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Vol 58, No 1 (2024)
Invited Review Article
Submitted: 2023-09-09
Accepted: 2023-10-31
Published online: 2023-12-06
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Normal pressure hydrocephalus, or Hakim syndrome: review and update

Philip W. Tipton1, Benjamin D. Elder2, Petrice M. Cogswell3, Neill Graff-Radford1
DOI: 10.5603/pjnns.97343
·
Pubmed: 38054275
·
Neurol Neurochir Pol 2024;58(1):8-20.
Affiliations
  1. Department of Neurology, Mayo Clinic, Jacksonville, Florida, United States
  2. Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, United States
  3. Department of Neuroradiology, Mayo Clinic, Rochester, Minnesota, United States

open access

Vol 58, No 1 (2024)
INVITED REVIEW ARTICLES — LEADING TOPIC
Submitted: 2023-09-09
Accepted: 2023-10-31
Published online: 2023-12-06

Abstract

This review makes the case that idiopathic normal pressure hydrocephalus (iNPH) is an outdated term because new information indicates that the syndrome is less idiopathic and that the cerebrospinal fluid (CSF) pressure of normal individuals is affected by several factors such as body mass index, age, and sex. Our review updates the epidemiology of iNPH and provides a clinical approach to the management of these patients. All the clinical features of iNPH are common in older individuals, and each has many causes, so the diagnosis is difficult. The first step in reaching an accurate diagnosis is to address the possible contributory factors to the gait abnormality and determine what if any role iNPH may be playing. The two best diagnostic tests are neuroimaging and cerebrospinal fluid (CSF) diversion (large volume lumbar puncture or external lumbar drainage) with pre/post gait evaluation. This review provides an update on the growing evidence that vascular disease, impaired CSF absorption, congenital, and genetic factors all contribute to the pathogenesis of iNPH. We suggest replacing the term iNPH with the term Hakim syndrome (HS) in acknowledgement of the first person to describe this syndrome. Lastly, we discuss the improvements in shunt technology and surgical techniques that have decreased the risks and long-term complications of shunt surgery.

Abstract

This review makes the case that idiopathic normal pressure hydrocephalus (iNPH) is an outdated term because new information indicates that the syndrome is less idiopathic and that the cerebrospinal fluid (CSF) pressure of normal individuals is affected by several factors such as body mass index, age, and sex. Our review updates the epidemiology of iNPH and provides a clinical approach to the management of these patients. All the clinical features of iNPH are common in older individuals, and each has many causes, so the diagnosis is difficult. The first step in reaching an accurate diagnosis is to address the possible contributory factors to the gait abnormality and determine what if any role iNPH may be playing. The two best diagnostic tests are neuroimaging and cerebrospinal fluid (CSF) diversion (large volume lumbar puncture or external lumbar drainage) with pre/post gait evaluation. This review provides an update on the growing evidence that vascular disease, impaired CSF absorption, congenital, and genetic factors all contribute to the pathogenesis of iNPH. We suggest replacing the term iNPH with the term Hakim syndrome (HS) in acknowledgement of the first person to describe this syndrome. Lastly, we discuss the improvements in shunt technology and surgical techniques that have decreased the risks and long-term complications of shunt surgery.

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Keywords

normal pressure hydrocephalus, dementia, gait impairment, genetics

About this article
Title

Normal pressure hydrocephalus, or Hakim syndrome: review and update

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 58, No 1 (2024)

Article type

Invited Review Article

Pages

8-20

Published online

2023-12-06

Page views

978

Article views/downloads

820

DOI

10.5603/pjnns.97343

Pubmed

38054275

Bibliographic record

Neurol Neurochir Pol 2024;58(1):8-20.

Keywords

normal pressure hydrocephalus
dementia
gait impairment
genetics

Authors

Philip W. Tipton
Benjamin D. Elder
Petrice M. Cogswell
Neill Graff-Radford

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