Neuromuscular (Week 6) Flashcards

1
Q

2 types of hypertonia

A

spasticity

rigidity

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2
Q

cause of hypertonia

A

damage to CNS (UMN)

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3
Q
  • Tone increases throughout the whole mvmt; not rate dependent
  • caused by basal ganglia lesion
A

rigidity

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4
Q

Tone changes when mvmt intensity changes

A

spasticity

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5
Q

What causes hypotonia? (4)

A
  1. Damage to CNS (not UMN lesions)
  2. PNS damage
  3. muscle damage
  4. Genetic conditions
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6
Q

T/F

Tone increases during REM sleep.

A

False

Decreases bc the areas of brain slowed down

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7
Q

Disorders that are genetic in nature and affect the PNS including muscle, NM junction, peripheral nerves, and motor neurons as they exit SC

A

Neuromuscular disorders

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8
Q

autosomal recessive disorder that causes a mutation on chrom. 5 and leads to progressive loss of muscle control

A

spinal muscle atrophy

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9
Q

T/F

Sensory and intelligence are affected in spinal muscle atrophy.

A

False

only motor neurons are affected

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10
Q

What protein is affected in spinal muscle atrophy?

A

SMN = survival of motor neuron protein

- protein helps sustain lower motor neurons

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11
Q

Test that measures response of muscle & nerve to electrical activity

A

Electromyogram (EMG)

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12
Q

SMA II symptoms and onset

A

aka. Werdig-Hoffman Disease
- sit but never walk
- onset = 6-36 months
- survive to early adult but very compromised

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13
Q

Type of SMA that only affects males and starts at 15 years old and affects talking, chewing, swallowing

A

SMA IV

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14
Q

Type of SMA that starts at birth, never able to sit/walk, kids do not live long, muscular system very wasted

A

SMA I

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15
Q

SMA that has milder weakness, can sit and walk but may need a wheelchair later, onset around 18 months

A

SMA III

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16
Q

5 Symptoms of SMA

A
  1. progressive weakness (proximal and symmetric)
  2. hypotonia
  3. areflexia (less reflexes)
  4. swallowing and breathing difficulties
  5. delayed milestones
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17
Q

Is there a cure for SMA?

A

No

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18
Q

Is Charcot-Marie-Tooth disease autosomal dominant, autosomal recessive, or x-linked gene mutation?

A

All of them bc it is linked to numerous genes and many ways to trigger it

19
Q

Peripheral polyneuropathy disease affecting multiple sensory and motor nerves

A

Charcot-marie-tooth disease

20
Q

When is the onset of CMT and what is prognosis?

A
  • onset is childhood to young adult
  • slowly progresses
  • normal life expectancy
21
Q

What disease has the following symptoms?

  • weakness and wasting of distal extremities
  • claw position of hand and foot
  • decrease sensation
  • foot drop and high stepping
  • flexion contractures
A

Charcot-marie-tooth disease

22
Q

Why does someone w/CMT need pain mngt?

A

demyelination causes axonal damage which is painful

23
Q

Syndrome that causes protein changes at neuromuscular junction disrupting signal b/w nerve and muscle
- typically autosomal recessive gene mutation

A

Congenital myasthenic syndrome

24
Q

T/F

Symptoms are constant with congenital myasthenic syndrome

A

False

they are highly variable

25
Q

Symptoms are indicative of?

  • fluctuating weakness of eye, jaw, swallow muscles
  • ptosis, diplopia, dysphagia
  • fluctuating energy levels
  • breathing difficulties
  • symptoms worsen w/activity
A

Congenital myasthenic syndrome

26
Q

CMS is sometimes misdiagnosed as myasthenia gravis. What is the difference?

A

They have different onset times and CMS is genetic and not from antibody

27
Q

What causes duchenne muscular dystrophy?

A
  • affects muscle

- absence of muscle protein dystrophin

28
Q

What is most common form of muscular dystrophy?

A

duchenne

29
Q

What is genetic chance of getting duchenne MD?

A
  • X-linked recessive (mom is carrier)
  • 50% boy will be affected
  • 50% girl will be carrier
30
Q

Onset and life expectancy if have duchenne MD

A
onset = 3-5 yrs
Live = 25-50 yrs
31
Q

What is Beckers dystrophy?

A

milder form of duchenne MD

32
Q

What is pseudohypertrophy?

A

 Muscle looks big but is actually fat or connective tissue based and not muscle
 Common in calf, shoulders, butt
- seen in muscular dystrophy

33
Q

What is Gower’s sign?

A
  • how someone w/ MD uses their arms and hyperextension of joints to get up bc of muscle weakness
34
Q

Symptoms are indicative of?

  • progressive destruction of skeletal muscle
  • start proximal hip and shoulder girdle muscle and move distally (not trunk)
  • lower IQ
  • pseudohypertrophy
  • gower sign
A

Duchenne MD

35
Q

T/F

Brachial plexus palsy is a neuromuscular disorder.

A

False

it is a nerve injury and is not genetic

36
Q

Does a brachial plexus palsy cause hypertonia or hypotonia and why?

A
  • Hypotonia

- LMN injury that affects peripheral NS

37
Q

What nerve roots are part of brachial plexus?

A

C5-T1

38
Q

Common cause of brachial plexus palsy

A

obstetric injury

- shoulder dystocia = baby’s shoulder gets stuck on mom’s pelvis causing traction as baby tries to get out

39
Q

4 possible damages to the nerve in brachial plexus palsy

A
  1. neurapraxia (nerve stretched)
  2. neuroma (scar tissue from stretching)
  3. rupture (part or all nerve torn but not root)
  4. avulsion (nerve torn at root=prognosis not good)
40
Q

What type of BPI injury occurs when C5 and C6 are affected?

A

Erb’s palsy - impacts shoulder and elbow

41
Q

What type of BPI injury occurs when C8-T1 are affected?

A

Klumpke’s palsy - impact wrist and hand “claw hand”

42
Q

Are musculoskeletal diseases and musculoskeletal disorders the same? Explain

A

No

  • diseases affect growth/development of joints, bones, muscles, connective tissue
  • disorders are degenerative or inflammatory conditions that affect joints, ligaments, tendons, nerves, muscles (ex. arthritis, carpal tunnel syndrome)
43
Q

T/F

Musculoskeletal diseases are neuromuscular disorders.

A

False

44
Q

3 types of musculoskeletal diseases

A
  1. arthrogryposis
  2. achondroplasia
  3. osteogenesis imperfect