Amyotrophic Lateral Sclerosis

ALS: What is It? Neurodegenerative Affects CNS Affects Motor Pathways Types: Sporadic and Familial Onset: 40-70 years of age Prognosis: 80% will die within 3-5 years of onset

Symptoms Muscle fatigue and weakness in limbs, hands, and muscles of speaking and breathing Manifestations in tripping, slurred speech, quiet voice projection, difficulty in everyday motor tasks using fine detail or expending muscle energy Later in the Disease progression: partial or full paralysis, inability to speak or breath

Biology: Cell Level Mitochondria: influx of SOD1 triggering apoptosis Glutamate: secretion of too much glutamate leads to toxic over-excitation of postsynaptic neuron Genetics- in as many a 15% of cases scientists have found a specific gene mutation causing RNA to make proteins clump Inflammation: damaged cells attract TNF alpha messengers from the immune system Axons: protein malfunction in axons affect structure and shuttling of molecules up and down its length

Biology IGF and GH: imbalance of growth hormones found in ALS patients. Apoptosis: cell suicide and cell death much higher in ALS patients due to damages cells Grey Matter: reduced volume of grey matter especially in front and temporal lobes and precentral gyrus

Treatment Riluzole: glutamate moderation presynaptically and postsynaptically

Future Treatments Gene therapy: targets SOD1 RNA therapy: matched RNA to prevent production of bad protein Current Drugs: insulin therapy and antibiotic therapy for glutamate Stem cell therapy: replace damaged cells