Abstract | INTRODUCTION: 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.
|
Authors | F Moutaouakil, H El Otmani, H Fadel, F Sefrioui, I Slassi |
Journal | Revue neurologique
(Rev Neurol (Paris))
Vol. 165
Issue 5
Pg. 482-5
(May 2009)
ISSN: 0035-3787 [Print] France |
Vernacular Title | La l-arginine, une thérapeutique symptomatique efficace au cours d'un syndrome MELAS. |
PMID | 18849057
(Publication Type: Case Reports, Journal Article)
|
Chemical References |
- Lactates
- Pyruvates
- Arginine
|
Topics |
- Arginine
(therapeutic use)
- Biopsy
- Brain
(diagnostic imaging, pathology)
- Calcinosis
(pathology)
- Child
- Disease Progression
- Humans
- Lactates
(blood)
- MELAS Syndrome
(blood, drug therapy, pathology, physiopathology)
- Male
- Muscle, Skeletal
(pathology)
- Pyruvates
(blood)
- Tomography, X-Ray Computed
|