Apraxia of speech in degenerative neurologic disease

Apraxia of speech in degenerative neurologic disease
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DOI:
10.1080/02687030600597358
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发表时间:
2006-06-01
期刊:
影响因子:
2
通讯作者:
Duffy, Joseph R.
Duffy, Joseph R.
中科院分区:
医学3区
文献类型:
--
作者:
Duffy, Joseph R.

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背景:只有有限数量的病例报告或病例系列提供了明确的证据,表明语言失用症(AOS)可能是神经退行性疾病的首要或主要症状。这可能反映了发生频率很低,但也可能反映了许多研究未能识别AOS或将其与原发性进行性失语(PPA)的诊断区分开来。这种区别可能对临床神经系统定位和诊断、预后、管理和组织病理学有重要意义。目的:描述一大群因退行性神经系统疾病导致的AOS患者的人口学特征、主诉、知觉属性、相关言语语言及相关临床表现、神经影像学表现和临床神经学诊断。方法与方法:回顾性分析1985年至2004年间出现的AOS患者,其严重程度不低于任何可能存在的失语症,其神经学评估得出的原因是退行性。结果与结果:共有80例患者符合纳入标准。在这些患者中,61%是男性。初次评估时的平均年龄为69岁(范围36-86岁)。言语语言困难是80%患者出现的第一个症状,56%患者在初次评估时的唯一主诉。初次评估时症状的平均持续时间为29个月。失语症和构音障碍分别明确存在于49%和50%的样本中。在80例AOS患者中,11%既没有失语也没有构音障碍。异常时,常经神经影像学鉴别为左或左。右半球异常。在这些患者中,44%的患者接受了纯粹描述性的临床神经学诊断,这在很大程度上取决于言语语言的发现;另有18%的患者被诊断为局限于左半球的局灶性/非对称皮质萎缩综合征;29%被诊断为运动表现突出的疾病,最常见的是皮质基底变性、进行性核上性麻痹或运动神经元疾病。结论:AOS可能是神经退行性疾病的首要和最突出的表现。它通常与失语症和/或构音障碍同时发生,但它可能是唯一的语言障碍。它似乎与任何单一的神经系统疾病无关,但当可能时,特定的神经系统诊断往往反映出具有突出运动表现的疾病。区分原发性进行性AOS和PPA非常重要。
Background: Only a limited number of case reports or case series have provided unequivocal evidence that apraxia of speech (AOS) can be the first or predominant sign of neurodegenerative disease. This may reflect a very low frequency of occurrence but may also reflect a failure in many studies to identify AOS or distinguish it from the diagnosis of primary progressive aphasia (PPA). The distinction may be important to clinical neurological localisation and diagnosis, prognosis, management, and histopathology.Aims: To describe the demographic characteristics, presenting complaints, perceptual attributes, associated speech- language and related clinical findings, neuroimaging findings, and clinical neurological diagnoses for a large group of patients with AOS due to degenerative neurologic disease.Methods & Procedures: Retrospective review of patients seen between 1985 and 2004, who had AOS that was never less severe than any aphasia that might have been present and whose neurologic evaluation concluded that the cause was degenerative.Outcomes & Results: A total of 80 patients met inclusion criteria. Of these patients, 61% were men. Average age at initial evaluation was 69 years (range = 36-86 years). Speech-language difficulty was the first symptom to emerge in 80% and the only patient complaint at the time of initial evaluation in 56%. Average duration of symptoms at initial evaluation was 29 months. Aphasia and dysarthria were unequivocally present in 49% and 50% of the sample, respectively. Of the 80 patients with AOS, 11% had neither aphasia nor dysarthria. When abnormal, neuroimaging often identified left or left. right hemisphere abnormalities. Of these patients, 44% received purely descriptive clinical neurologic diagnoses that were largely determined by speech-language findings; an additional 18% received diagnoses related to focal/ asymmetric cortical atrophy syndromes that localised to the left hemisphere; 29% received diagnoses of diseases with prominent motor manifestations, most often corticobasal degeneration, progressive supranuclear palsy, or motor neuron disease.Conclusions: AOS can be the first and most prominent manifestation of neurodegenerative disease. It often co- occurs with aphasia and/ or dysarthria, but it can be the only speech- language disorder. It does not appear to be associated with any single neurologic disease but specific neurologic diagnoses, when they are possible, tend to reflect conditions with prominent motor manifestations. It is important to distinguish primary progressive AOS from PPA.