Neuromuscular (Week 6) Flashcards
2 types of hypertonia
spasticity
rigidity
cause of hypertonia
damage to CNS (UMN)
- Tone increases throughout the whole mvmt; not rate dependent
- caused by basal ganglia lesion
rigidity
Tone changes when mvmt intensity changes
spasticity
What causes hypotonia? (4)
- Damage to CNS (not UMN lesions)
- PNS damage
- muscle damage
- Genetic conditions
T/F
Tone increases during REM sleep.
False
Decreases bc the areas of brain slowed down
Disorders that are genetic in nature and affect the PNS including muscle, NM junction, peripheral nerves, and motor neurons as they exit SC
Neuromuscular disorders
autosomal recessive disorder that causes a mutation on chrom. 5 and leads to progressive loss of muscle control
spinal muscle atrophy
T/F
Sensory and intelligence are affected in spinal muscle atrophy.
False
only motor neurons are affected
What protein is affected in spinal muscle atrophy?
SMN = survival of motor neuron protein
- protein helps sustain lower motor neurons
Test that measures response of muscle & nerve to electrical activity
Electromyogram (EMG)
SMA II symptoms and onset
aka. Werdig-Hoffman Disease
- sit but never walk
- onset = 6-36 months
- survive to early adult but very compromised
Type of SMA that only affects males and starts at 15 years old and affects talking, chewing, swallowing
SMA IV
Type of SMA that starts at birth, never able to sit/walk, kids do not live long, muscular system very wasted
SMA I
SMA that has milder weakness, can sit and walk but may need a wheelchair later, onset around 18 months
SMA III
5 Symptoms of SMA
- progressive weakness (proximal and symmetric)
- hypotonia
- areflexia (less reflexes)
- swallowing and breathing difficulties
- delayed milestones
Is there a cure for SMA?
No
Is Charcot-Marie-Tooth disease autosomal dominant, autosomal recessive, or x-linked gene mutation?
All of them bc it is linked to numerous genes and many ways to trigger it
Peripheral polyneuropathy disease affecting multiple sensory and motor nerves
Charcot-marie-tooth disease
When is the onset of CMT and what is prognosis?
- onset is childhood to young adult
- slowly progresses
- normal life expectancy
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
Charcot-marie-tooth disease
Why does someone w/CMT need pain mngt?
demyelination causes axonal damage which is painful
Syndrome that causes protein changes at neuromuscular junction disrupting signal b/w nerve and muscle
- typically autosomal recessive gene mutation
Congenital myasthenic syndrome
T/F
Symptoms are constant with congenital myasthenic syndrome
False
they are highly variable
Symptoms are indicative of?
- fluctuating weakness of eye, jaw, swallow muscles
- ptosis, diplopia, dysphagia
- fluctuating energy levels
- breathing difficulties
- symptoms worsen w/activity
Congenital myasthenic syndrome
CMS is sometimes misdiagnosed as myasthenia gravis. What is the difference?
They have different onset times and CMS is genetic and not from antibody
What causes duchenne muscular dystrophy?
- affects muscle
- absence of muscle protein dystrophin
What is most common form of muscular dystrophy?
duchenne
What is genetic chance of getting duchenne MD?
- X-linked recessive (mom is carrier)
- 50% boy will be affected
- 50% girl will be carrier
Onset and life expectancy if have duchenne MD
onset = 3-5 yrs Live = 25-50 yrs
What is Beckers dystrophy?
milder form of duchenne MD
What is pseudohypertrophy?
Muscle looks big but is actually fat or connective tissue based and not muscle
Common in calf, shoulders, butt
- seen in muscular dystrophy
What is Gower’s sign?
- how someone w/ MD uses their arms and hyperextension of joints to get up bc of muscle weakness
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
Duchenne MD
T/F
Brachial plexus palsy is a neuromuscular disorder.
False
it is a nerve injury and is not genetic
Does a brachial plexus palsy cause hypertonia or hypotonia and why?
- Hypotonia
- LMN injury that affects peripheral NS
What nerve roots are part of brachial plexus?
C5-T1
Common cause of brachial plexus palsy
obstetric injury
- shoulder dystocia = baby’s shoulder gets stuck on mom’s pelvis causing traction as baby tries to get out
4 possible damages to the nerve in brachial plexus palsy
- neurapraxia (nerve stretched)
- neuroma (scar tissue from stretching)
- rupture (part or all nerve torn but not root)
- avulsion (nerve torn at root=prognosis not good)
What type of BPI injury occurs when C5 and C6 are affected?
Erb’s palsy - impacts shoulder and elbow
What type of BPI injury occurs when C8-T1 are affected?
Klumpke’s palsy - impact wrist and hand “claw hand”
Are musculoskeletal diseases and musculoskeletal disorders the same? Explain
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)
T/F
Musculoskeletal diseases are neuromuscular disorders.
False
3 types of musculoskeletal diseases
- arthrogryposis
- achondroplasia
- osteogenesis imperfect
