Madoka Nakajima, Department of Neurosurgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8342, Japan,
Croat Med J. 2021 Aug 31;62(4):387-398. doi: 10.3325/cmj.2021.62.387.
Idiopathic normal pressure hydrocephalus (iNPH) is a condition resulting from impaired cerebrospinal fluid (CSF) absorption and excretion characterized by a triad of symptoms comprising dementia, gait disturbance (impaired trunk balance), and urinary incontinence. CSF biomarkers not only assist in diagnosis but are also important for analyzing the pathology and understanding appropriate treatment indications. As the neuropathological findings characteristic of iNPH have yet to be defined, there remains no method to diagnose iNPH with 100% sensitivity and specificity. Neurotoxic proteins are assumed to be involved in the neurological symptoms of iNPH, particularly the appearance of cognitive impairment. The symptoms of iNPH can be reversed by improving CSF turnover through shunting. However, early diagnosis is essential as once neurodegeneration has progressed, pathological changes become irreversible and symptom improvement is minimal, even after shunting. Combining a variety of diagnostic methods may lead to a more definitive diagnosis and accurate prediction of the prognosis following shunt treatment. Identifying comorbidities in iNPH using CSF biomarkers does not contraindicate shunting-based intervention, but does limit the improvement in symptoms it yields, and provides vital information for predicting post-treatment prognosis.
特发性正常压力脑积水(iNPH)是一种由脑脊液(CSF)吸收和排泄受损引起的疾病,其特征是三联征症状,包括痴呆、步态障碍(躯干平衡障碍)和尿失禁。CSF 生物标志物不仅有助于诊断,而且对于分析病理和了解适当的治疗指征也很重要。由于 iNPH 的神经病理学特征尚未确定,因此仍然没有 100%敏感和特异性的诊断方法。神经毒性蛋白被认为与 iNPH 的神经症状有关,特别是认知障碍的出现。通过分流改善 CSF 周转率可以逆转 iNPH 的症状。然而,早期诊断至关重要,因为一旦神经退行性变进展,病理变化就变得不可逆转,即使进行分流治疗,症状改善也很小。结合多种诊断方法可能会得出更明确的诊断,并准确预测分流治疗后的预后。使用 CSF 生物标志物识别 iNPH 的合并症并不妨碍基于分流的干预,但会限制其症状的改善,并为预测治疗后预后提供重要信息。