Literature DB >> 8657842

Porphyria: reexamination of psychiatric implications.

K Burgovne1, R Swartz, J Ananth.   

Abstract

Acute intermittent porphyria mimics a variety of commonly occurring disorders and thus poses a diagnostic quagmire. Psychiatric manifestations include hysteria, anxiety, depression, phobias, psychosis, organic disorders, agitation, delirium, and altered consciousness ranging from somnolence to coma. Some patients develop psychosis similar to schizophrenia. Psychiatric hospitals have a disproportionate number of patients with this disorder as only difficult and resistant patients accumulate there. Presence of photosensitive porphyrins in the urine is diagnostic. When porphyrins are absent, excess of alpha aminolevulinic acid and porphobilinogen are present in the urine. The definitive test is to measure monopyrrole porphobilinogen deaminase in RBCs. This diagnosis should be entertained in the following situations: (a) unexplained leukocytosis; (b) unexplained neuropathy; (c) etiologically obscure neurosis or psychosis; (d) 'idiopathic' seizure disorder; (e) unexplained abdominal pain; (f) conversion hysteria, and (g) susceptibility to stress. Porphyria is important in psychiatry as it may present with only psychiatric symptoms; it may masquerade as a psychosis and the patient may be treated as a schizophrenic person for years; the only manifestation may be histrionic personality disorder which may not receive much attention. Diagnosis is based on a high index of suspicion and appropriate investigation. Various psychotropic drugs exacerbate acute attacks. While it is important not to use the unsafe drugs in porphyric patients, it is also imperative to look for this diagnosis in cases where these drugs produce unprecedented drug reactions.

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Year:  1995        PMID: 8657842     DOI: 10.1159/000289001

Source DB:  PubMed          Journal:  Psychother Psychosom        ISSN: 0033-3190            Impact factor:   17.659


  8 in total

1.  Anxiety and depression in the acute porphyrias.

Authors:  L M Millward; P Kelly; A King; T J Peters
Journal:  J Inherit Metab Dis       Date:  2005       Impact factor: 4.982

2.  Self-rated psychosocial consequences and quality of life in the acute porphyrias.

Authors:  L M Millward; P Kelly; A Deacon; V Senior; T J Peters
Journal:  J Inherit Metab Dis       Date:  2001-12       Impact factor: 4.982

Review 3.  Psychiatric symptoms of inherited metabolic disease.

Authors:  Y Estrov; F Scaglia; O A Bodamer
Journal:  J Inherit Metab Dis       Date:  2000-02       Impact factor: 4.982

Review 4.  Psychiatric Aspects of Acute Porphyria: a Comprehensive Review.

Authors:  Laura Duque-Serrano; Liliana Patarroyo-Rodriguez; Dorothy Gotlib; Juan C Molano-Eslava
Journal:  Curr Psychiatry Rep       Date:  2018-02-02       Impact factor: 5.285

5.  Schizoaffective disorder with missed diagnosis of acute porphyria: a case report and overview.

Authors:  Gaurav Jain; Jeffrey I Bennett; David S Resch; John E Godwin
Journal:  Prim Care Companion CNS Disord       Date:  2011

6.  An analysis of six cases of acute intermittent porphyria (AIP).

Authors:  Soumitra Ghosh; Pranit Kr Chaudhury; Hiranya K Goswami
Journal:  Indian J Psychiatry       Date:  2006-07       Impact factor: 1.759

Review 7.  Psychiatric signs and symptoms in treatable inborn errors of metabolism.

Authors:  S Nia
Journal:  J Neurol       Date:  2014-09       Impact factor: 4.849

8.  Severe hydroxymethylbilane synthase deficiency causes depression-like behavior and mitochondrial dysfunction in a mouse model of homozygous dominant acute intermittent porphyria.

Authors:  Stefanie Berger; Miranda Stattmann; Ana Cicvaric; Francisco J Monje; Pierluca Coiro; Matej Hotka; Gerda Ricken; Johannes Hainfellner; Susanne Greber-Platzer; Makiko Yasuda; Robert J Desnick; Daniela D Pollak
Journal:  Acta Neuropathol Commun       Date:  2020-03-20       Impact factor: 7.801

  8 in total

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