Peripheral Nerve and Skeletal Muscle Path CIS

Question 1

39 year old white male. Recent recovery from pneumonitis and before that, mononucleosis. No pain, but increasing clumsiness. DTR’s decreased bilaterally and you admit to MICU with dx with, “gait abnormality r/o cerebellar disease”. The next day unable to walk and requires ventilator support.

Answer 1

acute, inflammatory demyelinating polyneuropathy –> Guillane Barre Syndrome

Question 2

preceeding GI infetion (mycoplasma, Campylobacter, EBV, CMV). Think…

Answer 2

Guillane Barre Syndrome

Question 3

What therapy does Guillane barre syndrome respond to?

Answer 3

immunosuppressive, NOT steroids

Question 4

CSF in ___ most likely to show increased protein, scant white cells

Answer 4

acute, inflammatory demyelinating polyneuropathy (Guillane Barre Syndrome)

Question 5

52 year old Mexican lady’s face has become lumpy “leonine facies”. Complains of “pins and needles” with b/l sensorimotor neuropathy. BMI 29. Ulcerations on shin regions. High PGL-1 antibodies on serologic titer.

Answer 5

inflammatory neuropathy - lepromatous leprosy.

Question 6

27 year old white female. Blurry vision and malaise. Significant weakness and fatigue, esp walking or with prolonged standing. Drooping eyelids and some nystagmus. Repeat physical exercise indicates rapid muscle fatigue. Most likely?

Answer 6

Myasthenia Gravis

Question 7

27 year old white female. Blurry vision and malaise. Significant weakness and fatigue, esp walking or with prolonged standing. Drooping eyelids and some nystagmus. Repeat physical exercise indicates lessening of muscle fatigue. Most likely?

Answer 7

Lambert-Easton mysathenic syndrome

Question 8

anterior superior mediastinal mass measuring 5.2cm (large) are associated with what peripheral neuropathy.

Answer 8

Myasthenia gravis - thymic tumor OR thymic hyperplasia (B cell mainfestation)

Question 9

67 year old white female with BMI 30 and serum glucose of 205. Complains of malaise, but Hb/Hct 14/42% (so not anemic). PE shows weakness localized to proximal leg extensors and hand/wrist muscles. What is most likely?

Answer 9

inclusion body myositis

Question 10

Myasthenia gravis
Mitochondrial myopathies
Thyroid dz (toxic myopathy)

Answer 10

eye signs (ptosis, diploplia, etc)

Question 11

20 month old white boy began crawling, but progressive lethargy and less active. Poor stimulus response. Calves hypertrophic. Muscle CK elevated. Cardiac studies show supraventricular tachycardia and episodic afib. Dystrophin mutations.

Answer 11

Duchenne Muscular Dystrophy.