The Challenges of Normal Pressure Hydrocephalus: A Case-Based Review

Clinician Reviews. 2015 December;25(12):44-49
December 1, 2015 | Clinician Reviews
Freddi Segal-Gidan, PA, PhD
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Freddi Segal-Gidan

Freddi Segal-Gidan is Director of the Rancho Los Amigos/University of Southern California (USC) Alzheimer’s Disease Center and Assistant Clinical Professor in the departments of Neurology and Family Medicine at Keck School of Medicine, USC, and in Gerontology at L. Davis School of Gerontology at USC, Los Angeles.

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Idiopathic normal pressure hydrocephalus (iNPH) is one of the few reversible causes ofdementia. Unfortunately, the symptoms of iNPH—cognitive impairment, gait change, andurinary incontinence—develop slowly and are often mistaken for those ofother conditions or for normal aging. This article explains when to suspect iNPHand the steps you need to take when iNPH is in the differential.

PROGNOSIS
The symptoms of iNPH are slowly progressive. Early recognition and intervention have been shown to improve outcomes.28 Long-term improvement following shunt surgery has been reported in up to 75% of patients when there is proper patient selection.13 A large body of literature has focused on proper patient selection and outcome predictors for shunting (see Table 2).

Gait and imbalance have repeatedly been reported to improve the most from shunting, particularly when gait disturbance precedes cognitive ­decline.29,30 Cognitive impairment, particularly once it reaches the degree of dementia, is least responsive to shunt placement, with only about 50% of patients experiencing improvement in cognition ­postsurgery.31

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The SINPHONI study (Study of Idiopathic Normal Pressure Hydrocephalus on Neurological Improvement) conducted in Japan found that mild impairment in any of the triad symptoms (gait, cognition, urinary incontinence) prior to shunt surgery predicted disappearance of symptoms following surgery; in addition, younger age was a predictor of disappearance of gait disturbance.32 Complete disappearance of symptoms is often not achievable, but significant improvement in symptoms may be a more attainable outcome goal. Long-term follow-up has found that symptom improvement is sustained in up to 25% to 47% of patients over three to five years.33,34

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