Idiopathic Adult Hydrocephalus Syndrome Research Articles

Sleep Disordered Breathing in Children and Adolescents with Chiari Malformation Type I

Chiari malformation Type I (CM-I) has been associated with sleep disordered breathing (SDB). The aim of this study was to evaluate the prevalence of SDB in CM-I and its clinical correlates in a population of children and adolescents. Fifty-three consecutive children and adolescents affected by CM-I were enrolled (27 girls and 26 boys, mean age 10.3 ± 4.3, range: 3-18 years). All patients underwent neurological examination, MRI, and polysomnography (PSG). Otorhinolaryngologic clinical evaluation was performed in patients with polysomnographic evidence of sleep-related upper airway obstruction. Mean size of the herniation was 9.5 ± 5.4 mm. Fourteen patients had syringomyelia, 5 had hydrocephalus, 31 presented neurological signs, 14 had epileptic seizures, and 7 reported poor sleep. PSG revealed SDB in 13 subjects. Patients with SDB, compared to those without SDB, had a higher prevalence hydrocephalus (p = 0.002), syringomyelia (p = 0.001), and neurological symptoms (p = 0.028). No significant difference was observed in age, gender, prevalence of epilepsy, and size of the herniation. Obstructive SDB was associated with syringomyelia (p = 0.004), whereas central SDB was associated with hydrocephalus (p = 0.034). In our population of CM-I patients the prevalence of SDB was 24%, lower than that reported in literature. Moreover, our findings suggest that abnormalities in cerebrospinal fluid dynamics in CM-I, particularly syringomyelia and hydro-cephalus, are associated with SDB.

Adaptive method for assessment of cerebrospinal fluid outflow conductance

Outflow conductance (C(out)) is important for predicting shunt responsiveness in patients with suspected idiopathic adult hydrocephalus syndrome (IAHS). C (out) is determined by performing an infusion test into the cerebrospinal fluid system, and the reliability of the test is dependent on the measurement time. The objective of this study was to develop an adaptive signal analysis method to reduce the investigation time, by taking the individual intracranial pressure variations of the patient into consideration. The method was evaluated on 28 patients with suspected IAHS. The results from full time investigations (60 min) were compared to the results of the new algorithm. Applying the new adaptive method resulted in a reduction of mean investigation time by 14.3 +/- 5.9 min (mean +/- SD), p<0.01. The reduction of reliability in the C(out) estimation was found clinically negligible. We thus recommend this adaptive method to be used when performing constant pressure infusion tests.

CSF Pulse Pressure and B Waves

Abstract Object. The appearance of numerous B waves during intracranial pressure (ICP) registration in patients with idiopathic adult hydrocephalus syndrome (IAHS) is considered to predict good outcome after shunt surgery. The aim of this study was to describe which physical parameters of the cerebrospinal fluid (CSF) system B-waves reflect and to find a method that could replace long-term B-wave analysis. Methods. Ten patients with IAHS were subjected to long-term registration of ICP and a lumbar constant-pressure infusion test. The B-wave presence, CSF outflow resistance (Rout), and relative pulse pressure coefficient (RPPC) were assessed using computerized analysis. The RPPC was introduced as a parameter reflecting the joint effect of elastance and pulsatory volume changes on ICP and was determined by relating ICP pulse amplitudes to mean ICP. Conclusions. The B-wave presence on ICP registration correlates strongly with RPPC (r = 0.91, p &lt; 0.001, 10 patients) but not with CSF Rout. This correlation indicates that B waves—like RPPC—primarily reflect the ability of the CSF system to reallocate and store liquid rather than absorb it. The RPPC-assessing lumbar short-term CSF pulse pressure method could replace the intracranial long-term B-wave analysis.

Brain energy metabolism and intracranial pressure in idiopathic adult hydrocephalus syndrome

Background: The symptoms in idiopathic adult hydrocephalus syndrome (IAHS) are consistent with pathology involving the periventricular white matter, presumably reflecting ischaemia and CSF hydrodynamic disturbance. Objective: To investigate whether a...

Cerebrospinal fluid pulse pressure method: a possible substitute for the examination of B waves

The appearance of numerous B waves during intracranial pressure (ICP) registration in patients with idiopathic adult hydrocephalus syndrome (IAHS) is considered to predict good outcome after shunt surgery. The aim of this study was to describe which physical parameters of the cerebrospinal fluid (CSF) system B-waves reflect and to find a method that could replace long-term B-wave analysis. Ten patients with IAHS were subjected to long-term registration of ICP and a lumbar constant-pressure infusion test. The B-wave presence, CSF outflow resistance (R(out)), and relative pulse pressure coefficient (RPPC) were assessed using computerized analysis. The RPPC was introduced as a parameter reflecting the joint effect of elastance and pulsatory volume changes on ICP and was determined by relating ICP pulse amplitudes to mean ICP. The B-wave presence on ICP registration correlates strongly with RPPC (r = 0.91, p < 0.001, 10 patients) but not with CSF R(out). This correlation indicates that B waves-like RPPC-primarily reflect the ability of the CSF system to reallocate and store liquid rather than absorb it. The RPPC-assessing lumbar short-term CSF pulse pressure method could replace the intracranial long-term B-wave analysis.

Intracerebral microdialysis and CSF hydrodynamics in idiopathic adult hydrocephalus syndrome

Background: In idiopathic adult hydrocephalus syndrome (IAHS), a pathophysiological model of “chronic ischaemia” caused by an arteriosclerotic process in association with a CSF hydrodynamic disturbance has been proposed. Objective: To...

Effects of transient and persistent cerebrospinal fluid drainage on sleep disordered breathing in patients with idiopathic adult hydrocephalus syndrome

OBJECTIVESTo examine sleep disordered breathing including obstructive sleep apnoea in patients with idiopathic adult hydrocephalus syndrome (IAHS) and to study the effects of CSF drainage and shunting procedure on sleep...

Regional cerebral blood flow, white matter abnormalities, and cerebrospinal fluid hydrodynamics in patients with idiopathic adult hydrocephalus syndrome.

OBJECTIVES--(1) to evaluate regional cerebral blood flow (rCBF) with single photon emission computed tomography and 99mTc-hexamethylpropyleneamine oxime in patients with the idiopathic adult hydrocephalus syndrome (IAHS); (2) to examine regional cerebral blood flow (rCBF), gait, and psychometric functions before and after CSF removal (CSF tap test); (3) to assess abnormalities in subcortical white matter by MRI. METHODS--Thirty one patients fulfilling the criteria for IAHS (according to history and clinical and neuroradiological examination) were studied. Quantified gait measurements, psychometric testing, and rCBF before and after removal of CSF were obtained. Pressure of CSF and CSF outflow conductance were investigated with a constant pressure infusion method. Brain MRI was used to quantify the severity of white matter lesions and periventricular hyperintensities. In IAHS a widespread rCBF hypoperfusion pattern was depicted, with a caudal frontal and temporal grey matter and subcortical white matter reduction of rCBF as the dominant feature. Removal of CSF was not accompanied by a concomitant increase in rCBF. Significant white matter lesions were detected only in a minority of patients by MRI. An altered CSF hydrodynamic state with a higher CSF pressure and lower conductance was confirmed. IAHS is characterised by an abnormal CSF hydrodynamic state, associated with a widespread rCBF reduction with preference for subcortical white matter and frontal-temporal cortical regions. Furthermore in most patients MRI did not show white matter changes suggestive of a coexistent subcortical arteriosclerotic encephalopathy. At least in the idiopathic group of patients with AHS, measurements of rCBF before and after temporary relief of the CSF hydrodynamic disturbance will not provide additional information that would be helpful in the preoperative evaluation but is suggestive of a preserved autoregulation of rCBF.

The predictive value of cerebrospinal fluid dynamic tests in patients with th idiopathic adult hydrocephalus syndrome.

To evaluate the predictive value of the cerebrospinal fluid (CSF) tap test and CSF outflow conductance in the selection of patients with the idiopathic adult hydrocephalus syndrome, defined exclusively on a clinical basis, for shunt surgery. A prospective, consecutive case series. All patients were assessed before surgery and at 3 months after shunt placement. Preoperatively, CSF pressure, conductance, and CSF formation rate were assessed by a constant-pressure infusion method. Improvement in gait and cognitive functions after removal of CSF was noted (ie, with the CSF tap test). Postoperatively, the infusion method was used to evaluate shunt function. Tertiary, academic referral center. Thirty-five patients with idiopathic adult hydrocephalus (normal-pressure hydrocephalus) syndrome based on conservative clinical criteria. No predictive tests were used for inclusion. All patients had a typical gait disturbance and a communicating hydrocephalus. Twenty-eight patients also had dementia or incontinence, or both. The CSF diversion (Cordis, [Orbis-Sigma]) valve, six patients; Hakim's standard system (Cordis Hakim standard system), 29 patients. OUTCOMES OF SURGERY: Serial videotaping of gait, a comprehensive neuropsychologic battery, and the Bartel index of activities of daily life. Gait was improved in 25 (72%) of the 35 patients, whereas the Bartel index remained unchanged. The conditions of five of seven patients with gait disturbance as the sole symptom improved. The spatial function (37% improved) and the findings from the Fuld object memory tests (29% improved) were significantly improved. Shunt dysfunction could not explain the lack of effect of an operation in the remaining patients. It should be noted that these results obtained at 3 months postoperatively may not be applicable in a long-term perspective. The CSF outflow conductance or CSF tap test were not able to identify those patients who would or would not benefit from a CSF diversion procedure. However patients had a lower conductance and a higher CSF pressure than did control subjects. Considerable improvement in gait was seen, but cognitive function was little affected. The CSF tap test or conductance does not provide additional information that is necessary to distinguish between patients whose conditions will or will not respond to shunting, when selection for surgery is based on conservative clinical criteria.

Cerebrospinal fluid shunt dynamics in patients with idiopathic adult hydrocephalus syndrome.

The objective was to assess CSF dynamics of different shunt constructions in patients with adult hydrocephalus syndrome and correlate these findings to clinical outcome, neuroradiology, and the specifications of the shunts provided by the manufacturer. Thirty four patients with idiopathic adult hydrocephalus (normal pressure hydrocephalus) syndrome were included in a prospective, consecutive case series. A differential pressure valve (Cordis Hakim standard system) was used in 28 patients and a variable resistance valve (Cordis Orbis-Sigma) in six. A constant pressure infusion method was used; CSF pressure and conductance were determined before surgery. Three months after shunt placement CSF pressure, the "pressure v flow" curve, and gravity induced flow were measured. There was no difference between mean preoperative and postoperative resting CSF pressures in patients with Hakim shunts. The opening pressures of the Hakim shunts were higher than the value proposed by the manufacturer. A pronounced gravity effect induced CSF flow and decrease of the CSF pressure. In functioning variable resistance valves, CSF dynamics normalised postoperatively. There was no gravity effect and the characteristics shaped "pressure v flow" curve was sometimes seen. Six patients (three differential pressure valves, three variable resistance valves) had non-functioning shunts. Four of these patients were improved after the operation but improvement was transient in three. In all patients, there was no relation between the width of the ventricles and clinical improvement or CSF pressure. In conclusion, the differential pressure valve system does not behave according to the specifications provided by the manufacturer. A decrease in CSF pressure in patients with this shunt was solely due to the effect of gravity. Eleven percent of the differential pressure valves and 50% of the variable resistance valves were non-functioning. In the functioning variable resistance valves, the antisiphon system seems to be effective.