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NMD 2 Neuro || with Accurate Answers 100%.

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Charcot-Marie-Tooth disease correct answers Involves both sensory and motor nerve fibers Weakness and numbness usually starts in feet - UE weakness/atrophy begins later Characteristic foot deformity - high arch and hammertoes Foot srop common CMT: PT interventions correct answers Shoe a...

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NMD 2 Neuro || with Accurate Answers 100%.
Charcot-Marie-Tooth disease correct answers Involves both sensory and motor nerve fibers
Weakness and numbness usually starts in feet
- UE weakness/atrophy begins later
Characteristic foot deformity
- high arch and hammertoes
Foot srop common

CMT: PT interventions correct answers Shoe and orthotic intervention and gait training
Orthopedic surgery:
- straighten toes
- lengthen heel cord
- lower arch of foot

Exercise interventions to maintain strength in residual muscles
Balance interventions
Energy conservation

ALS correct answers Progressive degeneration of motor cells in spinal cord, brainstem and brain
- gives rise to both UMN and LMN s/s

Likely caused by intersection of certain genetic/environmental factors
- 90% idiopathic, 10 percent familial

ALS pathophysiology: LMN correct answers LMN symptoms:
- atrophy and weakness
- muscle cramps
- muscle fasciculations
- diminished reflexes, hypotonicity
What's happening:
- massive loss of anterior horn cells in spinal cord
- massive loss of motor cranial nerve nuclei (CN V, VII, IX, X, XII)

ALS pathophysiology: UMN correct answers - Betz cells in motor cortex degenerate
- Demyelination and gliosis of corticospinal and corticobulbar tracts occur after nerves due
- Proliferation of astrocytes and scarring of lateral columns of spinal cord

Symptoms may include: spasticity, hyperreflexia, muscle weakness

UMN and LMN involvement occurs simultaneously in ALS correct answers Disease tends to
spread in contiguous manner, involving spinal segments with close anatomical proximity initially

Diagnostic criteria for ALS correct answers All 4 must be present

- s/s of LMN degeneration

, - s/s of UMN degeneration
- steady progression of symptoms in 1 body region and ultimately other body regions
- exclusion of symptoms inconsistent with ALS (Electrophysiological or neuroimaging evidence
of other disease processes)

Pattern of weakness with ALS correct answers Focal weakness usually first symptom

Asymmetrical pattern of weakness is distinguishing feature
- could begin in UE, LE, or bulbar muscles
- marked atrophy as disease progresses

Sparing of certain pathways
- CN nuclei for nerves III, IV, VI
- neural control of striated muscles associated with the urethral and anal sphincters

ALS: bulbar and respiratory involvement correct answers Can see either flaccid or spastic bulbar
palsy
- dysarthria
- dysphagia

Respiratory weakness leads to fatigue, dyspnea, ineffective cough

Accessory muscles used for breathing

ALS: symptoms correct answers As muscle fibers denervate, muscle cramping and/ or
fasciculation may occur
- thought to result from hyper excitability of motor neurons
- hands and feet especially
- usually worse in more involved muscles

Sensation generally NOT involved

ALS: Cognitive impairment correct answers 25% of cases there us some degree of
frontotemporal lobar degeneration

Sometimes see pseudobulbar affect: bouts of uncontrollable laughter or crying

ALS: pharmacologic intervention correct answers 4 drugs approved by FDA:
- Riluzole: glutamate agonist
- Tiglutik
- Radicava: thought to counteract excessive oxidative stress leading to motor neuron death
- Nuedexta: treats pseudobulbar affect

ALS outcome measures correct answers Muscle performance
Gait and balance
Cranial nerve screening

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