Neurologic aspects of porphyria

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Abstract
A report is presented of 2 patients with acute intermittent porphyria and involvement of the nervous system. The clinical picture was that of progressive, peripheral polyneuropathy, predominatly motor in its manifestations. In 1 patient, diffuse cerebral involvement occurred. Both patients died of respiratory paralysis. The pathologic changes were those of a nonspecific parenchymatous neuropathy with associated spinal root involvement of a lesser degree. In the central nervous system, axonal chromatolysis of cells occurred in the anterior horn and vagal nuclei. Nonspecific changes took place which were consistent with respiratory death. The pathophysiology is considered to be an abnormality in the metabolism of porphyrin and purine compounds. Resulting disturbance of intracellular enzymes is reflected in widespread functional nervous system changes.

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