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[苍白球-齿状核钙化(基于7例解剖学-临床病例报告)]

[Pallido-dentate calcifications (apropos of 7 anatomo-clinical case reports)].

作者信息

Constantinidis J, Aubert M, Tissot R

出版信息

Schweiz Arch Neurol Neurochir Psychiatr. 1978;122(2):237-51.

PMID:694468
Abstract

"Fahr's Disease" is characterized by bilateral and symmetrical calcifications of the Globus Pallidus (systematically extending to the Commisura Anterior and the Capsula Interna, and less commonly to the Putamen, the Centrum Semi-Ovale and the Cerebral Cortex), and of the Cerebellar Nucleus Dentalus (with spreading to the White Matter and the Cortical Lamellae). Lesions or absence of Parathyroids are frequently related, with subsequent metabolic disorders of Phosphorus and Calcium, but idiopathic cases without hypoparathyroid disturbances are also found. A Morgagni-Morel Hyperostosis Frontalis Interna is often associated with "Fahr's Disease", and there could be a relationship between these two affections. We found in three cases the association between "Fahr's Disease" and Morel's Nodular Dysgenesis of the Frontal Cortex. Most of the cases are sporadic, but observations with a clear familial incidence are also found. Clinically, various Neurological Disorders (cerebellar, extrapyramidal, pyramidal, dysarthria, epileptic seizures) are often but not always observed; the Psychiatric Disorders found in some cases could be fortuitious associations (psychoses), connected to hypothyroidism (oligophrenia), and in aged patients, to unrelated cerebral vascular or degenerative lesions; very seldom, a dementing state could be connected to the spreading of calcifications to the Cerebral Cortex.

摘要

“法尔病”的特征是双侧对称性苍白球钙化(系统性地延伸至前连合和内囊,较少累及壳核、半卵圆中心和大脑皮质),以及小脑齿状核钙化(并蔓延至白质和皮质板层)。甲状旁腺病变或缺失常与之相关,随后出现磷和钙的代谢紊乱,但也有未伴有甲状旁腺功能减退的特发性病例。额骨内板增厚症常与“法尔病”相关,这两种病症之间可能存在某种联系。我们发现3例“法尔病”与额叶皮质莫雷尔结节发育异常相关联。大多数病例为散发性,但也有明显家族发病的观察报告。临床上,常可观察到各种神经系统疾病(小脑性、锥体外系、锥体束、构音障碍、癫痫发作),但并非总是如此;在某些病例中发现的精神障碍可能是偶然关联(精神病),与甲状腺功能减退(智力发育迟缓)有关,而在老年患者中,与不相关的脑血管或退行性病变有关;钙化蔓延至大脑皮质时,极少会导致痴呆状态。

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