Neuro Potpourri

Question 1

Name the 3 elements associated with peripheral neuropathy? Which is most common?

Answer 1

Sensory - most common
Motor
Autonomic

Question 2

Name 2 hereditary causes of PN

Answer 2

Charcot-Marie Tooth disease (M.C hereditary PN)

Porphyria

Question 3

What are some acquired causes of PN?

Answer 3

• Inflammatory (GBS)
• Infections (Lyme’s dz)
• Rheumatic (many! associated with vasculitis)
• Organ failure (esp renal failure)
• Endocrine (DM, hypothyroid)
• Vitamin deficiency (B12, B1…_
• Medications/Toxins

Question 4

What diagnostic test would help you differentiate bw nerve and muscle disorders?

Answer 4

electromyography (EMG)

Question 5

Charcot-Marie-Tooth disease background info

Answer 5

syndrome of many distinct d/o, CMT1 the MC
demyelination d/o
usu 1st -3rd decade of life, many asymptomatic
progressive motor and sensory loss of UE and LE

Question 6

What are some clinical presentations of Charcot-Marie-Tooth disease

Answer 6

distal leg weakness (footdrop) 
foot deformities (pes cavus, hammer toes) 
muscle atrophy below the knee
reduced or absent DTRs 
sensory deficits

Question 7

Porphyria

background info

Answer 7

Autosomal dominant inheritance

metabolic d/o caused by enzyme deficiency in the HEME biosynthetic pathway

attacks brought on by certain medications, hormonal changes, dietary restrictions

Question 8

Presentation of porphyria

Answer 8

sharp abd pain, followed by agitation, hallucinations, or seizures, days later.. extremity pain followed by weakness

Question 9

most common PN in developed countries?

Answer 9

Diabetic neuropathy - distal symmetric sensory or sensorimotor PN

may also have autonomic sxs

Question 10

Lyme disease

Answer 10

spirochete infection, Borrelia burgdoferi

transmitted by deer tick

neuro complications occur in 2nd and 3rd stages of infections (facial neuropathy most common - Bells Palsey Ddx)

Question 11

Cobalamin deficiency (B12) deficiency etiologies

Answer 11

pernicious anemia 
Diet deficiency 
Gastric bypass 
IBD 
Bacterial overgrowth 
Medication related (H2 blockers, PPIs, metformin)

Question 12

B12 deficiency presentation

Answer 12

glossitis (tongue sorness)
paresthesias (hand numbness)
sensory loss affecting proprioception and vibration
diffuse hyperreflexia, absent Achilles reflexes
behavior changes (AMS, dementia)

Question 13

Chronic alcohol abuse is associated with what vitamin deficiency? and disease?

Answer 13

B1 - Thiamine

beriberi dz - dry beriberi

Question 14

Bells’s Palsy treatment

Answer 14

**prednisone
60 mg once daily x 7 days - followed by 5 day taper
Start within 3 days of symptom onset

valacyclovir - also an option and may combo with prednisone but no strong data as monotherapy

Question 15

Complex regional pain syndrome (CRPS) triad

Answer 15

burning pain
autonomic dysfunction
trophic changes

usually preceded by surgery or trauma

Question 16

describe the 3 stages of CRPS

Answer 16

1. Acute stage
   - affected limb swollen, red, burning
   - increased diaphoresis
   - all sxs near site of original injury
2. Dystrophic stage
   - skin of limb is cool and diaphoretic
   - Sudek’s atrophy of bone on x-ray
   - pain occurs throughout limb, not just site of injury
3. Atrophic stage
   - skin becomes pale and shiny
   - atrophy of muscle and bone in affected limb
   - pain may be constant even w treatment

Question 17

Tx for tourette syndrome

Answer 17

if mild tics and non-disabling - CBT 1st line

Moderate -severe tics
Clonidine or guanfacine - 1st line

Question 18

Copropraxia

Answer 18

obscene gestures