Board Review: Neurology

Matthew Volk6/11/2010

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

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

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

relapsing-remitting 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