Harati Y, Jackson J A, Benjamin E
Arch Intern Med. 1984 Dec;144(12):2425-7.
We treated a family with idiopathic calcifications of symmetric areas of the brain, including the basal ganglia, dentate, and cerebral white matter. Dementia, progressive dysarthria, incontinence, propulsive-ataxic gait, fixed facies, and cogwheel rigidity without dysmorphic features develop in affected persons. Calcium, phosphorus, and parathyroid hormone levels were normal in the two siblings tested. The literature is reviewed and five other families with a similar syndrome are identified. These six families seem to be clinically distinct from the larger group of idiopathic cerebral calcifications usually referred to as Fahr's disease.
我们治疗了一个患有大脑对称区域特发性钙化的家庭，这些区域包括基底神经节、齿状核和脑白质。受影响者会出现痴呆、进行性构音障碍、尿失禁、推进性共济失调步态、表情固定以及齿轮样强直，但无畸形特征。接受检测的两名兄弟姐妹的钙、磷和甲状旁腺激素水平均正常。我们对文献进行了综述，并确定了另外五个患有类似综合征的家庭。这六个家庭在临床上似乎与通常被称为法尔病的更大的特发性脑钙化群体不同。
