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ALS (Progressive S/Sx (Spasticity, Dysarthria, Fatigue, Dysphagia, Atrophy…
ALS
Progressive S/Sx
Spasticity
Dysarthria
Fatigue
Dysphagia
Atrophy
Respiratory Complications
Weakness (cardinal sign)
Epidemiology
Risk Factors
Lead exposure
Military service (metals, chemicals, trauma, viral infections, stress)
Smoking (2x)
Types
Limb-onset: 70-80%
Bulbar-onset: 20-30% (CN IX-XII)
40-70yo most common, little to no risk after 70-80+
Men x1.5-2 > Women
Causes/Etiology
accumulation of misformed proteins
Disorganized immune response
Chemical Imbalance (increased glutamate levels in spinal fluid
Gene Mutation
Diagnosis
Clinical Presentation
EMG testing
Muscle Biopsy
blood/urine/CSF analysis (glutamate)
DD: MG, cervical myelopathy, multifocal motor neuropathy, hypoparathyroidism, inclusion body myosis, bulbospinal nueronopathy, lymphoma, radiation-induced effect
Signs and Symptoms
UMN signs (weakness, spasicity, hyperreflexia, pathological relfexes, psudobulbar affect, sialorrhea
LMN signs (weakness, muslce wasting, muscle cramps, fasciculations and fibrilations)
Other: dementia, sensory dysfunction, oculomotor dysfucntion, autonomic dusfucntion, pain
Managment
Medication (symptom managment)
Riluzole (antiglutamate): slows disease my months
Physical Therapy
Exercise (moderate fro muslces >3/5)
Education