Pedrola D, Pineda M, Fernández Alvarez E
Servicio de Neuropediatría, Hospital Sant Joan de Dèu, Universidad de Barcelona.
Rev Neurol. 1995 Jan-Feb;23(119):51-3.
A case of Hallervorden-Spatz's disease is described in a thirteen-year-old patient, with typical case symptoms of pyramidalism, dystonia and regressive motor control, over the last four years. MRI provided a bilateral palidal image in 'tiger eye'. The possible appearance of early forms have been stressed, characterised by a learning delay and a regressive syndrome starting between 5-10 years of age, along with later forms which manifest themselves mainly as Parkinson's disease. The MRI findings are of considerable value when diagnosing 'in vivo'; the pathogeny is not clear, despite current theories.
本文描述了一例13岁患有哈勒沃登 - 施帕茨病（Hallervorden-Spatz's disease）的患者，在过去四年中出现了锥体束征、肌张力障碍和进行性运动控制等典型症状。磁共振成像（MRI）显示双侧苍白球呈“虎眼征”。文中强调了早期症状可能的表现形式，其特征为5至10岁之间出现学习延迟和进行性综合征，以及后期主要表现为帕金森病的形式。MRI检查结果在“活体”诊断中具有重要价值；尽管有当前的理论，但病因仍不清楚。
