Clinical and pathological characteristics of later onset multiple system atrophy.
Clinical and pathological characteristics of later onset multiple system atrophy.
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DOI：
10.1007/s00415-022-11067-1
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发表时间：
2022-08
影响因子：
6
通讯作者：
中科院分区：
文献类型：
--
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在目前的共识标准中，75岁以后发病被认为不支持多系统萎缩（MSA）的诊断;然而，一些MSA患者在75岁以后出现。与通常发病的MSA（UO-MSA）相比，这种迟发性MSA（LO-MSA）的临床和病理特征仍然知之甚少。临床队列包括来自科比大学医院和天崎总医疗中心医院的患者，而尸检队列来自马约诊所佛罗里达的脑库。我们确定了临床队列中的83例患者和尸检队列中的193例患者。我们根据发病年龄将MSA分为两组：UO-MSA（≤ 75岁）和LO-MSA（> 75岁）。我们在临床队列中比较了两组之间的临床特征和结局，并将结果与尸检队列进行了比较。LO-MSA在临床队列中占8%，在尸检队列中占5%。在两个队列中，LO-MSA组从发病至死亡或至挽救生命的气管切开术的中位时间均显著短于UO-MSA组（临床队列为4.8 vs 7.9年，尸检队列为3.9 vs 7.5年; P = 0.043和P < 0.0001）。在临床队列中，LO-MSA中从诊断到死亡的中位时间小于3年。一些MSA患者发病年龄晚，生存期短，限制了临床决策的时间。MSA应考虑在老年患者的自主神经症状和锥体外系和/或小脑综合征的鉴别诊断。
In the current consensus criteria, onset after age 75 is considered as non-supporting for diagnosis of multiples system atrophy (MSA); however, some MSA patients present after age 75. Clinical and pathological characteristics of such later onset MSA (LO-MSA) compared to usual onset MSA (UO-MSA) remain poorly understood. The clinical cohort included patients from Kobe University Hospital and Amagasaki General Medical Center Hospital, while the autopsy cohort was from the brain bank at Mayo Clinic Florida. We identified 83 patients in the clinical cohort and 193 patients in the autopsy cohort. We divided MSA into two groups according to age at onset: UO-MSA (≤ 75) and LO-MSA (> 75). We compared clinical features and outcomes between the two groups in the clinical cohort and compared the findings to the autopsy cohort. LO-MSA accounted for 8% in the clinical cohort and 5% in the autopsy cohort. The median time from onset to death or to life-saving tracheostomy was significantly shorter in LO-MSA than in UO-MSA in both cohorts (4.8 vs 7.9 years in the clinical cohort and 3.9 vs 7.5 years in the autopsy cohort; P = 0.043 and P < 0.0001, respectively). The median time from diagnosis to death was less than 3 years in LO-MSA in the clinical cohort. Some MSA patients have late age of onset and short survival, limiting time for clinical decision making. MSA should be considered in the differential diagnosis of elderly patients with autonomic symptoms and extrapyramidal and/or cerebellar syndromes.
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影响因子：
8.6
作者：
Ling, Helen;Asi, Yasmine T.;Holton, Janice L.
通讯作者：
Holton, Janice L.
DOI：
10.1097/00005072-199009000-00007
发表时间：
1990-09-01
影响因子：
3.2
作者：
NAKAZATO, Y;YAMAZAKI, H;YAMAGUCHI, H
通讯作者：
YAMAGUCHI, H
影响因子：
4.1
作者：
Koga, Shunsuke;Cheshire, William P.;Dickson, Dennis W.
通讯作者：
Dickson, Dennis W.
影响因子：
--
作者：
Hara, Kenju;Momose, Yoshio;Tsuji, Shoji
通讯作者：
Tsuji, Shoji
影响因子：
14.5
作者：
Cykowski, Matthew D.;Coon, Elizabeth A.;Parisi, Joseph E.
通讯作者：
Parisi, Joseph E.