Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus: Second Edition

Abstract

Among the various disorders manifesting dementia, gait disturbance, and urinary incontinence in the elderly population, idiopathic normal pressure hydrocephalus (iNPH) is becoming of great importance. After the publication of the first edition of the Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus in 2004 (the English version was published in 2008), clinical awareness of iNPH has risen dramatically, and the number of shunt surgeries has increased rapidly across Japan. Clinical and basic research on iNPH has increased significantly, and more high-level evidence has since been generated. The second edition of the Japanese Guidelines was thus published in July 2011, to provide a series of timely evidence-based recommendations related to iNPH. The revision of the Guidelines has been undertaken by a multidisciplinary expert working group of the Japanese Society of Normal Pressure Hydrocephalus in conjunction with the Japanese Ministry of Health, Labour and Welfare research project on``Studies on the epidemiology, pathophysiology, and treatment of normal pressure hydrocephalus.'' This English version of the second edition of the Guidelines was made to share these ideas with the international community and to promote international research on iNPH.

Nationwide hospital‐based survey of idiopathic normal pressure hydrocephalus in Japan: Epidemiological and clinical characteristics

“…Treatments recommended in the guidelines are VP shunt (ventriculoperitoneal shunt), VA shunt (ventriculoatrial shunt) and, if the patient shows no spinal canal stenosis and adequate CSF passage, LP shunt (lumboperitoneal shunt) (Marmarou, Bergsneider, Klinge, Relkin, & Black, 2005; Marmarou, Black, Bergsneider, Klinge, & Relkin, 2005; Marmarou, Black, Bergsneider, Klinge, & Relkin, 2005; Mori et al., 2012; Relkin, Marmarou, Klinge, Bergsneider, & Black, 2005; Sasaki et al., 2008; Toma, Holl, Kitchen, & Watkins, 2011). In this survey, LP shunt was the first choice (55.1%) of treatment for iNPH, followed by VP shunt (43.2%), suggesting that, at present, these two shunt operations are the mainstays for the treatment of iNPH.…”

“…While iNPH has been shown to be treatable by shunt operation, as its name suggests, its fundamental etiology or pathology has yet to be clarified. In 2012, the Guideline (Mori et al., 2012) proposed three diagnostic levels, possible, probable, and definite, and recommended treating the disease accordingly.…”

“…This study was performed, using diagnostic criteria based on the Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus: Second Edition, 2012 (Mori et al., 2012). Analysis was performed by dividing the patients into three diagnostic levels, possible, probable, and definite, according to the Guidelines.…”

“…In this survey, all selected medical centers were asked to follow the Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus: Second Edition, 2012 (Mori et al., 2012), which was published and widely available in our country. According to the guideline, the CSF removal test is performed by removal of a small volume of CSF (approximately 30–50 ml) via lumbar puncture (tap test).…”

Complications of Lumboperitoneal Shunts for Normal Pressure Hydrocephalus

“…However, in Japan, normal pressure hydrocephalus is not infrequently managed with lumboperitoneal (LP) shunt surgery [5]. In some institutions, the LP shunt comprises up to 40% of all CSF shunting procedures [6].…”