Del Gamba Claudia, Bruno Antonino, Frosini Daniela, Volterrani Duccio, Migaleddu Gianmichele, Benedetto Nicola, Perrini Paolo, Pacchetti Claudio, Cosottini Mirco, Bonuccelli Ubaldo, Ceravolo Roberto
Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Pisa, Italy.
Department of Translational Research and of New Surgical and Medical Technologies, Nuclear Medicine Unit, University of Pisa, Pisa, Italy.
Neurol Sci. 2021 Feb;42(2):723-726. doi: 10.1007/s10072-020-04743-5. Epub 2020 Oct 1.
Idiopathic normal pressure hydrocephalus (iNPH) is a debated entity with controversial pathogenesis, diagnostic criteria, and predictors of response after ventriculoperitoneal shunt (VPS). Parkinsonian signs are frequently reported in the clinical picture, sometimes due to the coexistence of an underlying neurodegenerative parkinsonism and sometimes in the absence thereof. To distinguish these two scenarios is crucial, since they may carry different long-term response to CSF drainage. I-FP-CIT-SPECT was believed to be helpful in this regard, however its role in predicting surgical outcome has been disputed. We illustrate a patient presented with gait disturbance, urinary incontinence, and asymmetrical parkinsonian signs, who underwent a 3T brain MRI and a I-FP-CIT-SPECT. VPS was performed. The patient repeated a I-FP-CIT-SPECT, 18 months after the operation, and was clinically followed up for 24 months. Our patient displayed clinical and radiological criteria for iNPH and an abnormal asymmetrical uptake in I-FP-CIT-SPECT, consistent with her asymmetrical parkinsonism. However, the organization of the substantia nigra studied with iron-sensitive sequences in 3T brain MRI scan appeared intact. The patient revealed an improvement both clinically and in I-FP-CIT-SPECT at postsurgical follow-up. Our report suggests that abnormal I-FP-CIT-SPECT may not necessarily reveal an overlap with neurodegenerative parkinsonism; its partial reversibility may suggest that the mechanical effect exerted on the striatum by ventriculomegaly ultimately leads to downregulation of dopaminergic transporters which may improve after VPS.
特发性正常压力脑积水(iNPH)是一个存在争议的疾病实体,其发病机制、诊断标准以及脑室腹腔分流术(VPS)后反应的预测指标均存在争议。帕金森氏体征在临床表现中经常被报道,有时是由于潜在的神经退行性帕金森病共存,有时则并非如此。区分这两种情况至关重要,因为它们对脑脊液引流可能有不同的长期反应。I-FP-CIT-SPECT在这方面曾被认为是有帮助的,然而其在预测手术结果中的作用一直存在争议。我们举例介绍一位出现步态障碍、尿失禁和不对称帕金森氏体征的患者,该患者接受了3T脑MRI和I-FP-CIT-SPECT检查。进行了VPS手术。患者在术后18个月重复进行了I-FP-CIT-SPECT检查,并进行了24个月的临床随访。我们的患者表现出iNPH的临床和影像学标准,以及I-FP-CIT-SPECT中异常的不对称摄取,这与她的不对称帕金森病相符。然而,在3T脑MRI扫描中用铁敏感序列研究的黑质结构看起来是完整的。患者在术后随访中临床症状和I-FP-CIT-SPECT检查结果均有改善。我们的报告表明,I-FP-CIT-SPECT异常不一定揭示与神经退行性帕金森病的重叠;其部分可逆性可能表明脑室扩大对纹状体施加的机械效应最终导致多巴胺能转运体下调,而这在VPS后可能会改善。