NEUROLOGICAL COMPLICATIONS OF ACUTE INTERMITTENT PORPHYRIA PRECIPITATED BY PORPHYRINOGENIC DRUGS AND EFFICIENCY OF HEME –ARGINATE TREATMENT

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Published Dec 4, 2009
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Vol. 87 No. 9 (2009): Issue Sep 2009

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Rousanna Akopova-Larbi
Ilhem Ben Youssef Turki
Amina Gargouri
Ichraf Kraoua
Riadh Gouider
Neziha Gouider-Khouja

Abstract

Background :Acute intermittent porphyria (AIP) is a rare metabolic disorder of heme biosynthesis characterized by enzymatic defect of porphobiligen desaminase with accumulation and increased excretion of porphyrins and their precursors. Clinical picture is characterized by attacks with a triad of abdominal pain, psychiatric
disorder and neurological involvement (central and peripheral). Peripheral nervous system manifestations, often precipitated by porphyrinogenic medications are of poor outcome.
Aim : We report a new cases A 13-year-old girl who presented several attacks of AIP and developed acute severe axonal motor neuropathy, three weeks after porphyrinogenic medications (Famotidin , Phenobarbital and Nifedipine).
Conclusion : We stress on the importance of early diagnosis of AIP to prevent serious neurological complications often precipitated by medications and the efficiency of heme arginate treatment when administrated early during the attacks.

Keywords:

acute intermittent porphyria, polyneuropathy, porphyrinogenic drugs

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