Hallervorden- Spatz症候群在電腦斷層攝影上的發現──病例報告──

COMPUTED TOMOGRAPHY IN HALLERVORDENSPATZ SYNDROME - A CASE REPORT

李志明(C.M. Lee) ； 劉祥仁(S.J . Ryu) ； 蔡長晃(C.H. Tsai) ； 蔡春泉(C.C. Tsai)

《中華放射線醫學雜誌》 12卷3期 (1987/09) Pp. 283-286

摘要

Hallervorden-Spatz症候群（Hallervorden-Spatz syndrome, HSS）是一種罕見之隱性遺傳病症（autosomal recessive inheritance），其主要的病理變化是在蒼自球（globus pallidus）和黑質之網狀部分（reticular zone of the substantia nigra）有過量之鐵質沈積。本文患者是二十一歲的男性，其臨床表現為進行性步伐障礙、構音困難（dysarthria)、肌張力異常（dystonia）、頸部強直（rigidity）和進行性的癡呆（progressive dementia），已有四年之有。腦部電腦斷層攝影顯示病人的大腦和小腦均有廣泛性萎縮，而在兩側蒼白球區內有高吸收值點：測量兩側頸腦室外角之寬度（width of frontal horns, FH）與兩側尾狀核間距離（intercaudate' distance, CC），其比率FH:CC為1.42，與Huntington氏舞蹈症在CT上的發現相似。在臨床上懷疑是HSS的引導下，利用立體定位手術（stereo tactic surgery），在右側蒼白球上取得檢體，病理證實有鐵質的沈積。因此，配合臨床的症狀，CT對HSS的診斷可提供很好的參考。

並列摘要

Hallervorden-Spatz syndrome (HSS) is a rare hereditary disorder that starts early in childhood and progressive to significant dementia, usually accompanied by extrapyramidal syndrome. Pathological examination mainly reveals iron deposits in the globus pallidus and the reticular zone of the substantia nigra. Recently, we had a case of HSS. This 21-year-old man suffered from progressive gait disturbance, dysarthria, neck rigidity and dementia for about 4 years. Brain CT showed generalized cerebral and cerebellar atrophy. The ratio between the width of the frontal horns and the intercaudate distance was 1.42, being similar to those found in patient with Huntington's chorea. CT also showed spots of increase attenuation in bilateral globus pallidus regions. Biopsy of the right globus pallidus was performed by stereotactic technique, iron deposits were found in the specimen. So taking together with clinical features, CT may be helpful in reaching the correct diagnosis.