NEUROLOGICAL COMPLICATIONS OF ACUTE INTERMITTENT PORPHYRIA PRECIPITATED BY PORPHYRINOGENIC DRUGS AND EFFICIENCY OF HEME –ARGINATE TREATMENT
Keywords:
acute intermittent porphyria, polyneuropathy, porphyrinogenic drugsAbstract
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.
Downloads
References
GEROLAMI R., PORTAL I., BOURLIERE M. Porphyries hépatiques. Encycl Med Chir ( Elsevier, Paris), Hépatologie 2000 ; 7-200- A-20 : 8
BAROHN R.J., SANCHEZ J.A., ANDERSON K.E. Acute peripheral neuropathy due to hereditary coproporpyria. Muscle Nerve 1994; 17: 793-799.
NORDMANNY.Porphyries héréditaires. Encycl Med Chir (Elsevier, Paris), Pédiatrie 1999; 04-059- Q-10 : 6 p.
BECKER D.M., KRAMER S. The neurological manifestations of porphyria : a review. Medicine (Baltimore) 1977; 56: 411-423
DEYBACH J.C.Les porphyries, Encyclopedie, Orphanet. Avril, 1999 http://www.orpha.net/data/pato/FR/fr-porphyr.pdf
SUAREZ J.I., COHEN M.L., LARKIN J., ET al. Acute intermittent porphyria : clinicopathologic correlation. Neurologie 1997; 48 : 1678-1683
UTZ N., KINKEL B., HEDDE J.P., BEWERMEYER H. MR imaging of acute intermittent potphyria mimicking reversible posterior leukoencephalopathy syndrome. Neuroradiology 2001; 43: 1059-1062
FRESSINAUD C. PORHYRIES. DANS P. BOUCHE ET G.M. Vallat édition. Neuropathies périphériques : polyneuropathies et mononeuropathies multiples, 1992 : 607-618
ALBERS J.W., FINC J.K. Porphyric neuropathy. Muscle Nerve 2004; 30 : 410-422
BYLESJO I., FORSGREN L., LITHNER F., BOMAN K. Epidemiology and clinical characteristics of seizures in patients with acute intermittent porphyria. Epilepsia 1996; 37: 230-235
KAIDO M., FUKADA K., MORIYA M., et al. Porphyria with double errors in the heme biosynthetic pathway. J. Neurol. 2001; 248: 328-329
HAHN M., GILDEMEISTER O.S., KRAUSS G.L., et al. Effects of new anticonvulsant medications on porphyrin synthesis in cultured liver cells: potential implications for patients with acute porphyria. Neurology 1997; 49: 97-106
LABINER D.L., AHERN G.L., KOLB M.A., et al. Gabapentin is both safe and effective in treating seizures associated with porphyria. Epilepsia 1994; 35, (Suppl). 8: 53
BONKOWSKY H.L., SINCLAIR P.R., EMERY S., SINCLAIR J.F. Seizure management in acute hepatic porphyria: risks of valproate and clonazepam. Neurology 1980; 30: 588-592
KRAUSS G.L., SIMMONS-O'BRIEN E., CAMPBELL M. Successful treatment of seizures and porphyria with gabapentin. Neurology 1995; 45: 594-595
AHLE G., WIERZBA S., HAUPTS M., et al. Acute left hemispheric syndrome with cortical lesions un a patient with secondary porphyrinyria. J. Neurol. 2005; 252: 983-984
LAI C.W., HUNG T.P., LIN W.S. Blidness of cerebral origin in acute intermittent porphyria. Report of a case and postmortem examination. Arch. Neurol. 1977; 34: 310-312
THE GUILLAIN-BARRÉ SYNDROME STUDY GROUP. Plasmapheresis and acute Guillain-Barré syndrome. Neurology 1985; 35: 1096-1104
KING P.H., BRAGDON A.C. MRI reveals multiple reversible cerebral lesions in an attack of acute intermittent porphyria. Neurology 1991; 41: 1300-1302
