Artikel
Deep brain stimulation in multiple system atrophy: a clinicopathological report
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Autoren
Veröffentlicht: | 4. Mai 2005 |
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Gliederung
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Objective
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 has been improved by DBS for one year.
Results
A 63 years old parkinsonian patient has been treated by levodopa for 6 years with a persistent good response. He had developed for one year disabling fluctuations with severe axial syndrome and vegetative non motor symptoms in OFF periods. After checking usual contraindications, he was included in surgical procedure (bilateral STN stimulation). During the first year after surgery, the clinical status improved with disappearance of non motor fluctuations, a 45% decrease of the OFF UPDRS III score, and a 35% reduction of the treatment. However after one year, axial symptoms reappeared with recurrent falls, and increasing dysarthry and deglutition difficulties which were only slightly improved by levodopa. He developed severe urinary troubles increased by a prostatic adenoma which led to surgical treatment. During post operative period, 2 years after DBS, he died suddenly for an unexplained reason. A cerebral autopsy was performed and showed a good position of the 2 electrodes in STN. Microscopic studies revealed a severe neuronal depletion in substantia nigra but no Lewy bodies, and immunohistochemical methods demonstrated numerous argyrophilic glial cytoplasmic inclusions positive for alpha synuclein and ubiquitin in STN, putamen, globus pallidus, basis pontis nuclei and cerebellar white matter, significant of MSA.
Conclusions
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.