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Amyotrophic Lateral Sclerosis (ALS) (Epidemiology (Diagnosis made by…
Amyotrophic Lateral Sclerosis (ALS)
Etiology
Largely Unknown
Sporadic ALS (90%)
Probably Genetic & Environmental (Multi-factorial)
Familial ALS (5-10%)
-Mutation in more than a dozen genes suspected
-chromosome 9 open reading frame 72 (C9ORF72) gene
-superoxide dismutase 1 (SOD1) gene
Epidemiology
-As at 2016, 14,000-15,000 Americans have ALS
-2-3 per 100,000 of the US population
-Symptoms commonly develop between ages 55-75
-Incidence is slightly higher in men than women
-Caucasians and non-hispanics most at risk
-Listed as a military service connected disease by the VA
Diagnosis made by eliminating other conditions
-Nerve Conduction Velocity (NCV) test
-Electromyography (EMG) test
-Magnetic Resonance Imaging (MRI)
-Blood tests
-Lumbar puncture
Muscle biopsy (done if presentation is atypical)
Treatment & Management
-Focus is on maintaining quality of life
-Control symptoms
-Prevent Complications
-Providing adaptive devices to increase mobility and optimize self care
Collaborative Team
-Dietician
-Physical therapy
-Occupational & speech therapy
-May need respiratory care
Medication
-Riluzole (Rilutek)
Effective in slowing disease progression
Antispasmodics
-Baclofen (Lioresal)
-Dantrolene Sodium (Dantrium)
-Diazepam (Valium)
NSAIDS & Analgesics
-Ibuprofen
-Naproxen
Tramadol (Ultram)
Antidepressants recommended, high incidence of depression with ALS
Clinical Manifestations
-Difficulty swallowing (dysphagia)
-Difficulty chewing
-Slurred and nasal speech (dysarthria)
-Fatigue
--Muscle cramps
-Tight and stiff muscles (spasticity)
Fasciculations (muscle twitches)
-Arms
-Legs
-Shoulder
-Tongue
Muscle Weakness
-Arms
-Legs
-Neck
-Diaphragm