heme-arginate and Vasospasm--Intracranial

Acute intermittent porphyria (AIP) is an autosomal-dominant disease caused by a deficiency of porphobilinogen (PBG) deaminase. Patients with AIP present with neurological syndromes such as autonomic neuropathy, peripheral axonal neuropathy or central nervous system dysfunction. We report serial MRI of a patient with AIP who had cortical and subcortical cerebral changes. A 29-year-old woman with a 6-month history of AIP had an attack with severe hyponatraemia and generalised convulsions, treated with haem arginate and supportive therapy. MRI showed central pontine and extrapontine myelinolysis and cortical laminar necrosis. These are not common in AIP, but are likely to have been caused by rapid correction of hyponatraemia and by vasospasm, which could be induced by AIP.

Topics: Adult; Arginine; Brain; Female; Fluid Therapy; Heme; Humans; Hyponatremia; Myelinolysis, Central Pontine; Necrosis; Porphyria, Acute Intermittent; Saline Solution, Hypertonic; Seizures; Vasospasm, Intracranial