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REVIEW QUESTIONS -

1. Histopathologic changes characteristic of Alzheimer's disease (AD) are:

A. Lewy bodies

B. Neurofibrillary tangles

C. Amyloid angiopathy

D. Neuritic plaques

E. Neuronal losses

2. Neurofibrillary tangles are:

A. Best revealed with phosphotungstic acid hematoxylin (PTAH)

B. Argyrophilic

C. Composed of fibrillary acidic protein

D. Composed of hyperphosphorylated tau protein

E. Common in the hippocampus

3. A neuritic plaque is composed of:

A. An amyloid core

B. Rod-shaped eosinophilic structures

C. Dystrophic neurites

D. Activated microglial cells

E. Reactive astrocytes

4. Neuritic plaques:

A. Are common in the white matter

B. Are argyrophilic

C. Contain amyloid precursor protein (APP)

D. Contain Ap-peptide

E. Are revealed with thioflavin-S in polarized light

5. Genes associated with early-onset, dominantly inherited AD are:

A. Presenilin 1 on chromosome 14

B. APP gene on chromosome 21

C. Gene of apolipoprotein (APOE)4 on chromosome 4

D. All of these

E. None of these

6. Pathologic features of Pick's disease include:

A. Knife-edge atrophy of the parieto-occipital lobes

B. Argyrophilic cytoplasmic inclusions

C. Knife-edge atrophy of the frontotemporal lobes

D. Eosinophilic cytoplasmic inclusions

E. Tau-positive cytoplasmic inclusions

7. Dementia with parkinsonism linked to chromosome 17 is characterized by:

A. Mutations in a-synuclein gene

B. Depigmentation of the substantia nigra

C. Argyrophilic inclusions in the neurons

D. Mutations in tau-gene on chromosome 17

E. Astrocytic plaques in the cortex

8. Clinical features of dementia with corticobasal degeneration include:

A. Limb dystonia

B. Akinetic rigidity

C. Alien limb phenomenon

D. All of these

E. None of these

9. The pathology of dementia with corticobasal degeneration includes:

A. Tau-positive neuronal inclusions

B. Argyrophilic astrocytic inclusions

C. Achromatic neurons

D. Lewy bodies in the substantia nigra

E. Hirano bodies in the hippocampus

10. Histopathologic features of amyotrophic lateral sclerosis (ALS) are:

A. Degeneration of corticospinal tracts

B. Degeneration of spinothalamic tracts

C. Degeneration of spinal motor neurons

D. Eosinophilic inclusions in the motor neurons

E. Neurogenic muscle atrophy

11. Characteristics of spinal muscular atrophies are:

A. They are common in children.

B. They present with weakness, atrophy, fasciculation and loss of vibration sensation.

C. They are caused by trinucleotide repeat expansion.

E. None of these.

12. All of the following characterize idiopathic Parkinson's disease (iPD) except:

A. Akinetic rigidity

B. Dopamine deficiency

C. Depigmentation of substantia nigra

D. Tau-positive argyrophilic inclusions in the neurons of the substantia nigra

E. In dominantly inherited PD mutation in a-synuclein gene (Park 1) on chromosome 4

13. Clinical findings characteristic of progressive supranuclear palsy (PSP) include:

A. Neck dystonia

B. Vertical gaze palsy

C. Bilateral Babinski signs

D. All of these

E. None of these

14. Pathologic findings characteristic of PSP are:

A. Degeneration of corticospinal tracts

B. Degeneration of substantia nigra

C. Neurofibrillary tangles in the hippocampus

D. Lewy bodies in the neocortex

E. Tau-positive inclusions in the astrocytes

15. Lewy body dementia is clinically distinguished by:

A. Parkinsonian features

B. Visual hallucinations

C. Syncope

D. All of these

E. None of these

16. Characteristics of Huntington's disease (HD) are:

A. It is dominantly inherited.

B. The huntingtin gene maps to chromosome 4.

C. It is a disorder of CAG trinucleotide repeat expansion.

D. Juvenile cases are associated with short repeats.

E. Juvenile cases are associated with long repeats.

17. Somatic features of ataxia telangiectasia include:

A. Retarded growth

B. Progenic changes (early aging) of skin

C. Delayed sexual development

D. All of these

E. None of these

18. Histopathologic characteristics of olivopontocerebellar atrophy (OPCA) are:

A. Degeneration of Purkinje cells

B. Degeneration of brachium conjunctivum

C. Tau-positive inclusions in the oligodendrocytes

D. a-Synuclein-positive inclusions in oligo-dendrocytes

E. Loss of sympathetic neurons in the spinal cord

19. Clinically, OPCA may present with:

A. Cerebellar ataxia

B. Autonomic dysfunctions

C. Parkinsonian features

D. All of these

E. None of these

20. All of the following characterize Friedreich's ataxia except:

A. It is an autosomal dominant disorder

B. It is associated with expansion of GAA repeats

C. Skeletal anomalies are present

D. The cerebellar dentate neurons are degenerated

E. The sensory nerve roots are degenerated

(Answers are provided in the Appendix.)

CHAPTER 6

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