Week 3 - Neuromuscular Flashcards by Ashley Pacheco

Q

ALS affects what neuron?

A

upper and lower motor neurons

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Q

what are some examples of a:

motorneuron disease:

neuromuscular junction disease:

muscle disease:

A

motorneuron disease:
- ALS

neuromuscular junction disease:
- Myesthenia Gravis

muscle disease:
- Muscular Dystrophy

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Q

__________ are a group of progressive neurological disorders that destroy motor neurons

A

motor neuron diseases

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Q

what is the epidemiology of ALS?

A

2500-3000 of Canadians live with ALS

more common in men

more common in elders

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Q

true or false?

people born will ALS last longer?

A

true because they have a slower progression of the disease

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Q

Amyotrophic means what?

Lateral means what?

Sclerosis means what?

A

A = No
Myo = muscle
Trophic = nourishment

“no-muscle-nourishment”

Lateral: area of the spinal cord where the nerve cells innervate the hand

Sclerosis: hardening, the result if degeneration

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Q

____ of people with ALS die within the 2-5 years

A

80%

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Q

_____ live > 20 yrs

A

5%

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Q

_____% of ALS cases are hereditary
________% of ALS cases is sporadic/ spontaneous

A

10%
90%

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Q

true or false ALS is difficult to diagnose?

A

true because symptoms are similar to other neuromuscular disorders

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Q

how to diagnose ALS?

A

EMG
MRI
Nerve conduction

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Q

is it a upper motor neuron or lower?

weakness

A

both upper and lower

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Q

is it a upper motor neuron or lower?

atrophy present

A

lower

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Q

is it a upper motor neuron or lower?

fasciculations (twitches)

A

lower

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Q

is it a upper motor neuron or lower?

increased reflexes

A

upper

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Q

is it a upper motor neuron or lower?

decreased reflexes

A

lower

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Q

is it a upper motor neuron or lower?

increased tone unless acute injury

A

upper

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Q

is it a upper motor neuron or lower?

decreased tone

A

lower

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Q

with someone who has a motor neuron disease large or small mV

A

large (5mV)

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Q

what are some early symptoms of ALS?

A

fasciculations: -
- spontaneous muscle twitching
- slight muscle weakness
- cramping

it takes alot of muscle cells to start seeing signs; 58% of motor neurons may be lost before noticeable symptoms

limb onset: 75%
- tripping, stumbling
bulbar onset:
- symptoms in face- slurred speech
- affect brainstem first

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Q

what are some progressive symptoms of ALS?

A

dysphagia
dysarthria
hyperreflexia
spasticity
weakness + atrophy
fasciculations

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Q

what are some late stage symptoms of ALS?

A

complete loss of voluntary movement
weakness in ventilatory muscles
cognition is unaffected

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Q

can you die from ALS if so how?

A

YES
1. diaphragm
2. swallowing muscles

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Q

what are the treatment options of ALS

A

non really its incurable

there is Riluzole (only FDA approved)
- because it has some ability to remove excess glutamate excitation

what types of therapies help ALS

physical therapy occupational therapy speech therapy

_______ results in a neurotransmitter problem that is an example of a neuromuscular junction

myasthenia gravis

_____ is an auto immune disorder that affects the neuromuscular junction

myasthenia gravis

________ - impacts only 200/ million - lead to muscle weakness + fatigability - affects young women (20 & 30's) or older men (60-70's)

myasthenia gravis

what are the causes of myasthenia gravis

- Antibodies bind to Ach receptors (block receptor action) - destruction of receptor proteins - alters the ability to stimulate muscle cells from motorneuron activity

______ normally breaks down ACh to end muscle contraction

AChE acetylcholine esterase

what is the hallmark MG (myasthenia gravis)

fatiguability (fatigue quickly)

the following are symptoms of: - muscles weaker during periods of activity - muscles affecting eyelid, facial expression, swallowing, breathing muscles

MG (myasthenia gravis)

how many classes of myasthenia gravis are there

5

myasthenia gravis create what eye symptoms

ptosis = eye lid drooping diplopia = double vision

'myasthenic crisis' means

you cant breathe which is the greatest concern for myasthenia gravis

______ of people with MG (myasthenia gravis) experience myasthenia gravis in the frst 2 years

20%

how to diagnose Myasthenia gravis ?

blood tests: - ACH receptor antibodies neuro exam - test fatigue/ weakness Images (MRI, CT) - thymoma, cancer of thymus Medical history

thymoma, cancer of thymus is tested why

it is highly correlated to Myasthenia gravis high % of people have both

what tests can be done to diagnose Myasthenia gravis

1. simpsons test: - hold upward gaze no head movement to test fatiguability of the levator muscles positive test: increased ptosis (eye lid drooping) 2. ice pack test: ice pack on pt's closed eye for 2 mins positive test: improvement in ptosis 3. tensilon test - intravenous administration of acetylcholinesterase inhibitor which improves rapidly to reduce weakness

SLIDE 43 TO 45 DAY 2 NEUROMUSCULAR DISORDERS

SLIDE 43 TO 45 DAY 2 NEUROMUSCULAR DISORDERS

Treatments of Myasthenia gravis

1. acetylcholinesterase inhibitor 2. drugs altering immune system limited and long time

Pyridostigmine is an example of what

acetylcholinesterase inhibitor

________ group of muscle cell diseases that are genetically inherited chronically progressive NS intact most= multisystem disorders

muscular dystrophy

what is the most well known muscular dystrophy?

duchenne muscular dystrophy

what are the causes of Duchenne MD?

- genetic disorder = problem with protein structure within muscle - mutation of DMD gene on X chromosome - lacks the protein dystrophin

the lack of dystrophin protein means what?

muscle breakdown muscle death muscle is replaced by fat and fibrotic tissue

________ provides structural link between muscle cyotoskeleton and ECM (extracellular matrix)

dystrophin

dystrophin helps with

- maintaining muscle integrity

intial symptoms of Duchenne MD occur at what age

between 3 and 5

what are the early signs of Duchenne MD

- enlarged calf muscles - difficulty climbing stairs/ standing unassisted weakness in limbs + pelvis is evident

by age ____ most are dependent on a wheelchair

12

what is the life expectancy of Duchenne MD

early teens to early 30's

symptoms of Duchenne MD

Contractures Pseudohypertrophy Fatigue Abnormal gait (forefoot walk, balls of feet) Frequent falls Increased lumbar lordosis

Duchenne MD symptoms

- fatigue - abnormal gait - frequent falls - difficulty running, hopping, jumping - increased lumbar lordosis

true or false inactivity makes Duchenne MD symptoms worse

true

_____ maneuver is used by individuals with MD to stand up and is seen in _______

gowers' maneuver children

why do children use gowers' maneuver

contractures no dorsiflexion no quad strength

how to diagnose Duchenne MD

blood test genetic screening muscle biopsy EMG (not really used)

is there a cure for Duchenne MD?

no cure but physical therapy is used to manage symptoms + use of braces + surgery if needed + emerging gene therapies (new)