[l-arginine efficiency in MELAS syndrome. A case report]. / La l-arginine, une thérapeutique symptomatique efficace au cours d'un syndrome MELAS.

INTRODUCTION: Mitochondrial encephalomyopathy lactic acidosis and stoke-like episodes (MELAS) is a rare neurodegenerative disease caused by mutations of mitochondrial DNA. CASE REPORT: We report the case of a 12-year-old child with MELAS syndrome who presented with recurrent migraine-like headache and sudden blindness suggesting stroke-like episodes. Furthermore, he developed progressive muscular impairment with bilateral hearing loss. Serum lactate and pyruvate levels were elevated and the muscle biopsy showed an aspect of red-ragged fibers with Gomori trichrome. Brain imaging showed calcifications of basal ganglia on the CT scan and a parieto-occipital high signal on diffusion-weighted MRI. A genetic analysis was not performed but the presence of hearing loss in the patient's mother was suggestive of maternal transmission. Stroke-like episodes in the form of migraine-like headache and blindness were the patient's major complaint and did not improve despite analgesic drugs. After oral administration of l-arginine at the dose of 0.4mg/kg per day, stroke-like symptoms totally and rapidly disappeared. DISCUSSION: The efficiency of l-arginine in stroke-like episodes was initially reported then confirmed in a controlled study. The pathophysiology of stoke-like episodes and the mechanisms underlying the action of l-arginine are discussed.

[Epidermoid cysts of the spleen. Report of 6 cases]. / Kystes épidermoïdes de la rate. A propos de 6 observations.

The authors report 6 cases of epidermoid cysts of the spleen. They emphasize the rarity of the lesion, its pathological and clinical features, and describe the abnormalities which can be noted on plain films, ultrasonography and computed tomography.