Board Review: Neurology

1. Board Review: Neurology Matthew Volk 6/5/2009

2. MKSAP Question #1

3. 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

4. Guillain-Barre Syndrome • Treatment • Supportive care • Follow FVC and NIFs • Mechanic 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

5. MKSAP Question #2

6. MKSAP Question #2

7. 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

8. 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

9. 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

10. MKSAP Question #3

11. 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

12. Parkinsonism • Essential Tremor • Restless Leg Syndrome • Focal/generalized dystonias • Cervical dystonia • Blepharospasm • Oromandibular dystonia • Spasmotic dysphonia • Ideopathic Parkinson’s Disease

13. 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

14. MKSAP Question #4

15. 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

16. Multiple Sclerosis • Treatment • Solumedrol followed by prednisone taper in acute exacerbations • Disease-modifying therapy – for relapsing-remitting disease • Interferon beta (Betaseron, Avonex, Rebif) • Glatiramer acetate (MHC interaction) • Combination therapy – for progressive dz • Combine with cyclophos or Mitoxantrone

17. MKSAP Question #5

18. 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

19. 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

20. MKSAP Question #6

21. MKSAP Question #7

22. Epilepsy • Two or more unprovoked seizures • Etiologies: unknown (ideopathic) or focal abn (symptomatic) • Vascular malformation • Tumor • Restricted scar • Focal cortical dysgenesis

23. 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%.

24. Epilepsy Treatment • Choice of medication • Absence – Ethosuximide • GTC – Phenytoin, Carbamazepine, Phenobarbital, Valproate • Partial – Gabapentin, Lamotrigine, topiramaate, oxcarbazepine • Cognitive impairment – Phenobarb, Phenytoin, Carbamazepine, Topiramate

25. 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

26. MKSAP Question #8

27. MKSAP Question #8

28. 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

29. 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.

30. MKSAP Question #9

31. 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

32. MKSAP Question #10

33. MKSAP Question #10

34. MKSAP Question #10

35. MKSAP Question #10

36. 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

37. Viral Encephalitis • CSF Findings • Elevated protein but <150 mg/dl • Normal glucose • Elevated WBC count but <250/mm3 • No red cells except in HSV

38. References • MKSAP 14 – Neurology • MKSAP 14 – Infectious Disease • Uptodate Online