BAL Therapy of Acute Porphyrinuria

Abstract

A 52 year old woman with acute porphyria and neurological symptoms including a recurrent toxic psychosis, bulbar cranial nerve dysfunction, severe peripheral neuropathy and hypertension showed a progressive downhill course despite pheno-barbital withdrawal, supportive measures and ACTH therapy. Dramatic recovery followed the institutionof BALtherapy (2,3-dimer-captopropanol) when death seemed imminent. Administration of BAL was followed by cessation of abnormal porphyrin excretion (uroporphyrin and porphobilinogen). Upon withdrawal of BAL the uroporphyrins reappeared in the urine but promptly disappeared with resumption of BAL. Initially BAL was administered in dosage of .5 cc. 10% solution 3 times daily for 12 days (180 mg.) The second course of BAL included 80 mg. over a 4 day period. Clinical recovery was complete with maintenance weekly injections of BAL. Abnormal porphyrins are excreted in urine and feces as a Zn metal complex. The possibility is suggested that BAL may act by removal of Zn or another heavy metal thus preventing further cellular metabolic deficit resulting when the porphyrins are withdrawn from the enzyme system and excreted in the urine. Further study of BAL therapy in both acute and chronic porphyria is urged.