MANAGEMENT OF NEUROLOGIC EXACERBATIONS OF HEPATIC PORPHYRIA

The porphyrias are dominantly inherited disorders of heme biosynthesis, and their clinical expression in exacerbation is often dependent upon the fact that the patient with undiagnosed porphyria is inadvertently exposed to known toxic medications. Recently, a more detailed understanding of the biosynthetic pathways and the means of inhibiting production of aminolevulinic acid synthetase has resulted both in the earlier recognition of presymptomatic porphyria and also the means of direct intervention in an evolving porphyric crisis. Thus, the knowledgeable physician can now prevent exacerbations of porphyria, can treat abdominal and emotional crises, and can significantly limit the neurologic damage arising in this situation. The future should bring a meaningful reduction of morbidity and of the mortality rate of hepatic porphyria in exacerbation.