Acute porphyria; two cases with autopsy reports

Two cases of acute porphyria are described with particular reference to the general course of the disease and to the findings demonstrable at autopsy. Both cases were characterized by rather typical exacerbations and remissions of symptoms referable primarily to the nervous system and to the gastrointestinal tract. The familial tendency was demonstrated in the first case. Histological study of tissues revealed the most significant changes in the peripheral nerves in which demyelinization was the outstanding feature. Loss of striations in skeletal muscle accompanied the neurological abnormalities. The identity and source of this toxic agent, as well as an interpretation of the pathogenesis of this type of disturbed metabolism, is still being sought.