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Amyotrophic Lateral Sclerosis

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.

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Amyotrophic Lateral Sclerosis

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  1. Amyotrophic Lateral Sclerosis

  2. 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

  3. 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

  4. 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

  5. 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

  6. Treatment • Riluzole: glutamate moderation presynaptically and postsynaptically

  7. 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

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