Introduction. La stimulation à haute fréquence des noyaux sous-thalamiques n’a jusqu’ici pas montré d’efficacité dans le traitement de l’atrophie multi-systématisée. Observation. Nous rapportons le cas d’un patient âgé de 63 ans qui présentait un syndrome parkinsonien évoluant depuis 6 ans, dopa-sensible, compliqué depuis un an de fluctuations motrices et non motrices. Le diagnostic de maladie de Parkinson idiopathique fut retenu et le patient bénéficia de la mise en place d’une stimulation bilatérale des noyaux sous-thalamiques. L’évolution fut favorable durant la première année : disparition des fluctuations, amélioration de 45 p. 100 du score moteur de l’UPDRS, et diminution de 39 p. 100 du traitement médicamenteux. Secondairement, l’état du patient se dégrada et il décéda brutalement après une intervention sur un adénome prostatique, à l’âge de 65 ans. L’examen neuropathologique conclut au diagnostic d’atrophie multi-systématisée. Conclusion. Ce cas montre que la stimulation cérébrale profonde peut apporter un bénéfice chez les patients atteints d’atrophie multi-systématisée. Néanmoins, son efficacité reste limitée dans le temps et cette technique ne peut être recommandée actuellement dans le traitement de cette affection.

Introduction. Efficacy of high frequency subthalamic nucleus (STN) stimulation has been demonstrated in idiopathic Parkinson’s disease (IPD). However, since it may be difficult to differentiate IPD from multiple system atrophy with parkinsonian presentation (MSA-P), a few cases of MSA-P has been treated by deep brain stimulation (DBS) and showed no sustained improvement of clinical signs. We report a patient with a clinical misdiagnosed MSA-P, later confirmed by neuropathological study, who was improved by DBS for one year. Case report. A 63-year-old parkinsonian patient had been treated by levodopa for 6 years with a persistent good response. Over one year he progressively developed disabling fluctuations with severe axial syndrome and vegetative non motor symptoms in off periods. After checking usual contraindications, he was included in surgical procedure protocol (bilateral STN stimulation). During the first year after surgery, the clinical status improved with disappearance of non motor fluctuations, a 45 percent decrease of the OFF UPDRS III score, and a 39 percent reduction of the treatment. However after one year, axial symptoms reappeared with recurrent falls, as well as increasing dysarthry and swallowing difficulties which were only slightly improved by levodopa. He developed severe urinary disorders increased by a prostatic adenoma which led to surgical treatment. During the post operative period, 2 years after DBS, he died suddenly from an unexplained cause. A cerebral autopsy was performed and showed a good position of the two electrodes in the STN. Microscopic studies revealed severe neuronal depletion in the substantia nigra but no Lewy bodies. Immunohistochemical methods demonstrated numerous argyrophilic glial cytoplasmic inclusions positive for α-synuclein and ubiquitin in the STN, putamen, globus pallidus, pontine nuclei and cerebellar white matter, significant of MSA. Conclusion. This case shows that DBS can improve parkinsonian signs in MSA-P with persistent dopa sensitivity. However, probably because of striatal degeneration progression, this improvement is time limited and STN DBS cannot be recommended in MSA.