Young-onset multiple system atrophy

被引:18
作者
Kim, Han-Joon [1 ,2 ,3 ,4 ]
Jeon, Beom S. [1 ,2 ,3 ,4 ]
Lee, Jee-Young [5 ]
Yun, Ji Young [1 ,2 ,3 ,4 ]
Kim, Young Eun [1 ,2 ,3 ,4 ]
Paek, Sun Ha [2 ,4 ,6 ]
机构
[1] Seoul Natl Univ Hosp, Coll Med, Dept Neurol, Seoul 110744, South Korea
[2] Seoul Natl Univ Hosp, Coll Med, Dept Movement Disorder Ctr, Seoul 110744, South Korea
[3] Seoul Natl Univ Hosp, Coll Med, Parkinson Dis Study Grp, Seoul 110744, South Korea
[4] Seoul Natl Univ Hosp, Coll Med, Neurosci Res Inst, BK21, Seoul 110744, South Korea
[5] Seoul Natl Univ, Boramae Hosp, Dept Neurol, Seoul 110744, South Korea
[6] Seoul Natl Univ Hosp, Coll Med, Dept Neurosurg, BK21, Seoul 110744, South Korea
关键词
Multiple system atrophy; Young-onset; PROGRESSION; MSA;
D O I
10.1016/j.jns.2012.04.016
中图分类号
R74 [神经病学与精神病学];
学科分类号
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 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 levodoparesponsive 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. (C) 2012 Elsevier B.V. All rights reserved.
引用
收藏
页码:168 / 170
页数:3
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