Board Review: Neurology

1. Board Review: Neurology Matthew Volk 6/11/2010

2. 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 • 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

5. A few kinds of weakness Guillaume-Barre (AIDP) Myasthenia Gravis NMJ dysfunction Chronic and progressive Worsens with exertion, late in the day Oculomotor first Normal reflexes Sensory not involved • Demyelination • Acute after viral infxn • Worsens over 2-4 weeks then plateaus, resolves • Proximal limbs first • Absent reflexes • Can include sensory

6. 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

7. 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.

8. Question #2

9. Question #2

10. 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

11. 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

12. 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

13. Question #3

14. 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

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

16. 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

17. Normal Pressure Hydrocephalus • Gait Impairment • Cognitive Decline • Urinary Incontinence

18. Some Dementias • Frontotemporal Dementia • Impaired executive function • Preserved visual-spatial function • Lewy Body Dementia • Visual hallucinations • Fluctuating cognition • Parkinsonism

19. Question #4

20. 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

21. 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

22. Question #5

23. 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

24. 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

25. 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

26. Question #6

27. Question #7

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

29. 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%.

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

31. 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

32. Question #8

33. Question #8

34. 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

35. 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.

36. Question #9

37. 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

38. Question #10

39. Question #10

40. Question #10

41. Question #10

42. 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

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

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