DIMITRI P. AGAMANOLIS, M. D. Akron Childrens Hospital NorthEastern Ohio Universities College of Medicine Neuropathology
TEST-CHAPTER SIX
DEMYELINATIVE DISORDERS
Questions marked with * are more appropriate for residents.
1. Multiple sclerosis plaques involve spinal roots
A. True
B. False
2. All MS follows a relapsing and exacerbating course
A. True
B. False
3. Is remyelination in the central nervous system possible?
A. Yes
B. No
4. Axons are not damaged in multiple sclerosis
A. True
B. False
5. MS is more common in northern countries
A. True
B. False
6. Which of the following is useful for treating MS?
A. INF Beta
B. INF Gamma
*7. A 32 year old woman developed acute neurologic deficits and contrast enhancing periventricular lesions. A stereotactic biopsy revealed changes shown below.
MS
Immunohistochemistry of this lesion will show:
A. A vast majority of B cells.
B. T cells.
C. A mixture of T and B cells
8. Conduction velocity in the peroneal nerve of a patient with MS and paraparesis is:
A. Increased
B. Decreased
C. Normal
D. Decreased only if the leg is totally paralyzed
9. The risk of MS in the identical twin of a patient with MS is:
A. 5%
B. 15%
C. 25%
D. 50%
10. Which of the following is not seen in old MS lesions?
A. Gliosis
B. Macrophages
C. Axonal loss
D. Dense perivascular mononuclear cells
11. Devic's disease is characterized by involvement of:
A. The optic nerve only
B. The optic radiation
C. The brainstem
D. The spinal cord only
E. None of the above
12. Which of the following is most commonly affected in MS?
A. Cerebellum
B. Brainstem
C. Periventricular white matter
D. The subcortical white matter
13. A 37 year old man had progressive neurologic deficits including hemiparesis and ataxia for nine months. CSF shows 37 lymphocytes, protein 54, glucose 50, and no oligoclonal bands. The most likely diagnosis is:
A. AIDS
B. Lyme disease
C. MS
D. Tuberculous meningitis
14. A 12 year old white male had progressive psychomotor decline for three years and the MRI findings shown below
X-linked_Adrenoleukodystrophy
The CSF shows 15 lymphocytes, glucose 67, protein 43, and normal CSF IgG/albumin ratio. The most likely diagnosis is:
A. MS
B. X-linked adrenoleukodystrophy
C. Mucopolysaccharidosis
D. Metachromatic leukodystrophy
*15. A 37 year old man with AIDS developed ataxia and paralysis. The MRI shows cerebellar and brainstem nonenhancing lesions. The cerebellar biopsy includes mostly normal cerebellar cortex and a small amount of white matter which shows a few mononuclear cells, macrophages and reactive astrocytes. The best use of the material in order to obtain a diagnosis is:
A. Reprocessing the material for EM
B. Using the material for viral PCR
C. Doing immunohistochemistry for papova viruses
D. Recommending a spinal tap with culture and PCR
16. Which of the following structures is most commonly affected in MS?
A. The internal capsule
B. The optic radiation
C. The optic chiasm
D. The brainstem
17. A 26 year old woman developed severe dizziness, hoarseness, extension of the neck, and right arm weakness in the course of 8 days. The MRI shows enhancing lesions in the lower brainstem and upper spinal cord. A stereotactic biopsy is shown below.
MS
The most likely diagnosis is:
A. Cerebral lymphoma
B. Herpes simplex encephalitis
C. Acute disseminated encephalomyelitis
D. Multiple sclerosis
*18. A 32 year old hemophiliac patient developed neurologic deficits and enhancing cerebral and cerebellar white matter lesions over a period of four months. A stereotactic biopsy of the cerebellum is shown below.
Progressive multifocal leukoencephalopathy
The most likely diagnosis is:
A. Glioblastoma multiforme
B. AIDS encephalitis
C. An opportunistic viral infection
D. Lhermitte-Duclos syndrome
19. The pathology illustrated in this myelin stained section of the pons is likely to occur in all of the following conditions except:
Central pontine myelinolysis
A. Severe burns
B. Advanced alcoholic cirrhosis
C. Severe bacterial infections
D. Vitamin B1 deficiency
E. Chronic renal failure
20. Stereotactic brain biopsy of a ring enhancing lesion of the left frontal lobe in a previously healthy 37 year old woman who had neurological symptoms for 2 months. Work up showed no fever, sinusitis or other findings. The most likely diagnosis is:
MS
A. MS
B. Cerebral abscess
C. A viral infection
D. Cerebral lymphoma
22. The pathology in a 17 year old male shown below represents:
X-linked Adrenoleukodystrophy
A. An inflammatory demyelinative disease
B. A metabolic disorder of peroxisomes
C. A metabolic disorder of lysosomes
D. Post anoxic demyelination