[Neurological complications of acute intermittent porphyria precipitated by porphyrinogenic drugs and efficiency of heme-arginate treatment].
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.


OBJECTIVE
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).


CONCLUSIONS
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.
Akopova-Larbi
Rousanna
R
Service de Neurologie, Hôpital Razi, Manouba, Tunis.
Ben Youssef
Turki Ilhem
TI
Gargouri
Amina
A
Kraoua
Ichraf
I
Gouider
Riadh
R
Gouider-Khouja
Neziha
N
D000208:Acute Disease; D000293:Adolescent; D001120:Arginine / Q000008:administration & dosage / Q000627:therapeutic use*; D004576:Electromyography; D015738:Famotidine / Q000009:adverse effects; D005260:Female; D006418:Heme / Q000008:administration & dosage / Q000627:therapeutic use*; D006419:Heme Oxygenase (Decyclizing); D006801:Humans; D009543:Nifedipine / Q000009:adverse effects; D010523:Peripheral Nervous System Diseases / Q000139:chemically induced / Q000209:etiology*; D010634:Phenobarbital / Q000009:adverse effects; D011164:Porphyrias / Q000150:complications* / Q000188:drug therapy*; D011165:Porphyrinogens / Q000009:adverse effects*; D013997:Time Factors
D011165:Porphyrinogens; D006418:Heme; D015738:Famotidine; D001120:Arginine; D006419:Heme Oxygenase (Decyclizing); D009543:Nifedipine; C048849:heme arginate; D010634:Phenobarbital