Otsuki M, Soma Y, Yoshimura N, Tsuji S
Department of Neurology, Niigata University, Japan.
Eur Neurol. 1997;37(2):100-3. doi: 10.1159/000117418.
The case of a 69-year-old female with slowly progressive limb-kinetic apraxia (LKA) of the right hand over 3.5 years is reported. She did not show any other neurological or neuropsychological symptoms except for a defect in two-point discrimination, and 3.5 years after onset she developed a slight cogwheel-like muscle tone in the right wrist. Brain MRI revealed atrophic changes in the left central region including the precentral and postcentral gyri and the superior parietal region, and among them, most strikingly at the postcentral gyrus. 123I-IMP SPECT revealed decreased 123I uptake in the atrophic lesion as revealed by MRI. We should suspect corticobasal degeneration as the etiology for this patient taking her symptoms and findings on MRI and SPECT into consideration. However, her symptoms and course were quite unique among the case reports of patients with slowly progressive LKA as she had only LKA and a defect in two-point discrimination for more than 3.5 years without any other symptoms. This characteristic also indicated that the simultaneous appearance of LKA and a defect in two-point discrimination may suggest a mechanism of LKA in the present patient.
报告了一例69岁女性患者,右手出现缓慢进展性肢体运动性失用症(LKA)达3.5年。除两点辨别觉缺陷外,她未表现出任何其他神经或神经心理学症状,发病3.5年后,其右手腕出现轻微的齿轮样肌张力。脑部MRI显示左侧中央区域包括中央前回、中央后回和顶上叶区域有萎缩性改变,其中最明显的是中央后回。123I-IMP SPECT显示,如MRI所示,萎缩性病变处123I摄取减少。综合该患者的症状以及MRI和SPECT检查结果,我们应怀疑皮质基底节变性为病因。然而,在缓慢进展性LKA患者的病例报告中,她的症状和病程相当独特,因为她仅患有LKA和两点辨别觉缺陷超过3.5年,无任何其他症状。这一特征还表明,LKA和两点辨别觉缺陷同时出现可能提示了该患者LKA的发病机制。