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Idiopathic Normal Pressure Hydrocephalus

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Cerebrospinal Fluid Disorders

Abstract

Hydrocephalus is characterized by overaccumulation of cerebrospinal fluid (CSF) within the cerebral ventricles due to a mismatch between CSF production and CSF resorption into systemic circulation [1]. While many forms of hydrocephalus are associated with raised intracranial pressure (ICP) [2, 3], some patients have clinical and radiologic signs of hydrocephalus in the presence of low-to-normal ICP. This form of hydrocephalus was described in the 1960s by Salomon Hakim, who called it normal pressure hydrocephalus (NPH) [4–6]. The patients that Hakim described had an antecedent cause of hydrocephalus such as hemorrhage, trauma, or infection; however, patients were later found to have the NPH syndrome in the absence of known risk factors, which was designated idiopathic normal pressure hydrocephalus (iNPH), which now constitutes the majority of cases [7]. Thus, NPH can be either idiopathic or secondary to known risk factors [8–10]. iNPH is primarily observed in adult patients (age ≥65 years) and is clinically characterized by the presence of neurologic gait impairment plus additional features of a symptom triad—cognitive impairment and urinary incontinence [11].

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Authors and Affiliations

  1. Division of Biology and Biomedical Sciences, Washington University in St. Louis, St. Louis, MO, USA

    Albert M. Isaacs

  2. Division of Neurosurgery, Department of Clinical Neuroscience, University of Calgary, Calgary, AB, Canada

    Albert M. Isaacs & Mark G. Hamilton

  3. Departments of Neurology and Neurological Surgery, University of Washington School of Medicine, Seattle, WA, USA

    Michael A. Williams

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  1. Albert M. Isaacs
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  2. Mark G. Hamilton
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  3. Michael A. Williams
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Correspondence to Albert M. Isaacs .

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Editors and Affiliations

  1. Department of Neurological Surgery, Washington University School of Medicine, St. Louis Children’s Hospital, St. Louis, MO, USA

    David D. Limbrick Jr.

  2. Nationwide Children’s Hospital, Department of Neurosurgery, The Ohio State University College of Medicine, Columbus, OH, USA

    Jeffrey R. Leonard

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Isaacs, A.M., Hamilton, M.G., Williams, M.A. (2019). Idiopathic Normal Pressure Hydrocephalus. In: Limbrick Jr., D., Leonard, J. (eds) Cerebrospinal Fluid Disorders . Springer, Cham. https://doi.org/10.1007/978-3-319-97928-1_12

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  • DOI: https://doi.org/10.1007/978-3-319-97928-1_12

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