Day 2 - Neuro3 Degenerative, PNS/Neuromuscular, (Neoplasms)

Question 1

Med for Parkinson’s sx

Answer 1

Levidopa/Carbidopa (replace dopamine, reduce decarboxylation) (2) Selegiline - selective MAOi type B, early disease, neuroprotective effects (3) Bromocriptine - ergot dopamine agonists (4) Pramiprexole, Ropinirole, Rotigotine - non-ergot dopamine agonists; Apomorphine can be used for sudden akinetic episodes (5) Entacapone, Tolcapone - COMT inhibitors - potentiate levodopa (6) Benztropine & Trihexyphenidyl - Anticholinergics for tremor (7) Amantadine - increase dopamine release, short-term monotherapy in mild disease (usually young patients)

Question 2

Feat. of ALS aka Lou Gehrig’s

Answer 2

Weakness w/ normal sensation; Asymmetric limb weakness (hands/fingers, foot drop, shoulder girdle, weakness of pelvic girdle); Bulbar UMN sx (e.g., Dysarthria/Dysphagia, Pseudobulbar affect - inappropriate laughter, crying, yawning); UMN - spasticity, slowness, movement stiffness, incoordination, hyperreflexia (i.e., spastic paralysis); Slowed RAMs; Gait disorder, LMN - weakness, gait disturbance, slowed reflexes, muscle atrophy, fasciculations; Cognitive defects - frontotemporal executive dysfunction; Neuromuscular respiratory failure after months to years (survival time from dx 3-5 years)

Question 3

Hungtington’s C’s

Answer 3

CAG repeat disorder, Chromosome cuatro (4), Caudate and putamen atrophy, ACh decrease, Crazy, Cuarenta (40 -age of onset), Choreiform movement

Question 4

Unique feat of dementia due to Pick’s disease

Answer 4

Behavior & personality changes (inappropriate social/personal conduct); Progressive aphasia or dysarthria

Question 5

Unique feat of Lewy body dementia

Answer 5

Dementia, Parkinsonian features (bradykinesia, shuffling gait, cogwheel rigidity), Visual hallucinations; Repeated unexpected falls (syncopal episodes)

Question 6

Dementia w/u

Answer 6

MMSE; CBC (r/o pathologies responsible for long-term delirium), U/A (UTI or meds like anticholinergics common causes of confusion in elderly), Lytes, BUN/Cr, LFTs, Ca/Mg, Phosphate, Syphilis, HIV, TSH, B12, Cranial CT or MRI (MRI better due to see brain parenchyma better); If all normal, then Alzheimer disease dx of exclusion

Question 7

Bells palsy dx requirements

Answer 7

Clincal dx: entire CN VII involvement - upper and lower face; (recall: both sides of brain innervate upper face, only one side of cortex innervate lower face - Stroke ~ only lower face paralysis)

Question 8

Important conditions (& methods) to r/o in case of Bells palsy

Answer 8

Lyme (history); Ottis Media (TM); Stroke (neuro. exam)

Question 9

Timeline of Bell’s palsy

Answer 9

Acute onset, comes on in 1-2 days; Progressive weakness for 3 weeks, recovery w/i 6 months;Anything other than above, requires CT & screening blood tests

Question 10

Tx Bell’s palsy

Answer 10

(1) Eye protectant: Artifical teas, lubricating ointment, patch covering eye (2) Steroids (~ 1wk) (3) Possibly Valacyclovir (NOT Acyclovir = no additional benefit over steroids)

Question 11

Guillain-Barre p/w

Answer 11

Symmetric muscle weakness over days to wks (~2 weeks), progressive; usually begins proximal legs, rarely start in arms or facial muscles; Ascending paralysis; Respiratory muscle paralysis requiring mechanical ventilation in 30% of cases; Facial muscle weakness &/or oropharyngeal weakness (~ b/l facial muscle paralysis); B/l facial muscle paralysis ddx: Guillain-Barre or Lyme disease; Autonomic dysfunction in 70% (tachycardia); Absent or depressed DTRs; Little, if any change in sensation; No fever at presentation; History of preceding Campylobacter jejuni infx in 20%, HIV, CMV/EBV, Mycoplasma, or other viral infx or immunizations (by up to couple of months)

Question 12

B/l Bell’s palsy (facial paralysis) ddx

Answer 12

Guillain-Barre

Question 13

DDx

Answer 13

Clincial presentation; CSF analysis - Albuminocytologic dissociation (elevated protein but normal WBC); Electrodiagnostic studies: EMG - demyelination

Question 14

Prognosis of Guillain-Barre

Answer 14

Spontaneous regression and complete recovery w/i 1 year in 80-90%; Relapse in 10%; Delayed or incomplete recovery in 5-10%; Death despite ICU care 5%

Question 15

Tx Guillain-Barre

Answer 15

Hospitalization for respiratory monitoring, incl. vital capacity, blood pressure monitoring, cardiac monitoring, daily abdominal auscultation for ileus (autonomic dysfunction); Mechanical ventilation in 30% of patients, impending respiratory failure; ICU monitoring for autonomic dysfunction required in 20% of pts; PLASMAPHARESIS OR IVIg (equally effective at shortening time to walking, but no added benefit); STEROIDS NOT RECOMMENDED

Question 16

Cause of slowing of nerve conduction velocity

Answer 16

Demyelination

Question 17

EMG w/ fasciculations and fibrillations at rest

Answer 17

LMN lesion

Question 18

EMG w/ silent (no muscle activity) at rest & decrease amplitude on muscle contraction

Answer 18

Intrinsic muscle disease (nerve signal gets there, but muscle not strong enough - e.g., myositis, muscular dystrophy)