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Diagnosis and Treatment of Idiopathic Normal Pressure Hydrocephalus

Williams, Michael A. MD, FAAN; Malm, Jan MD, PhD

CONTINUUM: Lifelong Learning in Neurology: April 2016 - Volume 22 - Issue 2, Dementia - p 579–599
doi: 10.1212/CON.0000000000000305
Review Articles

Purpose of Review: This article provides neurologists with a pragmatic approach to the diagnosis and treatment of idiopathic normal pressure hydrocephalus (iNPH), including an overview of: (1) key symptoms and examination and radiologic findings; (2) use of appropriate tests to determine the patient’s likelihood of shunt responsiveness; (3) appropriate referral to tertiary centers with expertise in complex iNPH; and (4) the contribution of neurologists to the care of patients with iNPH following shunt surgery.

Recent Findings: The prevalence of iNPH is higher than previously estimated; however, only a fraction of persons with the disorder receive shunt surgery. iNPH should be considered as a diagnosis for patients with unexplained symmetric gait disturbance, a frontal-subcortical pattern of cognitive impairment, and urinary urge incontinence, whose MRI scans show enlarged ventricles and whose comorbidities are not sufficient to explain their symptoms. Physiologically based tests, such as the tap test (large-volume lumbar puncture) or temporary spinal catheter insertion for external lumbar drainage with gait testing before and after CSF removal, or CSF infusion testing for measurement of CSF outflow resistance, can reliably identify patients who are likely to respond to shunt surgery. Properly selected patients have an 80% to 90% chance of responding to shunt surgery, and all symptoms can improve following shunt surgery. Longitudinal care involves investigating the differential diagnosis of any symptoms that either fail to respond to shunt surgery or that worsen after initial improvement from shunt surgery.

Summary: Neurologists play an important role in the identification of patients who should be evaluated for possible iNPH. With contemporary diagnostic tests and treatment with programmable shunts, the benefit-to-risk ratio of shunt surgery is highly favorable. For more complex patients, tertiary centers with expertise in complex iNPH are available throughout the world.

Address correspondence to Dr Michael A. Williams, University of Washington Medical Center, Department of Neurology, 1959 NE Pacific St, Box 356470, Seattle, WA 98195, maw99@uw.edu.

Relationship Disclosure: Dr Williams serves on the technical advisory board for and holds stock options in Aqueduct Critical Care, Inc, and has received personal compensation and travel expenses as a lecturer for Codman Neuro, Canada. Dr Williams has received research support from the National Space Biomedical Research Institute as principle investigator of study SMST02801, comparing the continuous noninvasive and invasive intracranial pressure management therapies, and as co-investigator of study CA02801, investigating the effects of microgravity on intracranial pressure. Dr Williams has received research support from NeuroDx Development for research funded by the National Institutes of Health. Professor Malm receives royalty payments from Likvor AB, where he holds patents related to the CELDA infusion device, which is approved within the European Union, but not in the United States, and receives payments for a patent of a new CSF shunt design created with Medtronic, Inc. Professor Malm receives research support as principal investigator for studies from the Swedish Heart-Lung Foundation and the Swedish National Space Board.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Williams and Professor Malm report no disclosures.

© 2016 American Academy of Neurology
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