Normal-pressure hydrocephalus

Peter Hedera MD PhD (Dr. Hedera of Vanderbilt University has no relevant financial relationships to disclose.)
Robert P Friedland MD (Dr. Friedland of the University of Louisville School of Medicine has no relevant financial relationships to disclose.)
Martin R Farlow MD, editor. (Dr. Farlow of Indiana University received research grant support from Accera, Biogen, Eisai,  Eli Lilly, Genentech, Roche, Lundbeck, Chase Pharmaceuticals, Novartis, Suven Life Sciences Ltd, and Boehringer Ingelheim; honorariums from Eisai, Forest Laboratories, Pfizer, Eli Lilly and Company and Novartis for speaking engagements; and fees from Accera, Alltech, Avanir, Axovant, Biogen, Eisai Med Res, Inc., Eli Lilly and Company, FORUM Pharmaceuticals, Genentech, Inc., Grifols, Helicon, INC Research, Lundbeck, Medavante, Medivation, Merck, Neurotrope Biosciences, Novartis, Pfizer, Prana, QR Pharm., Riovant Sciences Inc., Roche, Sanofi-Aventis, Schering-Plough, Toyama Pharm, Stemedica Cell Technologies Inc., vTv Therapeutics and UCB Pharma for consultancy. His spouse was employed by Eli Lilly.)
Originally released April 22, 1994; last updated June 29, 2017; expires June 29, 2020

This article includes discussion of normal-pressure hydrocephalus, communicating hydrocephalus, Hakim-Adams syndrome, hydrocephalic dementia, hydrostatic hydrocephalus, internal hydrocephalus, low-pressure hydrocephalus, nonobstructive hydrocephalus, tension hydrocephalus, and variotensive hydrocephalus. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Normal-pressure hydrocephalus is characterized by gait disorder, cognitive decline, and urinary incontinence, and shunt surgery is the only established effective treatment. In this updated article, the authors provide an overview of clinical features suggesting normal-pressure hydrocephalus as well as diagnostic approaches to establishing this diagnosis and selecting patients who have the highest likelihood of improvement after shunting. Although normal-pressure hydrocephalus is relatively rare, prompt recognition and surgical treatment of this condition can significantly reverse clinical features and improve the long-term outcome of these patients.

Key points

 

• Normal-pressure hydrocephalus is characterized by gait disorder, cognitive decline, and urinary incontinence.

 

• Prompt recognition and treatment may reverse clinical symptoms of normal-pressure hydrocephalus.

 

• Diagnosis of normal-pressure hydrocephalus is based on a combination of clinical symptoms, neuroradiologic abnormalities, and transient improvement after CSF drainage.

 

• Shunt surgery is the only established treatment of normal-pressure hydrocephalus.

Historical note and terminology

The triad of progressive dementia, gait disturbances, and urinary incontinence was originally described as a distinct syndrome by Adams and colleagues (Adams et al 1965; Hakim and Adams 1965). Several other terms have been used to describe this condition, reflecting uncertainties about the pathogenesis of normal-pressure hydrocephalus: "communicating," "low-pressure," "nonobstructive," "internal," "tension," "hydrostatic," "intermittent-tension," and "variotensive" hydrocephalus. The eponym "Hakim-Adams syndrome" is also occasionally used.

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