Caselli R J, Windebank A J, Petersen R C, Komori T, Parisi J E, Okazaki H, Kokmen E, Iverson R, Dinapoli R P, Graff-Radford N R
Section of Neurology, Mayo Clinic Scottsdale, AZ 85259.
Ann Neurol. 1993 Feb;33(2):200-7. doi: 10.1002/ana.410330210.
Articulatory and language impairment heralded rapidly progressive motor neuron disease in 7 patients aged 54 to 77 years. One patient had a family history of a similar disorder. Severe nonfluent aphasia developed in all 7 patients and 4 were anarthric within a year. Other cognitive domains were impaired, yet 2 patients lived alone until 1 month before their deaths. Four died within 2 years. Abnormalities were found on electromyography, computed tomography, magnetic resonance imaging, single-photon emission computed tomography, and electroencephalography. Neuropathological examination in 3 patients showed bilateral hemispheric atrophy with neuronal loss and gliosis predominantly of superficial cortical layers. Pigmented and hypoglossal nuclei were relatively preserved. At all spinal levels there was degeneration of corticospinal tracts and loss of anterior horn cells with gliosis. Rapidly progressive aphasic dementia and motor neuron disease are a distinctive clinical entity whose nosology is poorly understood.
7名年龄在54至77岁的患者出现构音和语言障碍，预示着快速进展的运动神经元病。1名患者有类似疾病的家族史。所有7名患者均出现严重的非流利性失语，4名患者在1年内完全不能说话。其他认知领域也受到损害，但2名患者在去世前1个月一直独居。4名患者在2年内死亡。肌电图、计算机断层扫描、磁共振成像、单光子发射计算机断层扫描和脑电图检查均发现异常。3名患者的神经病理学检查显示双侧半球萎缩，神经元丢失，胶质增生主要见于皮质浅层。色素核和舌下神经核相对保留。在所有脊髓节段，皮质脊髓束均有变性，前角细胞丢失并伴有胶质增生。快速进展性失语性痴呆和运动神经元病是一种独特的临床实体，其疾病分类学尚不清楚。
