Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus (Third Edition): Endorsed by the Japanese Society of Normal Pressure Hydrocephalus

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Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus (Third Edition): Endorsed by the Japanese Society of Normal Pressure Hydrocephalus

Madoka Nakajima 1, Shigeki Yamada 2, Masakazu Miyajima 3, Kazunari Ishii 4, Nagato Kuriyama 5, Hiroaki Kazui 6, Hideki Kanemoto 7, Takashi Suehiro 7, Kenji Yoshiyama 7, Masahiro Kameda 8, Yoshinaga Kajimoto 9, Mitsuhito Mase 10, Hisayuki Murai 11, Daisuke Kita 12, Teruo Kimura 13, Naoyuki Samejima 14, Takahiko Tokuda 15, Mitsunobu Kaijima 16, Chihiro Akiba 3, Kaito Kawamura 1, Masamichi Atsuchi 17, Yoshihumi Hirata 18, Mitsunori Matsumae 19, Makoto Sasaki 20, Fumio Yamashita 20, Shigeki Aoki 21, Ryusuke Irie 21, Hiroji Miyake 22, Takeo Kato 23, Etsuro Mori 24, Masatsune Ishikawa 25, Isao Date 8, Hajime Arai 1, research committee of idiopathic normal pressure hydrocephalusAffiliations expand

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Abstract

Among the various disorders that manifest with gait disturbance, cognitive impairment, and urinary incontinence in the elderly population, idiopathic normal pressure hydrocephalus (iNPH) is becoming of great importance. The first edition of these guidelines for management of iNPH was published in 2004, and the second edition in 2012, to provide a series of timely, evidence-based recommendations related to iNPH. Since the last edition, clinical awareness of iNPH has risen dramatically, and clinical and basic research efforts on iNPH have increased significantly. This third edition of the guidelines was made to share these ideas with the international community and to promote international research on iNPH. The revision of the guidelines was 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. This revision proposes a new classification for NPH. The category of iNPH is clearly distinguished from NPH with congenital/developmental and acquired etiologies. Additionally, the essential role of disproportionately enlarged subarachnoid-space hydrocephalus (DESH) in the imaging diagnosis and decision for further management of iNPH is discussed in this edition. We created an algorithm for diagnosis and decision for shunt management. Diagnosis by biomarkers that distinguish prognosis has been also initiated. Therefore, diagnosis and treatment of iNPH have entered a new phase. We hope that this third edition of the guidelines will help patients, their families, and healthcare professionals involved in treating iNPH.

Keywords: clinical guideline; diagnosis; idiopathic normal pressure hydrocephalus; management; treatment

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References

  1. Hakim S, Adams RD: The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci 2: 307–327, 1965 – PubMed

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