PNS Flashcards

0
Q

Morphology:

Albuminocytologic dissociation in CSF

A

GBS

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1
Q

Immune-mediated demyelinating neuropathy asso with Campylobacter jejuni
- Ascending motor paralysis

A

Guillain-Barre Syndrome

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2
Q

Increased CSF protein in the absence of increased WBCs

A

Albuminocytologic Dissociation

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3
Q

Aka Charcot-Marie-Tooth Disease

A

Hereditary Motor and Sensory Neuropathy

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4
Q

Distal symmetric neuropathy
Axonal neuropathy
Segmental demyelination

A

Diabetic neuropathy

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5
Q

aka Infantile Motor Neuron Disease

A

Spinal Muscular Atrophy

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6
Q

Destruction of anterior horn cells
Mutations affecting SMN1
Panfascicular atrophy

A

Spinal muscular Atrophy

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7
Q

Floppy Baby Syndrome

A

Spinal Muscular Atrophy

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8
Q

Muscular fibers undergo degeneration and are replaced by fibrofatty tissue and collagen
Mutations in gene coding for Dystrophin

A

Muscular Dystrophy

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9
Q

Types of Muscular Dystrophy

A

Duchenne Muscular Dystrophy

Becker Muscular Dystrophy

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10
Q

Fibrofatty replacement of muscles
Proliferation of endomysial connective tissue
Degeneration of muscle fibers

A

Muscular Dystrophy

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11
Q

Clinical Features of Muscular Dystrophy

A

Gower Sign

Calf Pseudohypertrophy

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12
Q

Sustained involuntary contraction of muscles

Asso with CTG trinucleotide repeats

A

Myotonic Dystrophy

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13
Q

Ring fiber

Sarcoplasmic mass

A

Myotonic Dystrophy

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14
Q

Sarcolemmal band of cytoplasm

A

Ring Fiber

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15
Q

Irregular mass of sarcoplasm

A

Sarcoplasmic mass

16
Q

MELAS Syndrome

A

Mitochondral Encephalomyopathy, Lactic Acidosis, Stroke-like Episodes

17
Q

Proximal muscle weakness
External ophthalmoplegia
Neurologic Symptoms

A

Mitochondrial Myopathies (MELAS)

18
Q

Aggregates of abnormal mitochondria
RAGGED FIBERS
PARKING LOT INCLUSIONS

A

Mitochondrial myopathies

19
Q

irrrgular fiber with subsarcolemm collections of mitochondria

A

RAGGED FIBERS

20
Q

alterations in structure of cristae

A

PARKING LOT INCLUSIONS

21
Q

Inflammatory disorder of the SKIN and SKELETAL MUSCLE

A

Dermatomyositis

22
Q

Lilac or HELIOTROPE rash on eyelids with periorbital edema
Dusky patched over the knuckles, elbows and knees (Gottron lesions)
PROXIMAL muscle weakness

A

Dermatomyositis

23
Q

Inflammatory disorders that present as Proximal Weakness

A

Dermatomyositis

Polymyositis

24
Q

Lacks cutaneous involvement
Occurs mainly in adults
Proximal muscle weakness

A

Polymyositis

25
Q

Asymmetric DISTAL muscle weakness

Adults age > 50

A

Inclusion Body Myositis

26
Q

PERIFASCICULAR ATROPHY

A

Dermatomyositis

27
Q

PANFASCICULAR ATROPHY

A

Spinal Muscular Atrophy -> Pan

Polymyositis -> Pan

28
Q

rimmed vacuoles (inclusion bodies)

A

Inclusion Body Myositis

29
Q

Immune-mediated loss of Ach Receptors due to Antibodies
Asso with thymic hyperplasia and thymoma
Morpho:
type 2 fiber atrophy
Immune complex deposition along postsynaptic membrane

A

Myasthenia Gravis