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Neuro Lab > Muscular Dystrophies > Flashcards

Muscular Dystrophies Flashcards

1
Q

Most common type of dytstrophy

A

Duchenne

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2
Q

Onset for DMD

A

1-5 years old

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3
Q

Progression for DMD

A

loss of walking by 9-10 y/o; death in late teens

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4
Q

Inheritance for DMD

A

X-Linked

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5
Q

Characterized by slow progression

A

BMD

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6
Q

Onset for BMD

A

5-10 y/o

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7
Q

Inheritance for BMD

A

X-Linked

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8
Q

Progression/course of BMD

A

Maintains walking past 16 y/o; Life span lasts up until the 3rd decade

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9
Q

Characterized by shortened lifespan

A

CMD

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10
Q

Onset for CMD

A

Birth

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11
Q

Inheritance for CMD

A

Recessive

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12
Q

Characterized by significant intellectual impairment

A

Congenital Myotonic Dystrophy (CMyD)

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13
Q

Inheritance for CMyD

A

Dominant

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14
Q

Onset for CMyD

A

Birth

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15
Q

Presents with loss of walking in later life

A

Childhood-Onset Fascioscapulohumeral (COFS)

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16
Q

Onset for COFS

A

1st decade

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17
Q

Inheritance for COFS

A

Either Recessive/Dominant

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18
Q

Characterized by cardiac abnormality but has a normal lifespan

A

Emery-Dreifuss Muscular Dystrophy (EDMD)

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19
Q

Onset for EDMD

A

Childhood to early teens

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20
Q

Inheritance for EDMD

A

X-linked

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21
Q

Caused by thyroid gland dysfunction

A

Endocrine myopathy

22
Q

Presents with inflamed muscles that cause pain

A

Inflammatory myopathy

23
Q

Where does muscle weakness present in pts c DMD

A

Proximal

24
Q

Sign commonly seen in pts c DMD

A

Gower’s sign

25
Q

Mean IQ for pts c DMD

A

88

25
Q

Orthotic management for pts c DMD

A

HKAFO
Scoliosis brace
Assistive devices

26
Q

Brace for above T6

A

Milwaukee

27
Q

Brace for below T6

A

Miami

28
Q

Brace for below T8

A

Boston
Yamamoto
Wilmington

29
Q

Hypertrophied muscles in DMD

A

Gastrocsoleus

30
Q

Second most common type of DMD

A

BMD

31
Q

Common site of weakness in pts c COFS

A

Facial muscles
Scapular stabilizers
Biceps/Triceps
Peroneals

32
Q

Type 1 LGMD

A

Dominant

33
Q

Type 2 LGMD

A

Recessive

34
Q

Common sites of affectation in LGMD

A

Arms and Shoulders
Hips and Legs

35
Q

Characterized by the inability to relax muscles

A

Myotonic Dystrophy (CMyD)

36
Q

Myotonic Dystrophy is more common among what age group?

A

Adults

37
Q

Clinical features of Myotonic Dystrophy

A

Fish-shaped mouth
Atrophied forearms
Bi-temporal narrowing

38
Q

Foot deformity commonly found among pts c Myotonic Dystrophy.

A

Club foot

39
Q

Inability to relax muscles

A

Myotonia

40
Q

Characterized by contractures in the neck, elbows, spine, and heels

A

EDMD

41
Q

EDMD is more common in what sex?

A

Males

42
Q

Clinical manifestations for pts c EDMD

A

Toe walking
Cardiac problems by 20 y/o

43
Q

Increased in thyroid hormones

A

Hyperthyroidism; Grave’s disease

44
Q

Decreased thyroid hormones

A

Hypothyroidism; Hashimoto’s disease

45
Q

Hypoparathyroidism results in

A

hypocalcemia

46
Q

Excess in cortisol

A

Hyperadrenalism; Cushing syndrome

47
Q

Lack of cortisol and aldosterone

A

Hypoadrenalism; Addison’s disease

48
Q

3 types of inflammatory myopathy

A

Polymyositis
Dermatomyositis
Inclusion body myositis

49
Q

which affects skeletal muscles on both sides of the body

A

Polymyositis

50
Q

which causes skin rashes and progressive muscle weakness

A

Dermatomyositis

51
Q

which is characterized by slow, progressive muscle weakness and muscle
shrinking and loss of muscle

A

Body Inclusion Myositis

Neuro Lab (1 decks)

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