vessels - CSF outflow routes Secondary injury: - ischemia - direct compression - stretch Central: Uncal: generalized hemisphere edema, acute hydro subdural, epidural, contusion midbrain & bilateral 3rd n. , aqueduct, post cerebral art. unilateral midbrain, unilateral 3rd n., aqueduct basilar art. stretch - pons basilar art stretch - pons & medulla ischemia & medulla ischemia Disrupted brainstem = decorticate posturing, respiratory depression, bradycardia, pupil dilation, lethargy, death Presentations differ depending on age Infants ◦ Poor feeding, vomiting, irritability, bulging fontanelle, altered mental status Children ◦ Headache, vomiting, visual changes, neck stiffness, focal neuro findings, seizure, altered mental status, lethargy, obtundation Cushing’s Triad ◦ Bradycardia, hypertension, respiratory depression Vascular abnormalities ◦ Fistulous connection of arteries and veins without normal intervening capillary bed Nearly all thought to be congenital Most commonly supratentorial (90%) Most common presentation is intracerebral hemorrhage Other presentations ◦ Headache ◦ Focal neurologic deficits Size of AVM not predictive of future hemorrhage ◦ Series of 168 patients Diagnostic imaging ◦ CT scan: good initial tool, especially in case of acute hemorrhage ◦ MRI with or without angiography (MRA) Superior to CT in unruptured AVMs Delineates details of AVM architecture Sensitive in revealing subacute hemorrhage Neurosurgical involvement immediately Surgical resection ◦ ◦ ◦ ◦ ◦ Major hemorrhage Progressive neurologic deterioration Inadequately controlled seizures Intractable headache Venous restrictive disease Overall mortality rate of AVM hemorrhage is 10% ◦ Certain locations such as basal ganglia and thalamus carry mortality rate of 42.9% ABCs ◦ Consider atropine in children <1 year to blunt vagal response to intubation ◦ Lidocaine at 1-2 mg/kg to prevent potential increased ICP by blunting airway reflexes Elevate head of bed Maintain C-spine immobilization Ventilation to maintain PaCO2 at 35-40 mmHg Continuous sedation to prevent agitation Hypertonic saline (6-10 mL/kg boluses) Mannitol (1 gram/kg) 4 year old male presents to ED with wobbly gait Parents admit persistence of symptoms for one month, but worsening over past few days Random episodes of emesis, worse in morning Physical exam ◦ ◦ ◦ ◦ Vitals normal EOMI, but boy blinks several times on testing Normal motor and sensory exam Wide-based gait with difficulty turning corners Enlarged Ventricles Mass MRI brain and spine with and without contrast ◦ Once that is done, then… Neurosurgical consult Pre-op labs ICP management Discuss with NES, oncology about Decadron Admission to ICU and preparation for resection likely with placement of ventriculostomy Obstructive ◦ Stenosis of cerebral aqueduct Congenital, tumor, following hemorrhage or infection ◦ Posterior fossa tumors, arachnoid cysts, congenital malformations (e.g. Dandy-Walker) Non-obstructive (communicating) ◦ As a result of scarring following intraventricular hemorrhage (IVH) in premature infants ◦ Following meningitis ◦ Congenital myelomeningocele ◦ Post-traumatic Clinical Recognition ◦ Infants: macrocephaly, sunsetting (downward eye deviation, lid retraction, convergence-retraction nystagmus) ◦ Older children: visual field deficits or double vision (from compression of optic chiasm or abducens nerve palsy) Diagnosis ◦ CT (initial test) or MRI (provides greater anatomical detail, also better for tumors) Treatment ◦ Airway and cardiorespiratory maintenance ◦ Neurosurgical consultation for definitive management Acute Subacute or Chronic Nausea Change in behavior Vomiting Neuropsychological signs Irritability Change in feeding patterns Seizures Developmental delay Headache Change in school performance Lethargy Change in attention span Coma Daily headaches Stupor Increase in head size Diagnostic imaging ◦ Shunt series (x-rays) ◦ Quick brain MRI (to compare size of ventricles to previous studies) Radiographic changes in asymptomatic patients with changes in physical exam (e.g. papilledema, increase in head circumference) ◦ Urgent neurosurgery consult Radiographic changes and symptomatic ◦ Emergent neurosurgery consult • • • • Previously healthy nine year old boy is walking down the hallway at school He feels like he can’t take the next step and falls down Brought by ambulance to hospital Physical Exam • Blood Pressure 150/90 • Patient seems unable to follow commands • Mild right facial droop • 5/5 strength LUE and LLE, and 3/5 strength RUE and RLE CT 20 hrs after onset shows L basal ganglia infarct Confirmed on MRI @ 24 hrs by axial T2 (left) and DWI (right) Relatively rare in children Classified as either ischemic or hemorrhagic At risk populations ◦ Sickle cell disease (6-9% incidence) ◦ Cardiac disease (valvular disease, endocarditis, arrhythmias, etc.) ◦ Metabolic conditions (homocystinuria, organic acidemias) ◦ Coagulopathies (e.g. hemophilia, Factor V Leiden, ATIII, protein C & S def.) ◦ Neurocutaenous syndromes (neurofibromatosis, tuberous sclerosis) ◦ Structural arterial disease (e.g. moyamoya) Unlike adults, there is currently no consensus on primary treatment for acute stroke in childhood Stabilization and prevention of secondary neuronal injury ◦ Normotension, normothermia, euglycemia, avoid hypoxia ◦ Treat hypertension cautiously (maintain cerebral perfusion) Initiate anti-coagulation therapy ◦ Aspirin, LMWH, or unfractionated heparin ◦ Thrombolytic therapy: safety and dose of alteplase for the treatment of children with arterial ischemic stroke have not been established (not FDA-approved for children <18) Hemorrhagic stroke ◦ Involvement of neurosurgery Can present with NO symptoms, mild symptoms like headache and vomiting, or severe symptoms like acute mental status changes, seizures, hemiparesis, CN deficits Clinical history may reveal dehydration, mastoiditis/otitis media, hypercoagulable state, inflammatory conditions (SLE, UC) Management ◦ ABCs, hydration ◦ Consider anticoagulation: LMWH or heparin drip ◦ IV antibiotics if underlying infection (mastoiditis) 11 year old male noted his arms were heavy when he boarded the bus for school On arrival to school, he collapsed on trying to stand and was unable to walk 2 episodes of urinary incontinence No fevers or sick contacts Physical Exam Mental status and CN intact Motor: normal bulk; spasticity legs > arms and left side > right side Decreased rectal tone Strength 2/5 in LUE and LLE, 3/5 in RUE and 2-3/5 in RLE Sensory: decreased in all modalities distal greater than proximal from C5 down ◦ Reflexes: 4+ at left ankle, 3+ in other lower extremities, 3+ upper extremities ◦ ◦ ◦ ◦ ◦ Sensory level and/or motor level and decreased rectal tone indicate spinal pathology Imaging: STAT MRI spine (want several segments above and below area to which lesion localized) Monitor respiratory and cardiac function very closely ◦ MD should accompany patient to imaging T2 Hyperintensity From C3-5 Trauma: MVCs, diving, sports, traumatic delivery, be aware of SCIWORA Infection/Parainfectious: usually febrile, local bone pain in diskitis, epidural abscess, TB, vertebral osteomyelitis, HIV Tumor: subacute or chronic symptoms/deficits and back pain, primary intrinsic neuroepithelial tumors, Schwannomas in NF, metastatic tumors Vascular/hemorrhagic: AVM, post-LP, intramedullary tumor, cord infarction Demyelinating: Transverse myelitis, ADEM, Multiple sclerosis Anatomic: Tethered cord, Chiari malformations, myelomeningoceles, atlanto-axial dislocations Rare, acquired autoimmune disorder Demyelination and inflammation in spinothalamic and pyramidal tracts, posterior columns Involves both halves of spinal cord over variable length May be post-infectious/idiopathic (~2/3) ◦ EBV, CMV, measles, mumps, Campylobacter, Mycoplasma May be associated with systemic illness (~1/3) ◦ SLE, scleroderma, multiple sclerosis Presentation can be acute or subacute Most common location is thoracic, but can occur anywhere Treatment ◦ ◦ ◦ ◦ ◦ Supportive 80% have some degree of recovery All children should be hospitalized Systemic corticosteroids (no RCTs, just consensus) Insufficient evidence to support IVIG Typically back pain precedes other symptoms ◦ Fever, headache, vomiting, spinal compression sxs Bony tenderness may indicate vertebral osteomyelitis or discitis Treatment ◦ Emergent surgical decompression ◦ Start antibiotics (anti-Staph aureus) Vancomycin along with 3rd or 4th generation cephalosporin Etiologies ◦ ◦ ◦ ◦ ◦ Congenital and acquired bleeding disorders Hemorrhagic tumors Spinal AVMs Instances of increased ICP Trauma MRI of spine is definitive diagnostic measure Decompression is key procedure to improve outcomes If no mass lesion: Lumbar puncture performed for cell count, gram stain/culture, protein, glucose, viral studies (myelin basic protein, oligoclonal bands, protein electrophoresis if white matter lesion) MRI brain with contrast to rule-out ADEM, look for lesions (suspicious for multiple sclerosis) – nonemergent Basic rheumatology labs (eg ANA, CRP, ESR, antiphospholipid antibodies) 4 year old male with no PMH presents to ED with inability to walk up stairs Mild URI symptoms for 2 days Physical exam ◦ ◦ ◦ ◦ Moderate respiratory distress, minimal effort Bilateral ptosis, uncomfortable with EOM Motor testing: proximal muscle weakness Strength decreases on repeated attempts at activity 10 year old male with no PMH presents with bilateral lower extremity pain and difficulty walking over past 2 days No bowel or bladder incontinence, no fevers Mother reports AGE 3 weeks prior Physical exam ◦ Motor testing: 2/5 strength in bilateral lower extremities, 4+/5 in bilateral upper extremities ◦ Reflexes: diminished at ankle and patellar ◦ Sensory: hyperesthesia to feet ◦ CNs intact, rectal tone normal 4 month old female ex-full term, no PMH, presenting with 3 days of poor breast latching and hypotonia Parents report no bowel movements over past 4 days (usually goes every day) No fevers Father is farmer Physical exam ◦ Mild hypotonicity ◦ Poor suck and gag reflexes ◦ Bilateral ptosis Anterior Horn Cell Peripheral Nerve Neuromuscular Junction Muscle ◦ Poliomyelitis, postasthmatic amyotrophy, SMA, amyotrophic lateral sclerosis ◦ Guillain-Barré syndrome, Erb/Klumpke palsy, heavy metal poisoning, toxins/ingestions, marine toxins, acute intermittent porphyria ◦ Botulism, myasthenia gravis, tick paralysis, organophosphates, neuromuscular blockers, snake envenomations ◦ Muscular and myotonic dystrophies, dermatomyositis, infectious (e.g. viral), metabolic abnormalities, periodic paralysis, rhabdomyolysis/myoglobinuria, endocrine disorders, steroid myopathy Acute management ◦ ◦ ◦ ◦ Cardiopulmonary monitoring Get vital capacity, negative inspiratory force CXR Emergent neurology consult EMG and Tensilon testing (have atropine at bedside) Decision on treatment (pyridostigmine (Mestinon)) ◦ If intubation required, avoid succinylcholine Have anesthesia available ◦ If patient rapidly deteriorating, other therapies include prednisone, IVIG, plasmapheresis ◦ Admission to PICU Failure of neuromuscular transmission due to antibodies against acetylcholine receptor at postsynaptic NM junction 3 forms in children ◦ Transient neonatal ◦ Infantile (congenital) ◦ Juvenile (most common – mimics adult disease) Has a fluctuating, non-predictable course ◦ Exacerbated by illnesses and certain drugs (e.g. aminoglycosides) ◦ Rapid worsening of symptoms and resp compromise (myasthenic crises) may be difficult to differentiate from cholinergic crises (overdose of anticholinesterases) Acute management ◦ Cardiopulmonary monitoring Autonomic dysfunction common ◦ Vital capacity, Negative inspiratory force ◦ Emergent neurology consult ◦ Admission for all patients with suspected GBS Chance of progression to respiratory failure (10-20%) ◦ LP Elevated protein, <10 WBCs, glucose normal ◦ Consider MRI - enhancement of spinal nerve roots ◦ IVIG or plasma exchange Primary demyelinating disorder causing symmetric ascending paralysis Autoimmune phenomenon Antecedent respiratory or GI illness ◦ 75% of childhood cases Weakness is commonly insidious Paresthesias/sensory complaints occur in 50% DTRs diminished or absent CN abnormalities occur in 30-40% of cases Miller Fisher variant ◦ Oculomotor palsies, ataxia, areflexia without motor extremity weakness Acute management ◦ Cardiopulmonary monitoring ◦ Rule-out other causes (consider LP, head/spine imaging) ◦ Admission for close observation for respiratory compromise (77% required intubation in a large series of 57 patients), NG tube feeds Think of botulism in an infant with worsening lethargy, feeding difficulties and constipation Infants usually <6 months old Intestinal colonization by Clostridium botulinum ◦ Honey ◦ Spores found in soil and agricultural products Diagnosis: C. botulinum toxin (A or B) in stool Treatment ◦ Human botulism immunoglobulin (BabyBIG) Trials have shown a decrease in duration of illness ◦ No evidence to support use of antibiotics, cathartics or other laxatives Neurological and neurosurgical emergencies are frequent causes of pediatric ER visits A good history and the temporal pattern of the symptoms often aid in diagnosis A good neurological examination helps to localize the problem and guide further testing and management Recognition and treatment in the ER may improve outcomes for these children