LATE ONSET PARKINSONIAN SYNDROME IN HALLERVORDEN-SPATZ DISEASE
LATE ONSET PARKINSONIAN SYNDROME IN HALLERVORDEN-SPATZ DISEASE
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DOI：
10.1136/jnnp.50.12.1665
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发表时间：
1987-12-01
影响因子：
11
通讯作者：
NAVARRO, A
中科院分区：
文献类型：
--
作者：
ALBERCA, R;RAFEL, E;NAVARRO, A
两个兄弟姐妹，从血亲父母，在他们20多岁的发展帕金森综合征。在老年人中，疾病发展了13年，尸检研究诊断为Hallervorden-Spatz病。年幼的兄弟姐妹在12年的疾病后受到严重影响。在过去4年中，对该患者进行的几项CT和一项MR研究均正常。骨髓组织细胞和血淋巴细胞的超微结构研究揭示了特殊的夹杂物。低剂量的溴隐亭被证明是一种有益的治疗这个病人。
Two siblings, from consanguineous parents, developed in their twenties a Parkinsonian syndrome. In the elder, the disease evolved for 13 years and the necropsic study was diagnostic of Hallervorden-Spatz disease. The younger sibling is severely affected after 12 years of the disorder. Several CT and one MR studies done in this patient during the last 4 years have been normal. Ultrastructural studies of the bone marrow histiocytes and blood lymphocytes disclosed peculiar inclusions. Bromocriptine in low doses proved to be a beneficial therapy for this patient.