Which porphyrias are associated with primarily neurologic manifestations?
Hepatic porphyrias, acute intermittent porphyria (AIP), and variegate (South African) porphyria can be distinguished from the rare “erythropoietic” forms that produce dermatologic symptoms without neurologic disease.
In AIP, clinical symptoms develop during crises, most often precipitated by ingestion or administration of drugs that adversely affect porphyrin metabolism.
These clinical symptoms include the following:
(1) abdominal pain with vomiting, constipation or diarrhea, and often a previous history of exploratory abdominal surgery;
(2) psychiatric disorder, with symptoms suggesting conversion reactions, delirium, or psychosis;
(3) peripheral neuropathy, primarily motor, often with autonomic abnormalities, that may be severe or fatal and mimic Guillain–Barré syndrome;
(4) central abnormalities, such as syndrome of inappropriate antidiuretic hormone or convulsions.