IPHY 2420 Lecture Notes - Lecture 25: Stephen Hawking, Riluzole, Idiopathy
15 Aug 2016
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Causes and types: causes unknown - idiopathic, two strains: 2-3 years, or 10 years or longer (steven hawking ridiculous case, sporadic, some of a genetic link (but only 10% of cases, multifactorial: genetic, biological, and environmental causes. Symptoms: requires destruction of 1/3 of motor neurons before atrophy begins, and symptoms are noticeable, treatment is on controlling symptoms, abnormalities with the neurotransmitter glutamate, so the one drug is riluzole (anti-glutamatergic agent) Pathophysiology: dead neurons are replaced with non-functional fibrous cells, neurons no stimulate muscle action no message getting through because of non-functional cells, muscles atrophy. Clinical signs: asymmetric muscle weakness and atrophy, hyperrflexia (stiffening of the muscles, uncontrolled twitching of the muscles, increasing paralysis, mechanical ventilation most people die of pneumonia because chest muscles = paralyzed, nutritional support. Medical issues: drug: riluzole 2-3 months longer lives, peg (tube feeding because can"t swallow) early, hypermetabolism: insufficient amounts of energy eating own body, muscle spasms, depression/anxiety.
