Kitazawa K, Ikeda M, Tsukagoshi H
Department of Neurology, Asahi General Hospital.
Rinsho Shinkeigaku. 1990 May;30(5):520-3.
A 37-year-old housewife, who had physical characteristics of cerebral gigantism, such as the tall stature, acromegaly, macrocephalia, high arched palate and antimongoloid slant, developed cerebellar ataxia and dysarthria. Her mother, uncle and grandmother were also reported to have slowly progressive gait disturbance. Her mother was also tall. Endocrinological studies failed to show any definite abnormality. CT and MRI revealed remarkable cerebellar atrophy. Though cerebral gigantism is often associated with clumsiness and incoordination, the etiology of the ataxia is poorly understood. This case indicates that the ataxia in cerebral gigantism may be, at least partly, caused by cerebellar atrophy.
一名37岁的家庭主妇，具有脑性巨人症的身体特征，如身材高大、肢端肥大、巨头畸形、高拱腭和反蒙古样斜眼，出现了小脑共济失调和构音障碍。据报道，她的母亲、叔叔和祖母也有缓慢进展的步态障碍。她的母亲也很高。内分泌学研究未显示任何明确异常。CT和MRI显示明显的小脑萎缩。虽然脑性巨人症常伴有笨拙和不协调，但共济失调的病因尚不清楚。该病例表明，脑性巨人症中的共济失调可能至少部分是由小脑萎缩引起的。
