AM Report 2/6/17: Neurology Board Review

Normal Pressure Hydrocephalus:
Crude mnemonic: Wet (incontinence), Wobbly (ataxia), and Wacky (dementia)
Abnormal gait typically develops first!
Magnetic gait” – is the characteristic gait of NPH – the patient’s walk appears as if his/her feet are stuck to the floor.
The gait is wide based, with slow, small steps, and reduced foot-to-floor clearance

Visualize hydrocephalus on CT/MRI – specifically ventriculomegaly that is disproportionate to corticol atrophy
picture1

LP: Normal (or slighty elevated) opening pressure and cell count

Standard of care: Ventriculoperitoneal (VP) shut – can perform large volume (30-50 mL) LP prior to placement to verify benefit

picture2

Cluster Headache:
Presentation: Shorter duration (usually < 3 hours), ipsilateral congestion and lacrimation with headache, nocturnal attacks
Epidemiology: Young or middle aged, male sex, history of cigarette smoking
Common trigger: Alcohol

First-line therapy: Inhalation of 100% oxygen and/or SQ or intranasal sumatriptans
Prevention medication: Verapamil

Transverse Myelitis:
TM is thought to be due to an autoimmune inflammation
~50% of the time it is preceded by an infection

Diagnosis requires:
– Presence of clinical features
– Evidence of inflammation (either leukocytosis in CSF or contrast enhancement on MRI)
– Exclusion of other potential causes

Treatment: IV methylprednisolone; plasmapheresis or cyclophosphamide for refractory disease

Alzheimer Disease:
Presentation: >60 years old, decline is insidious and progressive, definite impairment in 2 or more domains of cognition that impacts daily living

Treatment options:
– Cholinesterase inhibitors: Donepezil, Rivastigmine, Galantamine
– N-methyl-D-asapartate receptor antagonists: Memantine
*mild improvements in measured cognition and performance of some activities; no clinically significant outcome

Frontotemproal Dementia:
– 3rd most common type of neurodegenerative disorder (2nd in those < 65 years old); average age of onset is 50-60
– Social behavior / personality changes are variable
– Genetics of FTD are heterogenous ~40% of patients with FTD have at least one family member with a neurodegenerative disorder

Difference with Lewy Body Dementia – Lack of significant psychosis – especially visual hallucinations

Parkinson Disease:
4 main features:
Tremor: “pill-rolling” and always gets better with action
Bradykinesia
Rigidity
Gait/Postural impairment: “festinating gait” – meaning hurried small shuffles, often on tippy toes
* diagnostic criteria require the presence of bradykinesia with at least one other cardinal feature
* MRI is recommended to rule out vascular lesions and hydrocephalus when suspected

Treatment options:
– Non-pharmacologic: rigorous daily exercise
– Pharmacologic: Dopamine substrate (levodopa), Decarboxylase inhibitors (carbidopa), Dopamine agonists (Ropinirole, Bromocriptine, Pergolide), Catechol-O-methyltransferase inhibitors (Entacapone, Tolcapone), Monoamine oxidase type B inhibitors (Selegiline, Rasagiline), Glutamate antagonist (Amatadine), Anticholinergic agents (Benztropine)
– Surgical: Deep brain stimulation (DBS) – consider in patients who have sustained motor benefit from medication, but are limited by adverse effect of medications

Myasthenia Gravis:
Cause: autoimmune disease directed against post-synaptic NM junction
Labs:
Anti-acetylchoine receptor antibodies (~90% sensitive)
– Anti-muscle-specific kinase (MuSK) antibodies
EMG: repetitive nerve stimulation shows decremental response
Imaging: CT or MRI of mediastinum to screen for thymoma (~15% present, 85% hyperplasia)

Treatment:
– Cholinesterase inhibitors: pyridostigmine
– Glucocorticoids
– Immunosuppressive agents: azathioprine, cyclosporine, etc.

Crisis Treatment:
– Plasmapheresis or IVIG

Lambert-Eaton Syndrome:
Cause: autoantibodies against voltage-gated calcium channels located at the presynaptic NM junction
Associations: small cell lung cancer – can occur before tumor has been discovered; found in as many as 60% of patients

Differentiate from MG:
Repetitive contraction IMPROVES muscle strength
– Hyporeflexia and dysautonomia are also present

Treatment:
– Remove the malignancy
– IVIG +/- immunomodulators

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