Baptista M V, Vale J, Leitão O
Serviço de Neurologia, Hospital Egas Moniz, Lisboa.
Acta Med Port. 1997 Aug-Sep;10(8-9):563-7.
Striato-pallido-dentate calcifications (SPDC) is a well defined entity, characterized by calcium deposits in the basal ganglia, dentate nuclei and the centrum semiovale. Several metabolic derangements have been associated with this entity, particularly parathyroid disorders. The traditional designation of Fahr's syndrome should be restricted to the idiopathic cases. The authors report a study of seven patients with SPDC. Hypocalcemia was found in three cases, two with pseudohypoparathyroidism and one with hypoparathyroidism. Fahr's syndrome was diagnosed in four patients. Clinical and laboratory features are presented. Neurological manifestations included epilepsy, dementia and parkinsonism. Discussion focuses on the distinction of this entity from the small pallidal calcifications and on the pathophysiology of basal ganglia mineralisation, in view of recent reports.
纹状体-苍白球-齿状核钙化（SPDC）是一种明确的病症，其特征为基底神经节、齿状核和半卵圆中心有钙沉积。多种代谢紊乱与该病症相关，尤其是甲状旁腺疾病。法尔综合征的传统命名应仅限于特发性病例。作者报告了一项对7例SPDC患者的研究。3例患者发现有低钙血症，其中2例为假性甲状旁腺功能减退，1例为甲状旁腺功能减退。4例患者被诊断为法尔综合征。文中呈现了临床和实验室特征。神经学表现包括癫痫、痴呆和帕金森症。鉴于近期的报告，讨论集中于该病症与苍白球小钙化的鉴别以及基底神经节矿化的病理生理学。
