Hashimoto R, Taguchi T, Kano M, Hanyu S, Tanaka Y, Nishizawa M, Nakano I
Department of Neurology, Jichi Medical School, Tochigi.
Rinsho Shinkeigaku. 1999 May;39(5):520-6.
A 57-year-old woman presented with a slowly progressive gait disturbance in 1992 (53 years of age). Over the next year, she gradually began to talk less, but her speech itself became more rapid than before. He speech was frequently too fast even for family members to understand. In 1997, she was admitted to our hospital. On admission, the patient was disoriented but able to follow simple verbal commands, to name things, and to write simple words. Neither apraxia, aphasia, hemispatial neglect, nor a corpus callosum disconnection syndrome was observed. There was no muscle weakness or atrophy. She showed a positive Babinski sign with mild spasticity in the legs and Gegenhalten, but no rigidity. Her speech was monotonous and abnormally fast (cluttering-like speech). Her speech became faster and faster toward the end of sentences, skipping several syllables or even words. She was unable to speak slowly and clearly, even when efforts were made to pace her speech to the speed set by the examiner. She was able to stand only with a wide base of support and body flexion. When standing, she was unable to place one foot directly beside the other; as she tried to have one foot near the other, the former repelled the latter. She had great difficulties in taking her first step forward, and showed rapid freezing of gait even when she managed to succeed in starting. She was able to imitate walking or bicycling with her legs unloaded, indicating that her gait disturbance was a kind of apraxia of gait. Her intelligence was somehow difficult to assess because of her peculiar speech disturbance. However, her family members had noticed her memory disturbance and personality change (offensiveness) since 3 to 4 years before the admission. Moreover, she was defective not only on Hasegawa Damentia Scale-Revised but also on Raven's Colored Progressive Matrices which estimates non-verbal intelligence. It was also noted that she was inattentive and lazy in thinking on questionnaires. Thus we considered that she was at least mildly demented and the type of dementia was of frontal pathology. Laboratory data were all normal except for the head MRI, which demonstrated prominent and thinness of the corpus callosum from the anterior part of the body to splenium without any other brain lesions that could cause the thinness secondarily. Our case resembles two cases reported by Sunohara et al in 1985, together comprising a unique clinical feature. Although Sunohara et al did not refer to the thinness of the corpus callosum in their cases, the clinical profiles in our case and theirs raise the possibility that they form a new disease entity. A further study in a large number of similar cases, including autopsies will provide a conclusion.
一名57岁女性于1992年（53岁时）出现缓慢进展的步态障碍。在接下来的一年里，她逐渐话少，但语速比以前更快。她的讲话速度常常过快，连家人都难以理解。1997年，她住进我院。入院时，患者定向障碍，但能听从简单的口头指令、说出物品名称并书写简单词语。未观察到失用症、失语症、半侧空间忽视或胼胝体离断综合征。没有肌肉无力或萎缩。她双侧巴宾斯基征阳性，双下肢有轻度痉挛及 Gegenhalten 现象，但无强直。她的讲话单调且异常快速（类似语流紊乱）。句子结尾时她的语速越来越快，跳过几个音节甚至单词。即便努力按照检查者设定的语速讲话，她也无法缓慢而清晰地表达。她只能在宽基底支撑和身体前屈的情况下站立。站立时，她无法将一只脚直接放在另一只脚旁边；当她试图让一只脚靠近另一只脚时，前者会排斥后者。她向前迈出第一步非常困难，即使成功起步也会很快出现步态冻结。她能够在双腿不负重的情况下模仿行走或骑自行车，这表明她的步态障碍是一种步态失用症。由于她独特的言语障碍，其智力状况难以评估。然而，她的家人在入院前3至4年就注意到了她的记忆障碍和性格改变（攻击性）。此外，她不仅在修订版长谷川痴呆量表上表现不佳，在评估非言语智力的瑞文彩色渐进矩阵测试中也表现欠佳。还注意到她在问卷测试中注意力不集中且思维迟缓。因此，我们认为她至少存在轻度痴呆，且痴呆类型为额叶病变。除头部MRI外，实验室检查数据均正常，头部MRI显示胼胝体从体部前部到压部明显变薄，无任何其他可继发导致变薄的脑部病变。我们的病例与1985年 Sunohara 等人报道的两例病例相似，共同构成一种独特的临床特征。尽管 Sunohara 等人在其病例中未提及胼胝体变薄情况，但我们病例与他们病例的临床特征提示它们可能构成一种新的疾病实体。对大量类似病例进行进一步研究，包括尸检，将得出结论。
