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Intracranial pressure parameters in idiopathic normal pressure hydrocephalus patients treated with ventriculo-peritoneal shunts.

BACKGROUND: Although the mean intracranial pressure (ICP) is normal in patients with idiopathic normal pressure hydrocephalus (iNPH), there could possibly be alterations in their single ICP waves. METHOD: Thirty-nine consecutive patients treated for iNPH with ventriculo-peritoneal shunts were followed prospectively with regard to clinical and radiological findings. Changes in clinical state 12 months after shunt surgery were assessed as change on a 15-3 score NPH Grading Scale, while the changes in ventricular size were assessed by linear measures. The ICP recordings were performed as part of routine pre-operative assessment, stored as raw data files, and analyzed retrospectively. The mean ICP as well as single ICP wave amplitudes were computed and analysed in consecutive 6 second time windows. FINDINGS: Twelve months after shunt surgery, changes in NPH score of 5 or more (very significant improvement) were observed in 12 patients (31%), of 3 to 4 (significant improvement) in 6 patients (15%), of 1 to 2 (slight improvement) in 9 patients (23%) and of -4 to 0 (non-responders) in 12 patients (31%). The ventricular size did not change in any of the outcome categories. While the pre-operative mean ICP was similar between outcome groups, the mean ICP wave amplitude was significantly higher in patients improving clinically as compared to the non-responders. CONCLUSIONS: While pre-operative mean ICP was similar, the mean ICP wave amplitudes were significantly higher in iNPH patients improving clinically after shunt treatment as compared to the non-responders.

Adult↗

Cine-MR imaging of aqueductal CSF flow in normal pressure hydrocephalus syndrome before and after CSF shunt.

Reproducibility of the aqueductal CSF signal intensity on a gradient echo cine-MR sequence exploiting through plane inflow enhancement was tested in 11 patients with normal or dilated ventricles. Seven patients with normal pressure hydrocephalus (NPH) syndrome were investigated with the sequence before and after CSF shunting. Two patients exhibiting central flow void within a hyperintense aqueductal CSF improved after surgery and the flow void disappeared after shunting. One patient with increased maximum and minimum aqueductal CSF signal as compared to 18 healthy controls also improved and the aqueductal CSF signal was considerably decreased after shunting. Three patients with aqueductal CSF values similar to those in the controls did not improve, notwithstanding their maximum aqueductal CSF signals decreasing slightly after shunting. No appreciable aqueductal CSF flow related enhancement consistent with non-communicating hydrocephalus was found in the last NPH patient who improved after surgery. Cine-MR with inflow technique yields a reproducible evaluation of flow-related aqueductal CSF signal changes which might help in identifying shunt responsive NPH patients. These are likely to be those with hyperdynamic aqueductal CSF or aqueductal obstruction.

Adolescent↗

Dutch Normal-Pressure Hydrocephalus Study: randomized comparison of low- and medium-pressure shunts.

OBJECT: The goal of this prospective study was to compare outcome after placement of a low- or medium-pressure shunt in patients with normal-pressure hydrocephalus (NPH). METHODS: Ninety-six patients with NPH were randomized to receive a low-pressure ventriculoperitoneal shunt (LPV; 40 +/- 10 mm H2O) or medium high-pressure ventriculoperitoneal shunt (MPV; 100 +/- 10 mm H2O). The patients' gait disturbance and dementia were quantified by applying an NPH scale, and their level of disability was evaluated by using the modified Rankin scale (mRS). Patients were examined prior to and 1, 3, 6, 9, and 12 months after surgery. Primary outcome measures were determined by differences between preoperative and last NPH scale scores and mRS grades. The LPV and MPV shunt groups were compared by calculating both the differences between mean improvements and the proportions of patients showing improvement. Intention-to-treat analysis of mRS grades yielded a mean improvement of 1.27 +/- 1.41 for patients with LPV shunts and 0.68 +/- 1.58 for patients with MPV shunts (p = 0.06). Improvement was found in 74% of patients with LPV shunts and in 53% of patients with MPV shunts (p = 0.06) and a marked-to-excellent improvement in 45% of patients with LPV shunts and 28% of patients with MPV shunts (p = 0.12). All outcome measures indicated trends in favor of the LPV shunt group, with only the dementia scale reaching significance. After exclusion of serious events and deaths unrelated to NPH, efficacy analysis showed the advantage of LPV shunts to be diminished. Reduction in ventricular size was also significantly greater for patients in the LPV shunt group (p = 0.009). Subdural effusions occurred in 71% of patients with an LPV shunt and in 34% with an MPV shunt; however, their influence on patient outcome was limited. CONCLUSIONS: Outcome was better for patients who had an LPV shunt than for those with an MPV shunt, although most differences were not statistically significant. The authors advise that patients with NPH be treated with an LPV shunt.

Aged↗

Current treatment of normal-pressure hydrocephalus: comparison of flow-regulated and differential-pressure shunt valves.

FROM THE RECORDS of approximately 1500 shunt operations performed between 1987 and 1992, we identified 37 adults between ages 38 and 86 years (mean, 70 yr) with the normal-pressure hydrocephalus (NPH) syndrome who underwent surgery by a single surgeon. Since 1990, we have routinely used a flow-regulated shunt system (Orbis-Sigma valve [OSV]; Cordis Corporation, Miami, FL) in these patients. In this study, we compared the OSV system with conventional differential-pressure (DP) shunt systems uniformly used before 1990. This series (n = 37) consisted of 62% men (n = 23) and 38% women (n = 14). We excluded all patients with hydrocephalus associated with central nervous system neoplasms, intracerebral hemorrhage, or trauma as well those with radiographically documented late-onset aqueductal stenosis. All patients presented with the NPH clinical syndrome, chiefly with magnetic gait. In addition, 75% of patients experienced cognitive loss and 59% experienced urinary incontinence. The mean duration of preoperative symptoms was 35 months (range, 7-120 mo). Eight patients (22%) had undergone previous shunting procedures before referral to our service. A total of 89 shunt operations were performed in the 37 patients. Using actuarial methods and controlling for a history of prior shunt surgery, we found no significant difference in the time to initial malfunction (shunt survival) between the OSV and the DP shunts. There were three subdural hematomas and one infection in the OSV group compared with no complications in the DP valve group (P = 0.11). Thirty-six patients were available for follow-up, at a mean of 14 months after surgery. Nearly 90% of all patients experienced improvement in gait after shunting, regardless of the valve system that was used. There was one unrelated death. Realizing the limitations of a retrospective analysis and on the basis of the limited number of patients in this study, we conclude that using actuarial methods, we found no significant difference in shunt survival when comparing the OSV with the standard DP valve shunt systems with antisiphon devices in patients with NPH. Contrary to previous reports, the OSV is not free of overdrainage complications. Most patients (89%) with the NPH syndrome who primarily presented with gait disorder experienced significant improvement in gait after either OSV or DP shunting procedures when selected for surgery on the basis of the clinical syndrome and confirmatory radiographic data.

Adult↗

Positron emission tomography with [18F]fluorodeoxyglucose differentiates normal pressure hydrocephalus from Alzheimer-type dementia.

Because diagnostic criteria for normal pressure hydrocephalus have not been clearly determined, it is often difficult to differentiate patients with this potentially treatable condition from those with Alzheimer-type dementia. We have studied three patients with normal pressure hydrocephalus, 17 patients with Alzheimer-type dementia, and seven healthy elderly controls using positron emission tomography and [18F]Fluorodeoxyglucose (FDG). Both Alzheimer-type dementia and normal pressure hydrocephalus groups showed lower cortical rates of FDG utilisation than controls. However, the patterns of metabolic abnormality were distinctly different in the two dementia groups, with Alzheimer-type dementia subjects demonstrating bilateral temporoparietal hypometabolism while normal pressure hydrocephalus subjects showed globally diminished glucose use.

Aged↗

Normal pressure hydrocephalus and Parkinson's disease.

Three patients with normal pressure hydrocephalus and Parkinson's disease are reported. The recognition of this association is important because these two entities require specific therapeutic approaches. The presence of Parkinson's disease does not preclude an excellent response of the hydrocephalus to a shunting procedure. Although several reports of cases with the characteristic clinical manifestations of normal pressure hydrocephalus--progressive dementia, gait difficulty and urinary incontinence--have been published earlier, it was Adams and Hakim who emphasized the clinical triad and the effect of shunting the cerebrospinal fluid as a means of treatment. Messert and Baker stressed that the gait disturbance had a close resemblance to the freezing gait of parkinsonism. We are reporting three patients who had both conditions. Recognition of the existence of both disorders in the same patients is important since appropriate treatment of each of them led to marked improvement of their symptoms.

Aged↗

Predictability of intracranial pressure oscillations in patients with suspected normal pressure hydrocephalus by transcranial Doppler ultrasound.

Intracranial pressure (ICP) was monitored continuously for one night in 36 patients with suspected symptomatic normal pressure hydrocephalus (NPH) to identify patients who might benefit from subsequent shunting. In 33 of these patients middle cerebral artery (MCA) blood flow velocity by means of transcranial Doppler sonography (TCD) and ICP were recorded simultaneously. ICP B-waves always paralleled changes in the TCD signal (TCD B-wave equivalents). The relative frequency of ICP B-waves was predictable by TCD, albeit slightly underestimated due to a generally lower relative amplitude of the TCD B-wave equivalents. However, the same TCD B-wave equivalent amplitude could be accompanied by quite different ICP changes in different patients. Considering the baseline values in the absence of pressure waves, there was no significant relationship between ICP and TCD resistance index (Pourcelot) in different patients. Raising ICP by injection of 10 ml saline into the ventricle, however was accompanied by an increased TCD resistance index in the individual patient. As the relative frequency of B-wave activity is assumed to be an indicator for shunt responsiveness, continuous TCD monitoring can be used as a screening procedure to detect the presence and the relative frequency of B-wave activity in patients with suspected NPH. However, since neither the absolute ICP nor the amplitude of spontaneous oscillations can be predicted, TCD monitoring is not suitable to replace ICP monitoring.

Adult↗

Normal pressure hydrocephalus in a patient with systemic lupus erythematosus: a case report.

Neuropsychiatric symptoms in systemic lupus erythematosus (SLE) are varied. The exact etiology and appropriate treatment are difficult to determine. We describe a 43-year-old woman who was diagnosed 18 years earlier with SLE. She developed dementia, gait disturbance, urinary incontinence and deterioration of consciousness. Her cerebrospinal fluid pressure was not elevated. Brain computerized tomography scan revealed enlarged ventricle and cortical sulci. Normal pressure hydrocephalus (NPH) was diagnosed. She was treated by establishing a ventriculo-peritoneal shunt, resulting in the return of normal consciousness. But she still had slurred speech, slow mentation and poor calculation ability. Her urinary incontinence persisted. Along with a literature review on NPH, we discuss its etiology, diagnosis and treatment.

Adult↗

Correlation between decreased ventricular size and positive clinical outcome following shunt placement in patients with normal-pressure hydrocephalus.

OBJECT: It is well known that in patients with communicating hydrocephalus or normal-pressure hydrocephalus (NPH), ventricular size decreases following implantation of shunts with differential pressure valves. The aim of this study was to determine whether ventricular size correlates with a positive clinical outcome following shunt placement. METHODS: Hydrostatic valves (dual-switch valves) were implanted in 80 patients with NPH at Unfallkrankenhaus, Berlin, between September 1997 and January 2002. One year postoperatively, these patients underwent computerized tomography scanning, and their ventricular size was ascertained using the Evans Index. Among 80% of the patients who showed no postoperative change in ventricular volume, 59% nonetheless had good to excellent clinical improvements, 17% satisfactory improvement, and 24% no improvement. Furthermore, a moderate reduction in ventricular size was observed in 14% of patients in this cohort. Among these, 36% experienced good to excellent clinical improvements, 28% satisfactory improvement, and 36% unsatisfactory improvement. A marked reduction in ventricular size was observed in 6% of the patients. Of this latter group, 60% demonstrated good to excellent outcomes, whereas 40% had unsatisfactory outcomes. CONCLUSIONS: Favorable outcomes following the implantation of a hydrostatic shunt in patients with NPH did not correlate with decreased ventricular volume 1 year after surgery. In fact, better clinical outcomes were observed in patients with little or no alteration in ventricular size, compared with those in patients with a marked decrease in ventricular size. A postoperative change in ventricular volume should be assessed differently in patients with NPH compared with those suffering from hypertensive hydrocephalus.

Aged↗

Normal pressure hydrocephalus in children and young adults.

Normal pressure hydrocephalus (NPH) is generally considered to be a disorder of adult and geriatric patients. We report four patients who are children or young adults with chronic neurological disorders, recent deterioration of their levels of function, normal cerebrospinal fluid (CSF) pressures, and ventricular enlargement. All four patients improved after the placement or revision of a ventriculoperitoneal shunt. Frequent symptoms and signs included irritability (three patients), vomiting (three patients), and abnormal limb posturing (two patients). Correct diagnosis was hampered by two factors: (a) Multiple or prolonged recordings of CSF pressures were invariably well within the normal ranges with respect to age, and (b) the patients had chronic neurological deficits. After ventriculoperitoneal shunting, subjective and objective improvement was seen in all cases. Young patients with large ventricles may benefit from shunting procedures despite low CSF pressures. These patients may be clinically identified by symptoms of new neurological dysfunction in cases of previously static neurological disease or acceleration of slowly progressive neurological dysfunction. Some of these underlying neurological disorders may predispose children and young adults to NPH.

Adult↗

[Chronic neuropathy, a high level of protein in cerebrospinal fluid, and vitamin B1 and folate deficiency in a patient with normal-pressure hydrocephalus].

A 67-year-old woman presented with a 1-year history of gradual weight loss, reduced mental activity, muscle weakness, and urinary dysfunction. Neurological examination revealed mild lethargy, severe muscular atrophy, and diminished deep tendon reflexes in the extremities. The levels of vitamin B1 and folate in blood were low: 1.9 micrograms/dl (normal range 2.0-7.2) and 0.7 ng/ml (normal range 4.0-12.0). respectively. A lumbar puncture was done. The pressure of the cerebrospinal fluid was within normal limits, the level of protein was very high (467 mg/dl), and only a few lymphocytes were seen. A nerve-conduction study showed low amplitudes of action potentials and slow conduction velocities in both the motor and sensory nerves. Myelin irregularity, "onion bulb formation", and axonal atrophy were seen in a specimen obtained by sural nerve biopsy. A T2-weighted magnetic resonance image of the brain showed ventricular dilatation, high-intensity signals around the lateral ventricles, and a flow-void sign of the cerebral aqueduct. Radioisotope cisternography (111In-DTPA) disclosed ventricular reflux and slow clearance of the tracer from the ventricles. These findings indicated the presence of chronic inflammatory demyelinating polyneuropathy, nutritional polyneuropathy, vitamin B1 deficiency, folate deficiency, and normal pressure hydrocephalus. In this patient, the high level of protein in the cerebrospinal fluid may have caused the hydrocephalus.

Aged↗

Idiopathic normal pressure hydrocephalus: analysis of factors related to cerebrospinal fluid dynamics determining functional prognosis.

This investigation has been undertaken to analyze the findings with both the cerebrospinal fluid (CSF) pressure (Pcsf) and CSF pulse pressure (PP) in order to predict the outcome of patients with the syndrome of idiopathic normal pressure hydrocephalus (NPH). Accordingly, a prospective clinical study was planned in which two groups of patients with NPH, having analogous prevalence of several matched clinical and radiological parameters, were separated on the basis of their positive or negative response to shunting. Both the resting Pcsf and CSF PP profiles were compared in these two groups, and between them and normal controls. CSF PP amplitude and CSF PP latency correlated directly in conditions associated with either normal or high compliance (controls and patients with Alzheimer-like disorders), whereas this correlation was inverse in states of low compliance (NPH). On the other hand, shunt-responders showed a resting Pcsf significantly higher than both non-responders and controls. The following conclusions were obtained: 1) CSF PP is a high-amplitude and relative low-latency wave in NPH when compared with controls: 2) CSF PP amplitude and latency correlate directly in normal subjects and in those with primary cerebral atrophy; 3) a non-reversible stage of NPH could be conceived in contradistinction to the reversible one, in both of which an inverse correlation between the amplitude and the latency takes place, the main difference between them being the resting Pcsf, which is significantly lower in the former than in the latter, depending on the degree of atrophic changes developed.

Adult↗

[Infusion test in the normal pressure hydrocephalus (author's transl)].

Continuous monitoring of the intracranial pressure and ventricular infusion test were carried out in 13 cases in which normal pressure hydrocephalus (NPH) was suspected. The infusion test was performed by intraventricular bolus injection of 8 ml of saline. The result was collated with the effect of shunting operation, which was judged by means of the improvement in the clinical symptoms and the amplitude of contingent negative variation. The excellent effect of shunting operation was obtained in the patients who showed the T of more than 200 sec, the compliance of less than 1.0 ml/mmHg, the conductance to outflow of less than 0.8 ml/mmHg/min and CSF outflow resistance of more than 15 mmHg/ml/min. In 3 cases out of the 5 effective cases, A or B wave was seen. However, appreciable variation of the values was shown among the cases so that it was concluded that the all-round-consideration must be done to select the case for the shunting operation with the findings in RI-cisternography, pneumoencephalography, computerized tomography and so on. This fact might indicate the existence of the complicated pathophysiology in NPH resulted from not only the impairment of cerebrospinal fluid circulation but also the disturbance of cerebral blood flow, changes in plasticity of the brain and so on.

Adult↗

Depression and normal pressure hydrocephalus. A dilemma in neuropsychiatric differential diagnosis.

Normal pressure hydrocephalus (NPH) is a surgically correctable syndrome of progressive dementia, gait abnormalities, and urinary incontinence resulting from an occult hydrocephalus in association with normal cerebrospinal fluid pressure. Occurring most frequently in midlife and often idiopathic in origin, the early course of the illness may be characterized by symptoms of apathy, inattentiveness, agitation, and poverty of thought which mimic a depressive illness and may delay the recognition and treatment of the underlying structural defect. A review of the literature reveals that this association of depressive symptomatology and NPH has received little attention in the psychiatric literature, and the authors describe a case of NPH which presented as a severe, agitated depression. Clinical findings which suggest the presence of NPH are discussed, and the need to include NPH in the differential diagnosis of depression in the presenium is emphasized. The authors believe such diagnostic vigilance is necessary if the needless deterioration of potentially salvageable individuals is to be prevented.

Age Factors↗

On the optimal opening pressure of hydrostatic valves in cases of idiopathic normal-pressure hydrocephalus: a prospective randomized study with 123 patients.

Does the opening pressure of hydrostatic shunts influence the clinical outcome for patients suffering from idiopathic normal-pressure hydrocephalus (NPH)? Between September 1997 and January 2003, 123 patients with idiopathic NPH were surgically treated by implanting a hydrostatic shunt at the Departments of Neurosurgery of the Unfallkrankenhaus Berlin and the University Homburg/Saar. As part of a prospective randomized study, all patients were examined preoperatively, postoperatively, and 1 year after the intervention. Forty-three percent of the patients showed a very good outcome, 25% good outcome, 20% fair outcome, and 12% poor outcome 1 year after the shunt implantation. Patients treated with an opening pressure rating of 50 mmH2O in the low-pressure stage of the gravitational valve showed a better outcome than those with an opening pressure of 100 or 130 mmH2O. According to present knowledge, hydrostatic shunts with an opening pressure of 50 mmH2O for the low-pressure stage are the best option for patients with idiopathic NPH. Due to the prompt switching function when the patient changes posture (lying down, standing, sitting, slanting etc.), the Miethke gravity-assisted valve (GAV) is more suitable in such cases than the Miethke Dual-Switch valve (DSV).

Adult↗

Ventricular size after shunting for idiopathic normal pressure hydrocephalus.

It has been presumed that idiopathic normal pressure hydrocephalus is due to obstruction. We visualized the ventricles in 19 patients subsequent to shunting. We found no consistent relationship between clinical improvement after shunting and reduction in ventricular size. This suggests that the positive effect of shunting in at least some instances is due to some other factor than reduced ventricular size.

Aged↗

Adjustable valves in normal-pressure hydrocephalus: a retrospective study of 218 patients.

OBJECTIVE: We sought to assess the value of adjusting shunt valve opening pressure, complications, and outcomes with the use of an adjustable shunt valve in the treatment of patients with normal-pressure hydrocephalus (NPH). METHODS: In a single-center retrospective study, 231 adjustable valves (range, 30-200 mm H(2)O) were the first shunt implantations in 147 patients with idiopathic NPH (INPH) and 71 patients with secondary NPH (SNPH). The effect of adjustment on gait disturbance, cognitive impairment, urinary incontinence and other symptoms were evaluated, and an improvement index was created. RESULTS: In the INPH group, 138 adjustments were performed in 49.0% of the patients (average, 0.94 adjustments/patient). For the SNPH group, 49 adjustments were performed in 32.4% of the patients (average, 0.69 adjustments/patient). The reasons for adjustment were overdrainage in 48 patients (25.7%), underdrainage in 98 patients (52.4%), subdural hematoma in 37 patients (19.8%), and other reasons in 2 patients (2.1%). Clinical status improved after 56 (49.1%) of all 114 adjustments, whereas 23 (42.6%) of 54 minor (< or =20 mm H2O) and 33 (66.0%) of 50 larger adjustments improved the patient's clinical status. The correlation of the improvement index with the size of the individual adjustments was not significant. Complications occurred in 43 (19.7%) of 218 patients, valve malfunction occurred in 3 patients (1.3%), infection occurred in 14 patients (6.4%), and nontraumatic subdural effusion occurred in 15 patients (6.9%; 8 were treated by adjustment alone). The 5-year shunt survival rate was 80.2%. Outcomes were excellent or good in 71 (78.9%) of 90 patients with INPH and in 30 (69.8%) of 43 patients with SNPH. CONCLUSION: Noninvasive, particularly consecutive, minor or single larger adjustments to the valve opening pressure can further improve outcome in patients with NPH who undergo shunting.

Adolescent↗