特发性正常压力脑积水的诊断与治疗
Diagnosis and Treatment of Idiopathic Normal Pressure Hydrocephalus.
作者信息
Williams Michael A, Malm Jan
出版信息
Continuum (Minneap Minn). 2016 Apr;22(2 Dementia):579-99. doi: 10.1212/CON.0000000000000305.
PURPOSE OF REVIEW
This article provides neurologists with a pragmatic approach to the diagnosis and treatment of idiopathic normal pressure hydrocephalus (iNPH), including an overview of: (1) key symptoms and examination and radiologic findings; (2) use of appropriate tests to determine the patient's likelihood of shunt responsiveness; (3) appropriate referral to tertiary centers with expertise in complex iNPH; and (4) the contribution of neurologists to the care of patients with iNPH following shunt surgery.
RECENT FINDINGS
The prevalence of iNPH is higher than previously estimated; however, only a fraction of persons with the disorder receive shunt surgery. iNPH should be considered as a diagnosis for patients with unexplained symmetric gait disturbance, a frontal-subcortical pattern of cognitive impairment, and urinary urge incontinence, whose MRI scans show enlarged ventricles and whose comorbidities are not sufficient to explain their symptoms. Physiologically based tests, such as the tap test (large-volume lumbar puncture) or temporary spinal catheter insertion for external lumbar drainage with gait testing before and after CSF removal, or CSF infusion testing for measurement of CSF outflow resistance, can reliably identify patients who are likely to respond to shunt surgery. Properly selected patients have an 80% to 90% chance of responding to shunt surgery, and all symptoms can improve following shunt surgery. Longitudinal care involves investigating the differential diagnosis of any symptoms that either fail to respond to shunt surgery or that worsen after initial improvement from shunt surgery.
SUMMARY
Neurologists play an important role in the identification of patients who should be evaluated for possible iNPH. With contemporary diagnostic tests and treatment with programmable shunts, the benefit-to-risk ratio of shunt surgery is highly favorable. For more complex patients, tertiary centers with expertise in complex iNPH are available throughout the world.
综述目的
本文为神经科医生提供了一种实用的方法来诊断和治疗特发性正常压力脑积水（iNPH），内容包括：（1）关键症状、检查及影像学表现概述；（2）使用适当的检查来确定患者对分流手术有反应的可能性；（3）适当地转诊至在复杂iNPH方面有专业知识的三级中心；（4）神经科医生在分流手术后对iNPH患者护理中的作用。
最新发现
iNPH的患病率高于先前估计；然而，只有一小部分患有该疾病的人接受了分流手术。对于出现无法解释的对称性步态障碍、额颞叶皮质下认知障碍模式和尿急失禁的患者，若其MRI扫描显示脑室扩大且合并症不足以解释其症状，则应考虑iNPH的诊断。基于生理学的检查，如叩击试验（大量腰椎穿刺）或临时插入脊髓导管进行外部腰椎引流并在脑脊液引流前后进行步态测试，或脑脊液灌注试验以测量脑脊液流出阻力，可以可靠地识别可能对分流手术有反应的患者。经过适当选择的患者有80%至90%的机会对分流手术有反应，并且分流手术后所有症状均可改善。长期护理包括调查任何对分流手术无反应或在分流手术最初改善后恶化的症状的鉴别诊断。
总结
神经科医生在识别应评估是否可能患有iNPH的患者方面发挥着重要作用。通过当代诊断测试和可编程分流器治疗，分流手术的风险效益比非常有利。对于更复杂的患者，世界各地都有在复杂iNPH方面有专业知识的三级中心。
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