Blindness of cerebral origin in acute intermittent porphyria. Report of a case and postmortem examination.
Sudden permanent blindness of cerebral origin, in addition to severe abdominal pain, hypertension, convulsions, and peripheral neuropathy developed in a 21-year-old woman, a victim of acute intermittent porphyria. Findings of the pathological examination of the brain showed extensive infarction in both occipital lobes. The pathological changes were consistent with anoxia. We discuss and review the literature of the possibility of "vasospasm" of both posterior cerebral arteries. Follow-up studies with serial EEG showed either focal epileptogenic activity or diffuse slow waves. The most consistent epileptic discharges were found in the occipital regions. The favorable response to the treatment of seizures with carbamazepine in this patient might encourage further clinical trials.
D000328:Adult; D001766:Blindness / Q000209:etiology*; D001921:Brain / Q000473:pathology; D002220:Carbamazepine / Q000627:therapeutic use; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D005260:Female; D006801:Humans; D006973:Hypertension / Q000150:complications; D010243:Paralysis / Q000209:etiology; D010523:Peripheral Nervous System Diseases / Q000209:etiology*; D011164:Porphyrias / Q000150:complications* / Q000473:pathology; D014826:Vocal Cord Paralysis / Q000209:etiology
D002220:Carbamazepine