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progressive spinal muscular atrophy

One of the subgroups of motor neuron disease; a progressive degenerative disorder of the motor neurons of the spinal cord, manifested as progressive, often symmetrical, weakness and wasting, typically beginning in the distal portions of the limbs, particularly in the upper extremities, and spreading proximally; fasciculation potentials are often present, but evidence of corticospinal tract disease (e.g., increased deep tendon reflexes, Babinski sign) is not.