Current research of idiopathic normal pressure hydrocephalus: Pathogenesis, diagnosis and treatment

Idiopathic normal pressure hydrocephalus (iNPH) is caused by impaired cerebrospinal fluid absorption in the elderly; it is a surgically treatable form of dementia. Gait disturbance, dementia, and urinary incontinence are the triad of signs for iNPH. In addition to these clinical findings, imaging studies show characteristic ventricular enlargement. High Evans Index and ‘disproportionately enlarged subarachnoid hydrocephalus’ are other well-known imaging findings of iNPH. If the tap test shows improved symptoms, shunt surgery is performed. The disease was first described by Hakim and Adams in 1965, followed by the publication of the first, second, and third editions of the guidelines in 2004, 2012, and 2020, respectively. Recent studies signal the glymphatic system and classical cerebrospinal fluid (CSF) absorption from the dural lymphatics as aetiological mechanisms of CSF retention. Research is also underway on imaging test and biomarker developments for more precise diagnosis, shunting technique options with fewer sequelae and complications, and the influence of genetics. Particularly, the newly introduced ‘suspected iNPH’ in the third edition of the guidelines may be useful for earlier diagnosis. However, less well-studied areas remain, such as pharmacotherapy in non-operative indications and neurological findings other than the triadic signs. This review briefly presents previous research on these and future issues.