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Idiopathic normal pressure hydrocephalus

Background

Overview

Definition
INPH is a neurological disorder characterized by an imbalance in the production and absorption of CSF, leading to ventriculomegaly without elevated CSF pressure.
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Pathophysiology
INPH is caused by altered CSF dynamics, with inadequate CSF re-absorption or insufficient volume buffering reserve being key factors.
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Epidemiology
The incidence of INPH in Spain is estimated at 120 per 100,000 person-years in adults aged > 70 years.
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Disease course
Clinical manifestations include headaches increasing in frequency and severity, visual darkening, pulsating tinnitus, as well as dizziness, neck pain, back pain, and cognitive impairment.
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Prognosis and risk of recurrence
The prognosis of INPH can be unpredictable. While symptoms typically progress over time, the rate and severity of progression can vary among patients.
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Guidelines

Key sources

The following summarized guidelines for the management of idiopathic normal pressure hydrocephalus are prepared by our editorial team based on guidelines from the American Academy of Neurology (AAN 2015).
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Surgical interventions

Ventriculo-peritoneal shunt: as per AAN 2015 guidelines, consider offering shunting to relieve symptoms of INPH as well as associated gait disturbances.
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Patient education

General counseling: as per AAN 2015 guidelines, inform patients with INPH with elevated outflow resistance that they have an increased chance of responding to shunting compared with those without such elevation.
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