Demyelinating Disorders

Question 1

Typical findings in TM on cerebrospinal fluid analysis:

Answer 1

Pleocytosis and/or a raised IgG index

Question 2

__________ is an immune-mediated disorder that leads to bilateral motor weakness, autonomic dysfunction, and sensory dysfunction below the level of the affected segment

Answer 2

Transverse myelitis

Question 3

First-line treatment for TM:

Answer 3

high-dose IV glucocorticoids

Question 4

4 As of Guilllain-Barré syndrome:

Answer 4

• Acute inflammatory demyelinating polyradiculopathy
• Ascending paralysis
• Autonomic neuropathy
• Albuminocytologic dissociation(increased albumin in CSF)

Question 5

In addition to respiratory support, Guillain-Barré syndrome may be treated with:

Answer 5

IVIG and plasmapheresis

Question 6

Diagnosis of Guillain–Barré syndrome is made by clinical findings, CSF analysis, and_____

Answer 6

nerve conduction studies

Question 7

What is the diagnosis in a patient that presents with rapidly progressive limb weakness that ascends following GI/URT infection?

Answer 7

Guillain-Barré syndrome

Question 8

What is the likely diagnosis in a patient that presents with monocular vision loss accompanied by “washed-out” color vision, Marcus Gunn pupilS, and painful eye movements?

Answer 8

Optic neuritis

Question 9

What imaging modality is the gold standard for diagnosis of multiple sclerosis?

Answer 9

MRI

Question 10

_______ on high resolution electrophoresis is diagnostic of multiple sclerosis

Answer 10

Oligoclonal bands of IgG

Question 11

Young woman with blurry vision and focal neurological symptoms (weakness, numbness, tingling) separated by “time and space” is suggestive of

Answer 11

Multiple sclerosis

Question 12

What is the treatment of choice for acute exacerbations of multiple sclerosis with disabling neurologic symptoms?

Answer 12

Corticosteroids

Question 13

GBS results in a ____ motor neuron lesion

Answer 13

lower

Question 14

MS usually results in a _____ motor neuron dysfunction.

Answer 14

upper

Question 15

Patient whose laboratory studies show positive anti-Toxoplasma gondii IgG-antibodies and a CD4+ T-lymphocyte count of 90/mm3. Trimethoprim-sulfamethoxazole prophylactic therapy is begun. This medication will also prevent infection with:

Answer 15

Pneumocystis jirovecii

Question 16

Impaired adduction of the ipsilateral eye and abduction nystagmus of the contralateral eye (i.e., dissociated nystagmus) on lateral horizontal gaze should raise concern for:

Answer 16

internuclear ophthalmoplegia (INO)

Question 17

• Acute multiple sclerosis is managed with:
• Chronic multiple sclerosis is managed with:

Answer 17

• Glucocorticoids
• Interferon β, glatiramer, or natalizumab

Question 18

There is increased risk of _________ in patients taking natalizumab and rituximab

Answer 18

progressive multifocal leukoencephalopathy

Question 19

Multiple sclerosis most commonly manifests with fatigue, but may also present with (mnemonic = SIIIN)

Answer 19

-Scanning Speech
- Intention tremor
- Incontinence
- Internuclear ophthalmoplegia
- Nystagmus

Question 20

Patients with suspected Guillain-Barré syndrome should have their pulmonary function assessed with

Answer 20

serial spirometry

Question 21

Miller-Fisher syndrome is a variant of Guillain-Barré syndrome that presents with the triad of:

Answer 21

Ataxia
Ophthalmoplegia
Areflexia

Question 22

What MRI finding is associated with multiple sclerosis?

Answer 22

Periventricular plaques

Question 23

ALS presents with asymmetric ______ paralysis in the upper limbs and asymmetric ________paralysis in the lower limbs

Answer 23

flaccid
spastic

Question 24

_______is a treatment for amyotrophic lateral sclerosis (ALS) that increases survival by decreasing glutamate excitotoxicity

Answer 24

Riluzole

Question 25

Which test is most likely to establish amyotrophic lateral sclerosis (ALS)diagnosis?

Answer 25

Electromyography (EMG). EMG findings in ALS include spontaneous fasciculation potentials