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Amyotrophic Lateral Sclerosis (Stages & Symptoms (Middle…
Amyotrophic Lateral Sclerosis
Pathophysiology
Motor axonal degeneration
loss of lower motor neurons
loss of innervation of individual motor units
muscle atrophy of distal muscles
Atrophy of proximal muscles
Atrophy of respiratory muscles
Weakness of respiratory muscles
hypoventilation
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UMN signs
Increased DTR's
Pathological Reflexes
Babinski
Spasticity
Bulbar Signs
CN 9-12 involvement
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Dysarthria
Weakness of bulbar musculature (mm of tounge, pharynx, larynx etc)
LMN Signs
Decreased DTRs
Muscle fasciculations
Clinical Diagnosis
Differential Diagnosis
Must rule-out other neurodegenerative diagnoses
MS
Cancer
Myopathy
Peripheral Neuropathy
HIV
Lymes Disease
Diagnostic Testing
Blood Test
Elevated CPK levels due to muscle death
Abnormal EMG
Nerve degeneration
Larger motor unit potentials
widespread fibrillations
Myelogram
MRI
Muscle Biopsy
Spinal Tap
Stages & Symptoms
Middle
Heavy or clumsy legs
Mm fasciculations
Hyperreflexia
Ankle clonus
Exaggerated DTRs
Distal involvement > proximal
Weakness of bulbar mm
CN 9-12
Medulla
Late
Inability to swallow
G-Tube feeding
Aspiration pneumonia
Respiratory Failure
Loss of speech
Complete dependence with ADLs
Early
Patchy distribution of mm weakness
Rapid, progressive weakness
Mm irritability, cramps and spasms