Clinical and imaging characterization of progressive spastic dysarthria.
Clinical and imaging characterization of progressive spastic dysarthria.
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DOI：
10.1111/ene.12271
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发表时间：
2014-03
影响因子：
5.1
通讯作者：
Josephs KA
中科院分区：
文献类型：
--
作者：
Clark HM;Duffy JR;Whitwell JL;Ahlskog JE;Sorenson EJ;Josephs KA
描述一系列表现为进行性痉挛构音障碍(PSD)的患者的言语、神经学和影像特征，PSD是推测的神经退行性疾病的首发和主要症状。研究对象为25名以痉挛性构音障碍为唯一或主要言语障碍的患者。描述了临床特征、基于体素的形态计量学的MRI体积丢失模式和F18-氟代脱氧葡萄糖(FDG-PET)扫描的低代谢模式。所有患者都表现出与痉挛型构音障碍相一致的语音特征，包括嗓音质量紧张，说话速度慢，单音和单色，以及缓慢和规则的语音交替运动速率。8名患者在检查中没有额外的神经学发现。其余部分患者出现假性延髓效应、上运动神经元模式、肢体无力、痉挛、霍夫曼征和巴宾斯基反射阳性。23名患者进行了肌电评估，没有一名患者患有弥漫性运动神经元病，也没有一名患者符合El Ecore关于ALS的标准。基于体素的形态计量学显示双侧白质体积显著减少，影响到运动皮质(BA4)，包括额顶盖(BA43)并延伸至大脑中脚。FDG-PET显示，在一些患者中，特别是那些病程超过两年的患者，微妙的低代谢影响了运动前和运动皮质。我们的特点是一种神经退行性疾病，由于运动和运动前皮质受累，皮质脊髓和皮质球下行通路受累，导致局灶性构音障碍。我们建议使用“渐进性痉挛性构音障碍”这一描述性标签来最好地反映该综合征的主要表现特征。
To describe speech, neurological and imaging characteristics of a series of patients presenting with progressive spastic dysarthria (PSD) as the first and predominant sign of a presumed neurodegenerative disease. Participants were 25 patients with spastic dysarthria as the only or predominant speech disorder. Clinical features, pattern of MRI volume loss on voxel-based morphometry, and pattern of hypometabolism with F18-Fluorodeoxyglucose (FDG-PET) scan are described. All patients demonstrated speech characteristics consistent with spastic dysarthria, including strained voice quality, slow speaking rate, monopitch and monoloudness, and slow and regular speech alternating motion rates. Eight patients did not have additional neurological findings on examination. Pseudobulbar affect, upper motor neuron pattern limb weakness, spasticity, Hoffman sign and positive Babinski reflexes were noted in some of the remaining patients. Twenty-three patients had electromyographic assessment and none had diffuse motor neuron disease or met El Escorial criteria for ALS. Voxel-based morphometry revealed striking bilateral white matter volume loss, , affecting the motor cortex (BA 4), including the frontoparietal operculum (BA 43) with extension into the middle cerebral peduncle. FDG-PET showed subtle hypometabolism affecting the premotor and motor cortices in some patients, particularly in those who had a disease duration longer than two years. We have characterized a neurodegenerative disorder that begins focally with spastic dysarthria due to involvement of the motor and premotor cortex and descending corticospinal and corticobulbar pathways. We propose the descriptive label “progressive spastic dysarthria” to best capture the dominant presenting feature of the syndrome.
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影响因子：
1.9
作者：
Tomik, Barbara;Zur, Karnila. Anna;Szczudlik, Andrzej
通讯作者：
Szczudlik, Andrzej
影响因子：
10.6
作者：
Sled, JG;Zijdenbos, AP;Evans, AC
通讯作者：
Evans, AC
影响因子：
5.7
作者：
Genovese, CR;Lazar, NA;Nichols, T
通讯作者：
Nichols, T
DOI：
10.1044/jshr.1203.462
发表时间：
1969-01-01
期刊：
JOURNAL OF SPEECH AND HEARING RESEARCH
影响因子：
--
作者：
DARLEY, FL;ARONSON, AE;BROWN, JR
通讯作者：
BROWN, JR
影响因子：
2
作者：
Uttner, Ingo;Brettschneider, Johannes;Riecker, Axel
通讯作者：
Riecker, Axel