Literature DB >> 17136412

[Acute intermittent porphyria. A clinical chameleon: case study of a 40-year-old female patient].

M Zimmermann1, C Bonaccurso, C Valerius, G F Hamann.   

Abstract

Acute porphyrias are rare, but often misdiagnosed and may take a dramatic clinical course. The combination of various internal, psychiatric and neurological symptoms can mimic different other diseases. We report a 40-year-old female patient who was admitted with a subacute tetraparesis. During the last 2 months the patient was treated several times because of abdominal pain and just before admission to our clinic in a psychiatric hospital because of acute mental changes and hallucinations. The typical combination of abdominal pain, motor neuropathy and psychiatric symptoms confirmed by increased amounts of porphyrins and their precursors, led us to promptly diagnose acute intermittent porphyria. Better knowledge about the pathogenesis has clearly improved the prognosis of acute porphyria. In remission, measurement of enzyme activities or mutation screening can be the only diagnostic verification. A mutation screening for family members should be conducted to identify symptom-free carriers, especially in cases of a positive family history.

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Year:  2006        PMID: 17136412     DOI: 10.1007/s00115-006-2224-6

Source DB:  PubMed          Journal:  Nervenarzt        ISSN: 0028-2804            Impact factor:   1.214


  12 in total

Review 1.  The acute porphyrias.

Authors:  N Gordon
Journal:  Brain Dev       Date:  1999-09       Impact factor: 1.961

2.  Diffusion-weighted magnetic resonance imaging of porphyric encephalopathy: a case report.

Authors:  Pao-Sheng Yen; Chi-Jen Chen; Chun-Chung Lui; Yau-Yau Wai; Yung-Liang Wan
Journal:  Eur Neurol       Date:  2002       Impact factor: 1.710

Review 3.  Porphyrias.

Authors:  Raili Kauppinen
Journal:  Lancet       Date:  2005 Jan 15-21       Impact factor: 79.321

4.  Hormonal oral contraceptives, urinary porphyrin excretion and porphyrias.

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Journal:  Horm Metab Res       Date:  1995-08       Impact factor: 2.936

5.  A gonadotropin releasing hormone analogue prevents cyclical attacks of porphyria.

Authors:  K E Anderson; I M Spitz; C W Bardin; A Kappas
Journal:  Arch Intern Med       Date:  1990-07

6.  Motor neuropathy in porphobilinogen deaminase-deficient mice imitates the peripheral neuropathy of human acute porphyria.

Authors:  R L Lindberg; R Martini; M Baumgartner; B Erne; J Borg; J Zielasek; K Ricker; A Steck; K V Toyka; U A Meyer
Journal:  J Clin Invest       Date:  1999-04       Impact factor: 14.808

7.  Delta amino levulinic acid. Effect of a porphyrin precursor on an isolated neuronal preparation.

Authors:  H N Dichter; L Taddeini; S Lin; G F Ayala
Journal:  Brain Res       Date:  1977-04-22       Impact factor: 3.252

Review 8.  Alcohol and porphyrin metabolism.

Authors:  M O Doss; A Kühnel; U Gross
Journal:  Alcohol Alcohol       Date:  2000 Mar-Apr       Impact factor: 2.826

Review 9.  The little imitator--porphyria: a neuropsychiatric disorder.

Authors:  H L Crimlisk
Journal:  J Neurol Neurosurg Psychiatry       Date:  1997-04       Impact factor: 10.154

10.  Liver transplantation as a cure for acute intermittent porphyria.

Authors:  Zahir F Soonawalla; Taner Orug; Michael N Badminton; George H Elder; Jonathan M Rhodes; Simon R Bramhall; Elwyn Elias
Journal:  Lancet       Date:  2004-02-28       Impact factor: 79.321

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  1 in total

1.  Gene expression imputation across multiple brain regions provides insights into schizophrenia risk.

Authors:  Laura M Huckins; Amanda Dobbyn; Douglas M Ruderfer; Gabriel Hoffman; Weiqing Wang; Antonio F Pardiñas; Veera M Rajagopal; Thomas D Als; Hoang T Nguyen; Kiran Girdhar; James Boocock; Panos Roussos; Menachem Fromer; Robin Kramer; Enrico Domenici; Eric R Gamazon; Shaun Purcell; Ditte Demontis; Anders D Børglum; James T R Walters; Michael C O'Donovan; Patrick Sullivan; Michael J Owen; Bernie Devlin; Solveig K Sieberts; Nancy J Cox; Hae Kyung Im; Pamela Sklar; Eli A Stahl
Journal:  Nat Genet       Date:  2019-03-25       Impact factor: 38.330

  1 in total

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