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Evidence that congenital hydrocephalus is a precursor to idiopathic normal pressure hydrocephalus in only a subset of patients
  1. Robin K Wilson,
  2. Michael A Williams
  1. Adult Hydrocephalus Program, Department of Neurology, Johns Hopkins Hospital, Baltimore, Maryland, USA
  1. Correspondence to:
 Dr R K Wilson
 Johns Hopkins Adult Hydrocephalus Program, 600 N. Wolfe Street, Phipps 100, Baltimore, MD 21287, USA; Rwilso21{at}


Objective: To identify the proportion of patients in a large idiopathic normal pressure hydrocephalus (INPH) cohort with large head circumference (HC) who presumably have congenital hydrocephalus that has not become clinically apparent until late in life.

Methods: HC was measured in 158 patients diagnosed with communicating INPH and assigned to HC centile range adjusted for height and sex. The proportion of patients with INPH and HC above the 97th, 90th or 50th centiles was compared with the proportion expected in a normal population.

Results: The proportion of patients with HC >90th centile was significantly larger than would be expected in a normal distribution (19.6% vs 10%, p = 0.0001), as was the proportion of patients with HC >97th centile (8.9% vs 3%, p = 0.0001). The relative association between INPH and HC >97th centile was nearly tripled (relative association 2.95; CI 1.36 to 6.41), but the relative association between INPH and HC >50th centile was not significantly higher than predicted (relative association 1.13; CI 0.95 to 1.34).

Conclusion: A significantly larger proportion of elderly adults with INPH have a HC greater than the 90th or 97th centile than predicted by population norms, supporting the concept that compensated congenital hydrocephalus that does not become symptomatic until late in life is one aetiology of INPH, but is not responsible for all INPH. The mechanism that leads to the development of INPH in most patients remains elusive.

  • BEH, benign external hydrocephalus
  • CSF, cerebrospinal fluid
  • HC, head circumference
  • INPH, idiopathic normal pressure hydrocephalus
  • LIAS, late-onset idiopathic aqueductal stenosis
  • SHYMA, syndrome of hydrocephalus in young and middle-aged adults

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  • Published Online First 3 January 2007

  • Funding: None.

  • Competing interests: RKW has no personal or institutional financial interest in drugs, materials or devices described in this report. In the 12 months prior to the submission of this manuscript, MAW has received honoraria from Medtronic for speaking on the topic of adult hydrocephalus. The Johns Hopkins Adult Hydrocephalus Program has received support from Medtronic for research related to adult hydrocephalus, but not related to the topic of this manuscript.