Neuromuscular (Week 6)

Question 1

2 types of hypertonia

Answer 1

spasticity

rigidity

Question 2

cause of hypertonia

Answer 2

damage to CNS (UMN)

Question 3

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

Answer 3

rigidity

Question 4

Tone changes when mvmt intensity changes

Answer 4

spasticity

Question 5

What causes hypotonia? (4)

Answer 5

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

Question 6

T/F

Tone increases during REM sleep.

Answer 6

False

Decreases bc the areas of brain slowed down

Question 7

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

Answer 7

Neuromuscular disorders

Question 8

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

Answer 8

spinal muscle atrophy

Question 9

T/F

Sensory and intelligence are affected in spinal muscle atrophy.

Answer 9

False

only motor neurons are affected

Question 10

What protein is affected in spinal muscle atrophy?

Answer 10

SMN = survival of motor neuron protein

- protein helps sustain lower motor neurons

Question 11

Test that measures response of muscle & nerve to electrical activity

Answer 11

Electromyogram (EMG)

Question 12

SMA II symptoms and onset

Answer 12

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

Question 13

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

Answer 13

SMA IV

Question 14

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

Answer 14

SMA I

Question 15

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

Answer 15

SMA III

Question 16

5 Symptoms of SMA

Answer 16

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

Question 17

Is there a cure for SMA?

Answer 17

No

Question 18

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

Answer 18

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

Question 19

Peripheral polyneuropathy disease affecting multiple sensory and motor nerves

Answer 19

Charcot-marie-tooth disease

Question 20

When is the onset of CMT and what is prognosis?

Answer 20

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

Question 21

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

Answer 21

Charcot-marie-tooth disease

Question 22

Why does someone w/CMT need pain mngt?

Answer 22

demyelination causes axonal damage which is painful

Question 23

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

Answer 23

Congenital myasthenic syndrome

Question 24

T/F

Symptoms are constant with congenital myasthenic syndrome

Answer 24

False

they are highly variable

Question 25

Symptoms are indicative of? - fluctuating weakness of eye, jaw, swallow muscles - ptosis, diplopia, dysphagia - fluctuating energy levels - breathing difficulties - symptoms worsen w/activity

Answer 25

Congenital myasthenic syndrome

Question 26

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

Answer 26

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

Question 27

What causes duchenne muscular dystrophy?

Answer 27

- affects muscle | - absence of muscle protein dystrophin

Question 28

What is most common form of muscular dystrophy?

Answer 28

duchenne

Question 29

What is genetic chance of getting duchenne MD?

Answer 29

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

Question 30

Onset and life expectancy if have duchenne MD

Answer 30

``` onset = 3-5 yrs Live = 25-50 yrs ```

Question 31

What is Beckers dystrophy?

Answer 31

milder form of duchenne MD

Question 32

What is pseudohypertrophy?

Answer 32

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

Question 33

What is Gower's sign?

Answer 33

- how someone w/ MD uses their arms and hyperextension of joints to get up bc of muscle weakness

Question 34

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

Answer 34

Duchenne MD

Question 35

T/F | Brachial plexus palsy is a neuromuscular disorder.

Answer 35

False | it is a nerve injury and is not genetic

Question 36

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

Answer 36

- Hypotonia | - LMN injury that affects peripheral NS

Question 37

What nerve roots are part of brachial plexus?

Answer 37

C5-T1

Question 38

Common cause of brachial plexus palsy

Answer 38

obstetric injury | - shoulder dystocia = baby's shoulder gets stuck on mom's pelvis causing traction as baby tries to get out

Question 39

4 possible damages to the nerve in brachial plexus palsy

Answer 39

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)

Question 40

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

Answer 40

Erb's palsy - impacts shoulder and elbow

Question 41

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

Answer 41

Klumpke's palsy - impact wrist and hand "claw hand"

Question 42

Are musculoskeletal diseases and musculoskeletal disorders the same? Explain

Answer 42

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)

Question 43

T/F | Musculoskeletal diseases are neuromuscular disorders.

Answer 43

False

Question 44

3 types of musculoskeletal diseases

Answer 44

1. arthrogryposis 2. achondroplasia 3. osteogenesis imperfect