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Board Review: Neurology Matthew Volk 6/11/2010 Question #1 Guillain-Barre Syndrome Immune-mediated, demyelinating polyneuropathy Proximal and distal weakness – including respiratory failure; Distal sensory loss Autonomic and cranial nerve involvement Most cases triggered by infxn, surgery, or immunization CMV, EBV, HIV, Hepatitis, Lyme, campylobacter jejuni Guillain-Barre Syndrome Treatment Supportive care Follow FVC and NIFs Mechanical ventilation as needed IVIg – avoid in CKD, CHF, IgA deficiency Plasmapheresis – avoid in infxn, low BP No benefit to combination therapy Steroids not shown to help A few kinds of weakness Guillaume-Barre (AIDP) Demyelination Acute after viral infxn Worsens over 2-4 weeks then plateaus, resolves Proximal limbs first Absent reflexes Can include sensory Myasthenia Gravis NMJ dysfunction Chronic and progressive Worsens with exertion, late in the day Oculomotor first Normal reflexes Sensory not involved A few kinds of weakness ALS – chronic progressive, ocular muscle sparing, hyperreflexia and spasticity (UMN disease) Lambert Eaton – chronic but can resolve if malignancy-related, improves with exercise What does this patient have? HPI: 54 y/o F with worsening SOB and inability to swallow x 2 days. Has had fatigue, difficulty keeping eyelids open, intermittent double vision x 1 month. PE: shows bilateral ptosis, mild proximal weakness, normal reflexes. Question #2 Question #2 Acute Ischemic Stroke Inclusion criteria for tPA Age >18 years Clinical diagnosis of ischemic stroke Onset of symptoms within 3 hours of rx CT without evidence of ICH Key exclusion criteria Rapidly improving symptoms Persistent BP > 185/110 Acute Ischemic Stroke Aspirin to reduce rate of recurrent stroke; effect within 2 weeks Subcutaneous heparin to prevent DVT Airway protection/dysphagia screening Blood pressure control in certain cases Maintainence of normothermia Hypothermia not studied in acute stroke Aim for normoglycemia Acute Ischemic Stroke More on Blood Pressure control: Hypertension protective unless extreme Many would not treat unless >220 systolic EXCEPT treat to goal 140-150 with MI, aortic dissection, hemorrhagic conversion Recommended agents Nicardipine, labetalol, nitroprusside Question #3 Parkinsonism Drug-induced Parkinsonism Antiemetics, Antipsychotics, CCBs Reversible with removal of offending agent Neurodegenerative processes Progressive Supranuclear Palsy Multiple System Atrophy Corticobasal Degeneration Huntington’s Disease Parkinsonism Essential Tremor Restless Leg Syndrome Focal/generalized dystonias Cervical dystonia Blepharospasm Oromandibular dystonia Spasmotic dysphonia Ideopathic Parkinson’s Disease Parkinson’s Disease Symptoms: resting tremor, rigidity, bradykinesia, postural instability Treatments Levodopa/carbidopa – older patients Dopamine agonists – young patients Amantadine – mainly works with tremor Anticholinergics – young patients MAO inhibitors – adjunctive therapy Normal Pressure Hydrocephalus Gait Impairment Cognitive Decline Urinary Incontinence Some Dementias Frontotemporal Dementia Impaired executive function Preserved visual-spatial function Lewy Body Dementia Visual hallucinations Fluctuating cognition Parkinsonism Question #4 Multiple Sclerosis Signs and Symptoms – develop over hours to days to years Diplopia or Optic Neuritis Hemiparesis Hemisensory disturbance Band-like sensations around trunk Urinary retention Cognitive decline Multiple Sclerosis Treatment Solumedrol followed by prednisone taper in acute exacerbations Disease-modifying therapy – for relapsingremitting disease Interferon beta (Betaseron, Avonex, Rebif) Glatiramer acetate (MHC interaction) Combination therapy – for progressive dz Combine with cyclophos or Mitoxantrone Question #5 Migraine Headaches Throbbing pain with photophobia and phonophobia. Brainstem involvement results in nausea, pallor, flushing, tearing, rhinorrhea, and sinus congestion. 60-70% with prodrome 24 hr prior 15-25% with aura 1 hr prior Migraine Headaches Treatment NSAIDs – nonspecific; for mild headaches Triptans – direct trigeminal nerve binding; for moderate to severe headaches Contraindicated in CAD Ergot derivatives – hospitalized patients Rescue medications – Haldol, lidocaine, magnesium, dilantin, tizanidine, zyprexa. Opioids can be used occasionally Distinguishing Headaches Migraine/Cluster versus Tension Cause disability versus able to work through them Migraine versus Cluster Stay still versus pace and even strike head >4 hours versus <3 hours Question #6 Question #7 Epilepsy Two or more unprovoked seizures Etiologies: unknown (ideopathic) or focal abn (symptomatic) Vascular malformation Tumor Restricted scar Focal cortical dysgenesis Epilepsy Treatment After first seizure – decision to start treatment is individualized No driving for 6 months to 1 year Risk for recurrence is 30 to 60%. Abnormal EEG indicates higher risk After second seizure recurrance rate is 80 to 90%. Epilepsy Treatment Choice of medication Absence – Ethosuximide GTC – Phenytoin, Carbamazepine, Phenobarbital, Valproate Partial – Gabapentin, Lamotrigine, topiramate, oxcarbazepine Cognitive impairment – Phenobarb, Phenytoin, Carbamazepine, Topiramate Status Epilepticus Secure ABCs – including intubation Ativan 0.1 mg/kg then Phenytoin/phos-phenytoin 18 mg/kg Phenobarbitol 15 mg/kg Pentobarbitol 5-15 mg/kg Question #8 Question #8 Primary CNS Lymphoma Presentation: confusion, lethargy, memory loss, focal neuro signs, and/or seizures Solitary or multiple brain masses Diagnostic evaluation Evaluation for uveitis, retinitis CSF EBV viral load brain biopsy Treat with MTX and whole brain XRT Toxoplasmic Encephalitis Similar presentation to PCNSL Diagnostic criteria Seropositive for Toxo IgG antibody CD4 < 100 and not getting prophy Multiple ring-enhancing lesions on MRI If all three present 90% likelihood Presumptive pyrimethamine/sulfadiazine Otherwise brain biopsy recommended. Question #9 Compressive myelopathy Presentation: Initial spinal or radicular pain Bilateral motor or sensory dysfxn No brain or brainstem findings Evaluate with MRI spine Surgical decompression for epidural abscess and spondylosis Steroids and XRT vs. surgery for epidural tumors Question #10 Question #10 Question #10 Question #10 Viral Encephalitis Symptoms of encephalitis AMS – subtle to unresponsive Usually no meningeal signs Seizures common Focal neurologic findings; abn reflexes CT/MRI Findings VZV, HSV, HHV-6 – temporal lobe West Nile – temporal lobe, basal ganglia, thalamus, brainstem, cerebellum Viral Encephalitis CSF Findings Elevated protein but <150 mg/dl Normal glucose Elevated WBC count but <250/mm3 No red cells except in HSV References MKSAP 14 – Neurology MKSAP 14 – Infectious Disease Uptodate Online