Young-onset multiple system atrophy

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• 作者：Han-Joon Kim^a ; ^b ; ^c ; ^d ; Beom S. Jeon^a ; ^b ; ^c ; ^d ; ^{brain@snu.ac.kr} ; Jee-Young Lee^e ; Ji Young Yun^a ; ^b ; ^c ; ^d ; Young Eun Kim^a ; ^b ; ^c ; ^d ; Sun Ha Paek^b ; ^d ; ^f ; ^{paeksh@snu.ac.kr}
• 关键词：Multiple system atrophy ; Young-onset
• 刊名：Journal of the Neurological Sciences
• 出版年：2012
• 期刊代码：178_0022510x
• 类别：med
• 出版时间：15 August, 2012
• 卷：319
• 期：1-2
• 页码：168-170
• 文件大小：294 K

摘要

Background

Multiple system atrophy (MSA) rarely begins before the age of 40 and detailed descriptions of young-onset MSA are lacking.

Methods

Among 455 patients included in our MSA cohort, four developed disease before the age of 40. We reviewed the medical records of these patients.

Results

Case聽1 and 2 presented with cerebellar symptoms. Case 1 had clinical features and a course typical of MSA. Case 2 had a rapid course and died 3 years after onset. Case 3 and Case 4 presented with levodopa-responsive parkinsonism. Both developed motor fluctuations and peak-dose limb dyskinesias. Subthalamic deep brain stimulation (DBS) resulted in some improvements in motor symptoms, but they became totally dependent within a few years.

Discussion

Young-onset MSA is rare but does exist. Young-onset MSA with predominant parkinsonism may closely resemble Parkinson disease at onset and is likely to develop motor complications. Attention should be given to the possibility of young-onset MSA in selecting DBS candidates.