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Tijdschrift voor Psychiatrie 45 (2003) 5, 277 - 286

Review article

Acute porphyria: an intriguing imitator

I.M. Holslag, W. Snelleman, H.W. Hoek

background  The acute porphyrias deserve attention in mental health care because clinical manifestations may include a variety of psychiatric symptoms and sometimes psychiatric symptoms are in fact the only manifestations.
  To review the literature on the epidemiology, pathogenesis, diagnosis and treatment.
  The literature was reviewed with the help of Medline and the Cochrane Library, over the period from 1990 till June 2002, using as key words acute porphyria, incidence, prevalence, diagnosis, pharmaceutical preparations, psychopharmacology, mental disorders, depression, affective symptoms, psychotic disorders, and biological psychiatry. Also, cross references were included and some reference books.
results  Acute porphyria is a relatively rare disease. However, there is a growing need for acute porphyria to be diagnosed at the earliest opportunity because of the increasing use of (psychotropic) drugs that can cause iatrogenic attacks of porphyria. Acute attacks of neuropsychiatric porphyria cause considerable morbidity and even mortality. Since the administration of haem arginate can bring about remission, a prompt and accurate diagnosis is essential. The acute type of porphyria can be diagnosed by measuring porphyrin levels in urine, feces and plasma. Relatives of patients with acute porphyria should be screened so that gene carriers can avoid risk factors. Carriers of acute porphyria genes should not use porphyrinogenic (psychotropic) drugs.
  Acute porphyria should be considered as a possible diagnosis in patients who present with atypical, episodic, somatic and/or psychiatric symptoms, especially when these are combined with abdominal or back pain.

keywords acute porphyria, diagnosis, psychiatric symptoms, treatment