Neurology

Question 1

What is somatosensory evoked potentials (SEP) test used for?

Answer 1

• Diagnosing demyelinating process

* *Think testing for SEPeration of myelin from the nerve**

Question 2

When to order nerve conduction velocities

Answer 2

When concerned about peripheral neuropathy

Question 3

When to order EMG

Answer 3

• When concerned for muscular dystrophy

- Used to test for abnormal muscle activity (which may be due to neuropathy)

Question 4

Erb’s Palsy

Answer 4

• Brachial plexus injury at C5-6 or 7
• Results in paralysis of the upper arm
• “WAITER’S TIP” configuration if C7 involved
• Grasp and extension of the hand INTACT (i.e. the waiter can grasp his tip)
• May cause unilateral diagphragmatic paralysis in ~5% of cases (causing respiratory distress)

Question 5

Klumpke palsy

Answer 5

• Brachial plexus injury at C8 - T1
• Affects LOWER ARM and HAND -> CLAW HAND deformity with INABILITY to grasp
• Carries a worse prognosis than Erb’s palsy because nerves are typically torn
• Horner’s Syndrome (T1 lesion -> ptosis, miosis, anhydrosis and dry eye) usually present

Question 6

Horner’s Syndrome

Answer 6

• T1 lesion

- Ptosis, miosis, anhydrosis, dry eye

Question 7

Possible causes of CP

Answer 7

• Prematurity
• IUGR
• Intrauterine infections
• Asphyxia is responsible for only a small number of cases

Question 8

Athetoid cerebral palsy

Answer 8

• Motor impairment (spastic CP) + dystonia

Question 9

First step in evaluation a patient with progressive weakness or paralysis

Answer 9

ASSESS RESPIRATORY STATUS!

Question 10

Guillain-Barre Syndrome

Answer 10

• Classically ascending paralysis that progresses over days to weeks -> ataxia -> inability to walk
• May also present with back pain, fever, facial palsy and proximal muscle weakness prior to LE symptoms
• Decreased LE reflexes
• Associated with C. jejuni infection (recent diarrheal illness)
• Always consider tick paralysis in differential (look for history of camping, recent vacation, summertime, woods, or faster (hours - 2 days) progression of symptoms)

Question 11

Diagnosis of GBS

Answer 11

• LP that shows albuminocytologic dissociation (i.e. increased CSF protein in absence of increased WBCs)
• May also be referred to as absence of pleocytosis in CSF

Question 12

Treatment of GBS

Answer 12

• IVIG or plasmapheresis

- Steroids DO NOT HELP

Question 13

Symptoms of cord compression syndrome

Answer 13

• Loss of rectal tone
• Bowel/bladder incontinence
• Loss of sensation
• INCREASED LE reflexes (think of signal from LEs not being able to reach the brain, so the brain cannot dull the reflex that the spine produces)

Question 14

Tick paralysis symptoms and treatment

Answer 14

• Clinically can appear similar to GBS
• Ascending paralysis, ataxia, absent LE reflexes
• Caused by neurotoxin produced by ticks
• Normal CSF (GBS has increased CSF protein with no WBCs)
• Tx = remove the tick!

Question 15

Presentation of Myasthenia Gravis

Answer 15

• Baby with variable ptosis
• Older child with evidence of waxing and waning muscle weakness
• Diminished but NOT absent reflexes

Question 16

Cause of Myasthenia Gravis

Answer 16

• Autoimmune disease in which antibodies to the Ach receptors block them from functioning

Question 17

Testing for Myasthenia Gravis

Answer 17

• Serology for AchR-Ab (acetylcholine receptor antibody) or MuSK-Ab (muscle specific receptor tyrosine kinase antibody
• Tensilon (edrophonium) test -> positive test occurs when administration of edrophonium leads to improvement in ptosis or eye paralysis

Question 18

Treatment for Myasthenia Gravis

Answer 18

• Pyridostigmine
• Steroids
• Plasmapheresis
• Thymectomy (to permanently get rid of antibodies)

Question 19

What abnormal growth is associated with Myasthenia Gravis?

Answer 19

• Thymoma

- Consider quantitative immunoglobulins or imaging of the chest

Question 20

Spinal Muscular Atrophy Type 1 (i.e. SMA 1)

Answer 20

• Degeneration of anterior horn of spinal cord
• ONLY motor symptoms - NO sensory symptoms!
• Symptoms:
  
  • Tongue fasciculations
  • Hypotonia
  • Poor suck
• Most infants die before 1 year of age, often from respiratory failure

Question 21

Inheritance of Duchenne Muscular Dystrophy

Answer 21

X-linked (Xp21)

Question 22

Deficiency in Duchenne Muscular Dystrophy

Answer 22

Deficiency of muscles’ DYSTROPHIN protein

Question 23

Symptoms of DMD

Answer 23

• Toe walking
• Waddling gait
• Gower maneuver (using UEs to “walk up” body from squatting position)
• Pseudohypertrophy of calf muscles (2/2 fatty/fibrous tissue deposition)
• Poor head control
• Severe scoliosis
• Poor cough
• Frequent pneumonia
• Cardiomyopathy

Question 24

Progression of DMD

Answer 24

• Usually wheelchair-bound by 7-8 years of age

- Usually unable to walk by age 13 years

Question 25

Signs/symptoms of upper motor neuron disease

Answer 25

- Hyperactive reflexes - Increased muscle tone - + (up-going) Babinski (can be normal < 2 years) - NO fasciculations!

Question 26

Signs/symptoms of lower motor neuron disease

Answer 26

- Decreased/absent reflexes - Muscle atrophy - Fasciculations