Nervous System Demyelinating Disorders

Question 1

Which disorder has the following CSF finding:

elevated protein
otherwise normal

Answer 1

Guillain Barré Syndrome

Question 2

List the treatments for Guillain Barré Syndrome

Answer 2

1. Respiratory support
2. Plasmaphoresis (filtering out plasma w/circulating antibodies and returning just blood cells)
3. IV immune globulins (pooled antibodies from 1000’s of patients)

Question 3

A series of MRI imaging shows lesions that come and go

Answer 3

Multiple Sclerosis

Question 4

Clinical presentation:

multiple white matter lesions on imaging
slow onset encephalopathy (altered mental status;
focal neurological deficits)
HIV/really low CD4 (<200)

Answer 4

Progressive Multifocal Leukoencephalopthy (PML)

really poor prognosis (normally <1 yr)

Question 5

List characteristics of Charcot-Marie-Tooth Syndrome

aka Hereditary Motor and Sensory Neuropathy

Answer 5

Genetic
Onset: late childhood
Progressive peripheral nerve disorders
Most commonly affects lower extremities, esp. perineal nerve
Stork legs
Pes  cavus
Claw hands
Falls and clumsiness