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Practice

Idiopathic normal-pressure hydrocephalus

Tina Hu and Yuna Lee
CMAJ January 07, 2019 191 (1) E15; DOI: https://doi.org/10.1503/cmaj.180877
Tina Hu
Department of Family and Community Medicine (Hu) and Division of General Internal Medicine (Lee), St. Michael’s Hospital, Toronto, Ont.
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Yuna Lee
Department of Family and Community Medicine (Hu) and Division of General Internal Medicine (Lee), St. Michael’s Hospital, Toronto, Ont.
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Idiopathic normal-pressure hydrocephalus presents a diagnostic challenge

Although the complete triad is not seen in all cases, idiopathic normal-pressure hydrocephalus is characterized by gait dysfunction, cognitive impairment and urinary incontinence. These symptoms, however, are frequently encountered in older adults without the condition.1 The onset of cognitive impairment in idiopathic normal-pressure hydrocephalus, which is often insidious with slow progression, may resemble Alzheimer disease,2 but is potentially reversable.3 The prevalence varies from 0.3% to 3% among patients older than 65 years, and increases with age.3

Gait dysfunction described as “freezing” and “magnetic” should trigger suspicion for the condition

Gait disturbance, typically the first symptom of idiopathic normal-pressure hydrocephalus, is characterized by a short-stepped gait and difficulty initiating movements, resulting in postural instability and falls.1,3 Some gait abnormalities in the condition may mimic Parkinson disease, but idiopathic normal-pressure hydrocephalus is associated with a wide gait and normal arm swing.3

Magnetic resonance imaging of the brain showing ventriculomegaly with no obstruction to the flow of cerebrospinal fluid supports the diagnosis

Ventriculomegaly seen on imaging may be a result of progressive cortical atrophy. However, ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement and cortical atrophy increases suspicion for idiopathic normal-pressure hydrocephalus.1

A high-volume lumbar puncture can be used as a confirmatory test and to select patients for surgery

The opening pressure is normal (8–18 mm Hg) in idiopathic normal-pressure hydrocephalus. Cognitive testing and a timed gait assessment should be conducted before and 30–60 minutes after the procedure.2 Improved gait after a high-volume lumbar puncture (removal of 30–60 mL of cerebrospinal fluid) is a positive predictor of improvement with a ventriculoperitoneal shunt.2

A prompt referral to a neurosurgeon is recommended in selected patients

Ventriculoperitoneal shunts can improve symptoms, especially gait dysfunction, in up to 74% of patients.4,5 Early treatment within 3 months of diagnosis is associated with better outcomes.5

CMAJ invites submissions to “Five things to know about …” Submit manuscripts online at http://mc.manuscriptcentral.com/cmaj

Footnotes

  • Competing interests: None declared.

  • This article has been peer reviewed.

References

  1. ↵
    1. Relkin N,
    2. Marmarou A,
    3. Klinge P,
    4. et al
    . Diagnosing idiopathic normal-pressure hydrocephalus. Neurosurgery 2005; 57(Suppl): S4–16.
    OpenUrlCrossRefPubMed
  2. ↵
    1. Tsakanikas D,
    2. Relkin N
    . Normal pressure hydrocephalus. Semin Neurol 2007;27:58–65.
    OpenUrlCrossRefPubMed
  3. ↵
    1. Nassar BR,
    2. Lippa CF
    . Idiopathic normal pressure hydrocephalus: a review for general practitioners. Gerontol Geriatr Med 2016;2:2333721416643702.
    OpenUrl
  4. ↵
    1. Gölz L,
    2. Ruppert F-H,
    3. Meier U,
    4. et al
    . Outcome of modern shunt therapy in patients with idiopathic normal pressure hydrocephalus 6 years postoperatively. J Neurosurg 2014;121: 771–5.
    OpenUrl
  5. ↵
    1. Andrén K,
    2. Wikkelsø C,
    3. Tisell M,
    4. et al
    . Natural course of idiopathic normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry 2014;85:806–10.
    OpenUrlAbstract/FREE Full Text
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Canadian Medical Association Journal: 191 (1)
CMAJ
Vol. 191, Issue 1
7 Jan 2019
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Idiopathic normal-pressure hydrocephalus
Tina Hu, Yuna Lee
CMAJ Jan 2019, 191 (1) E15; DOI: 10.1503/cmaj.180877

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Tina Hu, Yuna Lee
CMAJ Jan 2019, 191 (1) E15; DOI: 10.1503/cmaj.180877
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    • Idiopathic normal-pressure hydrocephalus presents a diagnostic challenge
    • Gait dysfunction described as “freezing” and “magnetic” should trigger suspicion for the condition
    • Magnetic resonance imaging of the brain showing ventriculomegaly with no obstruction to the flow of cerebrospinal fluid supports the diagnosis
    • A high-volume lumbar puncture can be used as a confirmatory test and to select patients for surgery
    • A prompt referral to a neurosurgeon is recommended in selected patients
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