Diagnosis and management of idiopathic normal-pressure hydrocephalus: a prospective study in 151 patients

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Object. The diagnosis and management of idiopathic normal-pressure hydrocephalus (NPH) remains controversial, particularly in selecting patients for shunt insertion. The use of clinical criteria coupled with imaging studies has limited effectiveness in predicting shunt success. The goal of this prospective study was to assess the usefulness of clinical criteria together with brain imaging studies, resistance testing, and external lumbar drainage (ELD) of cerebrospinal fluid (CSF) in determining which patients would most likely benefit from shunt surgery.

Methods. One hundred fifty-one patients considered at risk for idiopathic NPH were prospectively studied according to a fixed management protocol. The clinical criterion for idiopathic NPH included ventriculomegaly demonstrated on computerized tomography or magnetic resonance imaging studies combined with gait disturbance, incontinence, and dementia. Subsequently, all patients with a clinical diagnosis of idiopathic NPH underwent a lumbar tap for the measurement of CSF resistance. Following this procedure, patients were admitted to the hospital neurosurgical service for a 3-day ELD of CSF. Video assessment of gait and neuropsychological testing was conducted before and after drainage. A shunt procedure was then offered to patients who had experienced clinical improvement from ELD. Shunt outcome was assessed at 1 year postsurgery.

Conclusions. Data in this report affirm that gait improvement immediately following ELD is the best prognostic indicator of a positive shunt outcome, with an accuracy of prediction greater than 90%. Furthermore, bolus resistance testing is useful as a prognostic tool, does not require hospitalization, can be performed in an outpatient setting, and has an overall accuracy of 72% in predicting successful ELD outcome. Equally important is the finding that improvement with shunt surgery is independent of age up to the ninth decade of life in patients who improved on ELD.

Article Information

Address reprint requests to: Anthony Marmarou, Ph.D., Department of Neurosurgery, Virginia Commonwealth University Medical Center, 1001 East Broad Street, Suite 235, Richmond, Virginia 23219–0449. email: marmarou@hsc.vcu.edu.

© AANS, except where prohibited by US copyright law.

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Figures

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    Bar graph demonstrating opening pressures measured electronically in 151 patients with idiopathic NPH during lumbar infusion studies. Pressure was allowed to stabilize for 30 seconds before taking a reading. The median pressure was 9 mm Hg. N = number of patients.

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    Bar graphs demonstrating the percentage of patients whose condition improved following ELD. Upper: Patients were classified according to the severity of gait disturbance. Although all patients shown experienced improvement, proportionally fewer patients with a severe gait disturbance improved after ELD. *p = 0.016, rank sum test. Center: Patients were classified according to the severity of dementia. Although all patients shown experienced improvement with ELD, those with mild or moderate memory loss had a greater tendency to improve with drainage compared with those patients with severe memory disturbance. **p = 0.006, rank sum test. Lower: A greater number of patients who had mild or moderate (mod) urinary incontinence on presentation demonstrated improvement following ELD compared with patients with severe incontinence. *p = 0.033, rank sum test).

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    Bar graph demonstrating neuropsychological profiles in patients suffering from idiopathic NPH whose condition improved compared with profiles in patients whose condition did not improve on ELD. Neuropsychological testing was performed before and immediately after removing the drain. Although most patients who improved on ELD seemed more responsive, more verbal, and more eager to engage in conversation during clinical rounds, this positive effect was not captured in the neuropsychological tests performed immediately postdrainage.

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    Graphs demonstrating examples of lumbar pressure response to bolus injections (4 ml) for the measurement of CSF outflow resistance. Upper: Pressure increased rapidly and then gradually returned to baseline values. This rate of recovery was typical for patients whose Ro was less than 4 mm Hg/ml/min. Lower: Repeated bolus injections (two) did not induce a rapid recovery of pressure. The Ro in this patient measured 6.7 mm Hg/ml/min and was typical for patients with Ro values greater than or equal to 4 mm Hg/ml/min. This Ro analysis performed in an outpatient setting has a sensitivity of 0.75 for predicting the outcome of an ELD performed in the hospital.

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    Magnetic resonance images (upper) obtained prior to shunt placement compared with CT scans (lower) obtained 1 year after shunt surgery in a 78-year-old woman with idiopathic NPH. The patient, initially wheelchair bound, experienced a remarkable improvement following ELD and subsequent placement of a ventriculoatrial (V-A) shunt with a programmable valve. In most patients, the improvement following shunt insertion did not always correlate with a reduction in ventricle size. Nevertheless, this case demonstrates the potential for tissue reconstitution in the elderly brain.

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    On discharge, patients and caregivers completed surveys on their daily responses to ELD (rated as better, same, or worse compared with predrainage symptoms). This graph depicts the assessments in patients who improved on ELD compared with those who did not improve. Patients who experienced improvement on ELD (circles) did so markedly immediately after discharge while in the home setting. This effect gradually decreased toward baseline levels over a 10-day period. In contrast, patients who did not improve (squares) remained symptomatic despite the mean 500 ml CSF drained. This survey was invaluable in assessing the risk/benefit ratio for surgical intervention.

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