Ohara K, Miyoshi K, Ueki A, Hayashi S
Department of Neuropsychiatry, Hyogo College of Medicine.
Rinsho Shinkeigaku. 1991 Jan;31(1):1-5.
We reported two families each propositus of which exhibited extrapyramidal signs and dementia with bilateral basal ganglia calcifications (BGC), while some of the other non-symptomatic family members showed BCG on brain CT by further examinations. Family 1) A 49-year-old woman was normal until her mid 40s when her memory began to fail. At age 40, dementia, finger-tremor and rigidity were observed and with brain CT and Magnetic Resonance Imaging, BCG and dentate calcifications were found. Her two daughters (20 years old and 26 years old) were free of any neuropsychiatric symptoms, but with CT examinations disclosed BCG. Family 2) A 40-year-old man. His symptoms started at 33 years old. He noticed gradually increasing finger-tremor, rigidity and dysarthria. At 40 years he showed mild dementia and BCG on Brain CT. His mother (64 years old) was non-symptomatic but CT showed that she had BCG. In the two families the calcium, phosphorous and parathyroid hormone levels, and Ellsworth-Howard test were normal. Other specific etiology including infections and somatic abnormalities was not discovered. Familial idiopathic basal ganglia calcification was considered to be rare. But the main purpose of this report is to point out that we must pay attention to the possibility of BCG of non-symptomatic family members if one showed dementia and extrapyramidal signs, and BCG on Brain CT in middle age.
我们报告了两个家族，每个家族的先证者均表现出锥体外系症状和痴呆，并伴有双侧基底节钙化（BGC），而其他一些无症状家族成员经进一步检查后，脑部CT显示有BCG。家族1）一名49岁女性，直到40多岁记忆力开始减退之前一直正常。40岁时出现痴呆、手指震颤和强直，脑部CT和磁共振成像显示有BCG和齿状核钙化。她的两个女儿（分别为20岁和26岁）没有任何神经精神症状，但CT检查发现有BCG。家族2）一名40岁男性。他的症状始于33岁。他逐渐注意到手指震颤、强直和构音障碍加重。40岁时，他表现出轻度痴呆，脑部CT显示有BCG。他的母亲（64岁）无症状，但CT显示她有BCG。在这两个家族中，钙、磷和甲状旁腺激素水平以及埃尔斯沃思-霍华德试验均正常。未发现包括感染和躯体异常在内的其他特定病因。家族性特发性基底节钙化被认为很罕见。但本报告的主要目的是指出，如果中年人出现痴呆、锥体外系症状且脑部CT显示有BCG，我们必须注意无症状家族成员出现BCG的可能性。
