Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy.
Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy; Department of Neurology, University Hospital Würzburg and Julius-Maximilians-University, Würzburg, Germany.
Parkinsonism Relat Disord. 2019 Sep;66:40-44. doi: 10.1016/j.parkreldis.2019.06.024. Epub 2019 Jun 28.
Idiopathic Normal Pressure Hydrocephalus (iNPH) is a complex syndrome of ventriculomegaly that can include parkinsonian-like features besides the classical triad of cognitive decline, urinary incontinence, and gait/balance disturbances. Pisa syndrome (PS) is a postural abnormality often associated with parkinsonism and defined as lateral trunk flexion greater than 10° while standing that resolves in the supine position. We reported a case series of classical "fixed" PS and one case of "Metronome" recurrent side-alternating PS in iNPH, displaying opposite electromyographic patterns of paraspinal muscles.
Eighty-five iNPH patients were followed longitudinally for at least one year through scheduled clinical and neuropsychological visits.
Five (5.9%) subjects revealed PS. None of them had nigrostriatal dopaminergic involvement detected by [123I]FP-CIT SPECT. Among these patients, four had "fixed" PS, whereas one showed a recurrent side-alternating PS which repeatedly improved after ventriculo-peritoneal shunt and following adjustments of the valve-opening pressure of the shunt system.
This is the first case series of PS in iNPH and the first report of "Metronome" PS in iNPH. The prompt response of the abnormal trunk postures through cerebrospinal fluid (CSF) shunt surgery suggests a causative role of an altered CSF dynamics. PS and gait disorders in iNPH could be explained by a direct involvement of cortico-subcortical pathways and subsequent secondary brainstem involvement, with also a possible direct functional damage of the basal ganglia at the postsynaptic level, due to enlargement of the ventricular system and impaired CSF dynamics. The early detection of these cases supports a proper surgical management.
特发性正常压力脑积水（iNPH）是一种脑室扩大的复杂综合征，除了认知能力下降、尿失禁和步态/平衡障碍的经典三联征外，还可能包括类似帕金森病的特征。比萨综合征（PS）是一种姿势异常，常与帕金森病相关，定义为站立时躯干侧向屈曲大于 10°，而在仰卧位时缓解。我们报告了一系列 iNPH 中的经典“固定”PS 和一例“节拍器”反复交替 PS 病例，表现出相反的脊柱旁肌肉肌电图模式。
85 例 iNPH 患者通过定期的临床和神经心理学随访进行了至少一年的纵向随访。
5 例（5.9%）患者出现 PS。他们中没有人通过[123I]FP-CIT SPECT 检测到黑质纹状体多巴胺能参与。在这些患者中，4 例有“固定”PS，而 1 例表现为反复交替的 PS，在脑室-腹腔分流后和调整分流系统的阀门开启压力后，症状反复改善。
这是 iNPH 中 PS 的首个病例系列，也是 iNPH 中“节拍器”PS 的首次报告。异常躯干姿势通过脑脊液（CSF）分流手术的迅速反应提示 CSF 动力学改变的因果作用。iNPH 中的 PS 和步态障碍可以通过皮质-皮质下通路的直接参与以及随后的脑干继发性受累来解释，也可能由于脑室系统扩大和 CSF 动力学受损，导致基底节的突触后水平发生直接功能损伤。这些病例的早期发现支持适当的手术治疗。
