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Med Conditions > Week 14 – Muscular Dystrophy > Flashcards

Week 14 – Muscular Dystrophy Flashcards

1
Q

DMD

A

Duchenne Muscular Dystrophy

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2
Q

Duschenne Muscular Dystrophy (basic description)

A
  • X-linked Recessive inherited disorder
  • Most common type of MD
  • proximal weakness of legs and arms
  • Affects lower extremities (LE) prior to the upper extremities (UE)
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3
Q

True or False: DMD is only seen in boys

A

True

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4
Q

MD

A

Muscular Dystrophy ~ a neuromuscular disorder characterized by
progressive muscle weakness resulting from a defect in
muscle protein leading to death of muscle cells

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5
Q

How many types of Md are there?

A

9 (we focus on 6 most common)

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6
Q

What is the most common type of MD?

A

DMD

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7
Q

Cause of DMD

A

absence or deficiency in dystrophin protein

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8
Q

Results of DMD

A

PHYSICAL:

  • proximal weakness of legs and arms
  • having a waddling gait
  • enlarged calf muscles
  • “valley sign” - an indent in between armpit and deltoid muscle area
  • PROGRESSES from proximal to distal

OTHER:

  • Children are usually using a wheelchair (W/C) by age 12
  • 1/3 have cognitive impairment
  • Life expectancy early 20’s
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9
Q

BMD

A

Becker Muscular Dystrophy

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10
Q

BMD (symptoms)

A
  • similar to DMD
  • only affects boys; x-linked recessive
  • appears between 6-18 y/o
  • PROGRESSIVE muscular weakness
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11
Q

True or False: muscle weakness progresses slower in BMD than DMD?

A

True

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12
Q

Progression of BMD

A
  • occurs BILATERALLY
  • progresses proximal to distal
  • starting at PELVIC GIRDLE & thigh muscles
  • goes to TRUNK & UE
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13
Q

Difference of BMD from DMD

A
  • w/c dependent in their 30’s

* survival rate is into early adulthood (25-30’s)

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14
Q

LGMD

A

Limb Girdle Muscular Dystrophy

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15
Q

True or false: LGMD accounts for 20% of the 30 subtypes of MD?

A

False: accounts for 50%!!! of all 30 subtypes of MD

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16
Q

True or false: Most LGMD are AUTOSOMAL RECESSIVE

both parents carry the affected gene and pass it to their children

A

True

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17
Q

True or False: (for LGMD) 50% are autosomal dominant

where a child inherits a normal gene from one parent and an affected gene from the other

A

False: only 10%

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18
Q

True or False: (for LGMD) more severe form with faster decline, meaning onset in childhood often death in 30’s. If there’s an adult onset, they may have a normal lifespan.

A

True

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19
Q

(for LGMD) Dystrophin levels are normal but there is decreased ________

A

GLYCOPROTEINS

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20
Q

LGMD affects ___ & ___ but not necessarily symmetrically (one side of body can be more involved than other)

A

shoulders & pelvis

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21
Q

MMD

A

Myotonic Muscular Dystrophy

22
Q

Autosomal dominant

A

causes by a child inheriting a normal gene from 1 parent and an affected gene from the other

23
Q

Symptoms of MMD

A
  • Autosomal Dominant
  • Muscle weakness and wasting begins in the face, lower legs, forearms, hands and neck; can have cognitive impairment
  • Affects GI system
24
Q

FSMD

A

Facioscapulohumeral Muscular Dystrophy

25
Q

Symptoms of FSMD

A
  • Initially affects the facial, shoulder and upper arm muscles with progressive weakness throughout the body in time, usually not symmetrical
  • Facial muscle weakness causes difficulty with closing the eyes, asymmetrical smile, drooping of the corners of the mouth, inability to whistle or pucker and wasting/atrophy of facial muscles
26
Q

True or False: FMSD is the 10th most common MD

A

False: 3rd most common

27
Q

EDMD

A

Emery-Dreifuss Muscular Dystrophy

28
Q

EDMD symptoms

A
  • muscle contractures before weakness
  • present in heal cords - toe walking; trips; falls
  • ELBOWS contract to 90 degrees
29
Q

(for EDMD) Contracture of ____ and ____ pulling the body

backwards with difficulty flexing forward

A

Spine & Posterior Neck

30
Q

X-Recessive Chromosome Diseases:

A
  • DMD

* EDMD

31
Q

What is an X-recessive chromosome disease?

A

mother is a carrier (on her X -chromosome) and sons can get the disorder

32
Q

Autosomal recessive diseases:

A
  • EDMD

* LGMD

33
Q

Autosomal Recessive Dominant (definition):

A

both parents must carry the disorder and pass on to children

34
Q

Autosomal dominant DISEASES:

A
  • EDMD
  • LGMD
  • FSMD
  • MMD
35
Q

True or False: Duchenne MD & Becker MD are the most common

types of MD and affect only females

A

FALSE: only affects MALES!!

36
Q

How is diagnosis confirmed?

A

through blood testing

37
Q

EMG - define

A

Electromyography ~ measures muscle response or electrical activity in response to a nerve’s stimulation of the muscle

38
Q

Muscle Biopsy - define

A

A muscle biopsy is a procedure used to diagnose diseases involving muscle tissue

39
Q

EMG - purpose

A

evaluate the electrical activity of skeletal muscle (for MD)

40
Q

Muscle Biopsy - purpose

A

used to distinguish the type of dystrophy

41
Q

True or false: (for MD) If one child is diagnosed, all siblings and parents need to be tested as well

A

TRUE

42
Q

Medical Complications with MD:

A
  • Respiratory
  • Skin Integrity
  • Pain
  • Nutrition
43
Q

Therapy Interventions for MD

A 
HEP (Home Exercise Program)
ADL
Equipment Needs & Home modifications
Splinting
*Adaption & Energy Conservation
44
Q

Purpose of corticosteroids in MD

A

to assist respiration and decrease muscle damage

45
Q

Purpose of Immunosuppressant drugs in MD

A

delay muscle degeneration

46
Q

Purpose of Anticonvulsants in MD

A

used to control muscle activity

47
Q

Antibiotics

A

for infection

48
Q

Purpose of OTC (over-the-counter) in MD

A

use for pain management and muscle cramping

49
Q

Contracture release - Define

A

relieve pressure on the nerves and free them from surrounding scar tissue

50
Q

Cardiac Stability Surgery would be used with which MD?

A

EDMD

Med Conditions (5 decks)

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