Department of Neurology, Myongji Hospital, Hanyang University College of Medicine, 55, Hwasu-ro 14beon-gil, Deogyang-gu, Goyang, 10475, South Korea.
BMC Neurol. 2021 Feb 3;21(1):51. doi: 10.1186/s12883-021-02080-4.
There is no established pathogenesis of hemiparkinsonism-hemiatrophy syndrome (HPHA), and the varied clinical presentations have been reported in several case studies. To the best of our knowledge, the present report describes the first case of HPHA with unusual brain imaging findings.
A 20-year-old man presented with a 6-month history of weakness and clumsiness in his right limbs. He showed right-sided parkinsonism with dystonic hand posture; however, body asymmetry was not noted. Brain imaging revealed hemiatrophy of the left hemisphere subcortical structures and brainstem, and iron deposition in the left globus pallidus and substantia nigra. In addition, dopamine transporter imaging demonstrated normal presynaptic dopaminergic function. The patient was treated with levodopa, which had little to no effect.
This case demonstrates the unique imaging characteristics of HPHA associated with widespread brain hemiatrophy and iron deposition. Further studies are needed to elucidate the diagnostic criteria for this heterogeneous syndrome.
尚未确定偏侧帕金森-萎缩综合征（HPHA）的发病机制，在几项病例研究中已经报道了其多种临床表现。据我们所知，本报告描述了首例具有异常脑部影像学表现的 HPHA 病例。
一名 20 岁男性因右侧肢体无力和笨拙 6 个月就诊。他表现为右侧帕金森病，伴有扭曲手姿势；然而，未注意到身体不对称。脑部影像学显示左侧大脑半球皮质下结构和脑干萎缩，以及左侧苍白球和黑质铁沉积。此外，多巴胺转运蛋白成像显示正常的突触前多巴胺能功能。患者接受了左旋多巴治疗，但效果甚微。
本病例展示了与广泛大脑偏侧萎缩和铁沉积相关的 HPHA 的独特影像学特征。需要进一步研究以阐明这种异质性综合征的诊断标准。
