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REVIEW
www.nature.com/clinicalpractice/neuro
The diagnosis and treatment of idiopathic normal pressure hydrocephalus
Gary L Gallia, Daniele Rigamonti and Michael A Williams*
SUMMARY
REVIEW CRITERIA
PubMed was used for a literature search from 1966 to the present for articleson normal pressure hydrocephalus. The abstracts of retrieved citations were reviewed and prioritized by relative content. Full publications were obtained and also searched for relevant references and related articles.
INTRODUCTION
Normal pressure hydrocephalus (NPH), which was first introduced into the English clinical literature in 1965, describes the syndrome of gait disturbance, cognitive deterioration and urinary incontinence that is associated with ventricular enlargement in the absence of elevated cerebro-spinal fluid (CSF) pressure.1,2 When it occurs secondarily to other disease processes, including subarachnoid hemorrhage, traumatic brain injury, cerebral infarction and meningitis, this syndrome is referred to as secondary NPH.3,4 NPH in patients without known precipitants is termed primary or idiopathic NPH (INPH). In 2002, an international NPH study group was assembled to develop guidelines for INPH. These guidelines, which were published in 2005, covered four major topics: the clinical diagnosis of INPH, the value of supplementary diagnostic tests, surgical management, and outcome of CSF shunting in INPH.59 Television and internet media campaigns have increased public awareness of INPH,10 and many patients and their families are now asking their physicians about the possibility of a diagnosis of NPH.
CLINICAL PRESENTATIONAND RADIOGRAPHIC EVALUATIONINPH is characterized clinically by the triad of gait disturbance, dementia and urinary incontinence.1,2 Symptoms typically develop insidiously, and generally occur between the sixth and eighth decades of life.1114 Gait disturbances are typically the first signs of INPH, and have been variously described as apraxic, bradykinetic, glue-footed, magnetic, parkinsonian and shuffling.8,15 Patients often present with a history of falls. The aberrant ambulation observed in INPH is characterized by a slow, wide-based gait, short shuffling steps, and difficulty in turning and tandem walking. Notably, there is no significant motor weakness.
The cognitive deficits are typically of the subcortical type, characterized by inattention, psychomotor retardation and difficulty with
Idiopathic normal pressure hydrocephalus (INPH) is a syndrome thatis characterized by gait impairment, cognitive decline and urinary incontinence, and is associated with ventriculomegaly in the absence of elevated cerebrospinal fluid (CSF) pressure. There is significant variation in the clinical presentation and progression of this...