Pathophysiology of als: What and how much do we know?
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Amyotrophic lateral sclerosis is a progressive neurodegenerative disorder primarily involving motor neurons in the cerebral cortex, brainstem and spinal cord. Most cases are sporadic, although 5-10% is familial, with 10-20% of these being linked to mutations in the enzyme superoxide dismutase-1. The clinical and pathological features of familial and sporadic cases are strikingly similar. In this review, the proposed mechanisms underlying the pathophysiology of ALS are focused with a hypothetical integrative model.