Zakład Pielêgniarstwa Neurologiczno-Psychiatrycznego, Gdański Uniwersytet Medyczny, Gdańsk, Polska.
Neurol Neurochir Pol. 2013 Jul-Aug;47(4):387-92. doi: 10.5114/ninp.2013.34557.
Hemiparkinsonism-hemiatrophy (HPHA) is a rare neurological syndrome. The main clinical features of HPHA consist of atrophy of one side of the body (face, trunk, limbs), ipsilateral hemiparkinsonism (bradykinesia, rigidity, tremor) and in many cases dystonia. There are no data on prevalence of HPHA as the condition is rare. The mean age of parkinsonism onset is earlier than in idiopathic Parkinson disease (43.7 years, range: 15-63). Changes in magnetic resonance imaging (MRI) (cortical, basal ganglia atrophy contralaterally to the side of clinical presentation) are described in 30% of patients. The pathogenesis of HPHA is unknown, but in many cases a history of prenatal injuries was reported. We present two male patients with HPHA - 45 and 55 years old, with left-sided parkinsonism, dystonia and hemiatrophy (to our knowledge, the first Polish cases). Both patients had no atrophic changes in MRI and levodopa treatment was ineffective. In the discussion the authors review current literature on HPHA.
半侧帕金森-半侧萎缩症（HPHA）是一种罕见的神经综合征。HPHA 的主要临床特征包括身体一侧（面部、躯干、四肢）的萎缩、同侧帕金森病（运动迟缓、僵硬、震颤），在许多情况下还伴有肌张力障碍。由于这种疾病很少见，因此没有关于 HPHA 患病率的数据。帕金森病发病的平均年龄比特发性帕金森病早（43.7 岁，范围：15-63）。30%的患者描述了磁共振成像（MRI）（与临床表现同侧的对侧皮质、基底节萎缩）的变化。HPHA 的发病机制尚不清楚，但在许多情况下，有产前损伤的病史。我们报告了两名男性 HPHA 患者，年龄分别为 45 岁和 55 岁，均患有左侧帕金森病、肌张力障碍和半侧萎缩（据我们所知，这是波兰首例）。两名患者的 MRI 均无萎缩性改变，且左旋多巴治疗无效。在讨论中，作者回顾了 HPHA 的现有文献。
