Sakai Kenji, Furui Eisuke, Komai Kiyonobu, Notoya Masako, Yamada Masahito
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science.
Rinsho Shinkeigaku. 2002 Feb;42(2):178-80.
We report a 57-year-old man with progressive supranuclear palsy (PSP) showing acquired stuttering (AS) as an early symptom. He had noticed micrographia at age 48, and thereafter he began to suffer from progressive speech disturbance at age 49. Neurological examination at age 57 revealed prominent acquired stuttering, supranuclear vertical gaze palsy, axial rigidity, disturbance of postural reflex, motor perseveration, and catalepsy, but no aphasia. A brain MRI study demonstrated atrophy of the midbrain tegmentum and dilatation of the third ventricle with a few lacunar infarcts in the basal ganglia. Anti-parkinsonian drugs were ineffective. We diagnosed his illness as PSP. His speech was characterized by repetition of sounds and syllables. It was more dominant at repetition of sentences than at that of words, and was improved with rhythmic stimulation. Although pathophysiology underlying AS remains unknown, the AS in this patient seems to be closely related to his motor perseveration and catalepsy. These symptoms may be caused by the disturbance of dopaminergic system from midbrain to limbic system and frontal cortex as reported in PSP. In conclusion, As can be an early and prominent symptom which may be related to characteristic impairment of the dopaminergic system.
我们报告一例57岁进行性核上性麻痹（PSP）男性患者，其早期症状为获得性口吃（AS）。他48岁时发现有小字症，此后49岁开始出现进行性言语障碍。57岁时的神经学检查显示明显的获得性口吃、核上性垂直凝视麻痹、轴性强直、姿势反射障碍、运动持续性和僵住症，但无失语症。脑部MRI研究显示中脑被盖萎缩、第三脑室扩张，基底节有少量腔隙性梗死。抗帕金森药物无效。我们将他的疾病诊断为PSP。他的言语特点是声音和音节的重复。句子重复时比单词重复时更明显，且通过节律性刺激可改善。尽管AS的病理生理学尚不清楚，但该患者的AS似乎与他的运动持续性和僵住症密切相关。这些症状可能如PSP报道的那样，是由从中脑到边缘系统和额叶皮质的多巴胺能系统紊乱引起的。总之，AS可以是一个早期且突出的症状，可能与多巴胺能系统的特征性损害有关。
