SDL-5 Neuromuscular Disease Pathology Flashcards

1
Q

Type (I/II/III) muscle fibers are white, have ‘fast’ myosin, primarily used in strength and speed, and are rich in glycolytic enzymes and glycogen.

A

Type II

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2
Q

Type (I/II/III) muscle fibers are red, have ‘slow’ myosin, primarily used in endurance, and are rich in mitochondria and myoglobin.

A

Type I

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3
Q

(T/F) Normal muscle fibers have congregations of type I and type II fibers that largely remain segregated.

A

False. Normal muscle fibers have a diverse distribution of type I and II fibers, often resembling a “checkerboard” pattern.

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4
Q

What is the typical progression of chronic denervation in muscle?

A

I. Normal muscle is present
II. Motor unit loss to normal muscle causes small group atrophy
III. Collateral axonal sprouting follows motor unit loss. Re-innervation is attempted, and fiber type is “grouped” (reinnervated fibers switch to coincide with new axon) with giant motor units
IV. Further motor unit loss causes large group atrophy

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5
Q

Early denervation in neurogenic atrophy causes:

A

I. Formation of small angular fibers

II. Involvement of type I and II fibers

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6
Q

Chronic denervation in neurogenic atrophy results in:

A

I. Axonal sprouting and reinnervation
II. Formation of large motor units
III. Fiber type grouping

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7
Q

Late denervation of muscles in neurogenic atrophy causes:

A

I. Loss of large motor units

II. Large group atrophy of muscle fibers

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8
Q

_____________ is an autoimmune disease in which antibodies are produced against the body’s acetylcholine receptors at synapses such as neuromuscular junctions.

A

Myasthenia gravis

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9
Q

What general myopathic changes may be observed in a patient with myasthenia gravis?

A

I. Myofiber degeneration and/or regeneration
II. Internalization of cell nuclei
III. Increased endomysial connective tissue (scar tissue formation)
IV. Inflammation
V. Vacuolation

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10
Q

Type II atrophy of muscle is typically seen accompanying:

A

I. Disuse
II. Chronic disease
III. Cachexia
IV. Corticosteroids

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11
Q

The two most common inflammatory myopathies are _____________ and ___________.

A

I. Polymyositis

II. Dermatomyositis

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12
Q

Intrafascicular inflammation is a pathology commonly seen in (polymyositis/dermatomyositis/inclusion body myositis).

A

Polymyositis

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13
Q

The pathogenesis of polymyositis is caused by (humoral response/cytotoxic t-cells/degeneration of cellular structures).

A

Cytotoxic T cells

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14
Q

(T/F) Clinical presentation of polymyositis includes pain and a noticeable rash.

A

False. This is the clinical presentation of dermatomyositis. Polymyositis presents without rash. Only pain is present.

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15
Q

Extrafascicular inflammation and perifascicular atrophy are pathologies commonly seen in (polymyositis/dermatomyositis/inclusion body myositis).

A

Dermatomyositis

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16
Q

The pathogenesis of dermatomyositis is caused by (humoral response/cytotoxic t-cells/degeneration of cellular structures).

A

Humoral response

17
Q

Inclusions and rimmed vacuoles are pathologies commonly seen in (polymyositis/dermatomyositis/inclusion body myositis).

A

Inclusion body myositis

18
Q

The pathogenesis of inclusion body myositis is caused by (humoral response/cytotoxic t-cells/degeneration of cellular structures).

A

Degeneration of cellular structures

19
Q

(T/F) Inclusion body myositis presents clinically with resistance to steroids.

A

True.

20
Q

A general systemic deficiency of dystrophin results in (Duchenne MD/Becker MD/Myotonic MD).

A

Duchenne MD

21
Q

A truncated dystrophin molecule caused by a multiple-of-three base deletion results in (Duchenne MD/Becker MD/Myotonic MD).

A

Becker MD

22
Q

A form of muscular dystrophy caused by trinucleotide repeats is (Duchenne MD/Becker MD/Myotonic MD).

A

Myotonic MD