Symptoms Of Normal Pressure Hydrocephalus Research Articles

LONG-TERM OUTCOME IN PATIENTS WITH SUSPECTED NORMAL PRESSURE HYDROCEPHALUS

To evaluate the outcome of patients with suspected normal pressure hydrocephalus at 6 months and 5 years after shunt surgery. Seventy-five patients (mean age, 72.5 6 9 yr), with normal pressure hydrocephalus symptoms were included. Fifty-four patients with positive lumbar infusion and/or cerebrospinal fluid tap tests received a cerebrospinal fluid shunt, whereas 21 patients with negative test results did not undergo operation. Walk, reaction time, memory, and identical forms tests were used as baseline (before surgery) tests and were repeated at short- (6.1 6 4.6 mo) and long-term (5.5 6 1.4 yr) follow-up evaluations. Activities of daily life functions were assessed using the Barthel index. At the 6-month follow-up examination, 83% of the operated patients improved in gait, 65% improved in reaction time, 46% improved in memory, and 31% improved in identical forms tests; 96% found themselves subjectively improved. Because of unrelated mortality (37%) and declining general health from comorbidity, only 27 patients were available for the 5-year follow-up evaluation. Twenty-three of these patients had been treated with a shunt and had a remaining improvement in close to 40% in gait and reaction time, whereas fewer than 10% had an improvement in cognitive tests. Fifty-six percent reported subjective improvement compared with preoperative findings. More patients (64%) improved if younger than 75 years; for patients older than 75 years, only 11% of the patients improved. The Barthel index was higher (P < 0.05) in improved patients. Patients with normal pressure hydrocephalus benefit from shunt surgery for at least 5 years. High mortality rate, comorbidity, and old age hamper good long-term outcome and emphasize the importance of patient selection.

IS AQUEDUCTAL STROKE VOLUME, MEASURED WITH CINE PHASE-CONTRAST MAGNETIC RESONANCE IMAGING SCANS USEFUL IN PREDICTING OUTCOME OF SHUNT SURGERY IN SUSPECTED NORMAL PRESSURE HYDROCEPHALUS?

To evaluate clinical usefulness of cerebrospinal fluid stroke volume (SV) assessed in the cerebral aqueduct, via cine phase-contrast magnetic resonance imaging, for predicting outcome after shunt surgery in suspected normal pressure hydrocephalus. Thirty-eight patients with suspected normal pressure hydrocephalus were included. SV was assessed using cine phase-contrast magnetic resonance imaging, and the results were kept blinded until postoperative follow-up after 7 +/- 5.8 months (mean +/- standard deviation). Selection to surgery was based on a positive lumbar infusion test or cerebrospinal fluid tap test, and outcome was evaluated with objective tests. Six patients were excluded from SV measurements because of technical difficulties. Eight patients were not operated (negative lumbar infusion test and cerebrospinal fluid tap test). SV in the not operated patients (mean, 66 +/- 53 microl) did not differ from the operated patients (95 +/- 78 microl; P = 0.335). Operated patients showed statistically significant improvements in walk (P = 0.020), reaction time (P = 0.006), and memory (P = 0.001) tests. Patients were divided into three groups according to SV range: low (0-50 microl), middle (51-100 microl), and high (>100 microl). No statistically significant (P > 0.05) improvements in any of the objective tests were found in any of the SV ranges. The numbers of individually improved patients were similar in the different SV ranges: six out of seven in the low, nine out of nine in the middle, and five out of eight in the high range. Weak correlations were found between SV and the initial pulse amplitude (Rs = 0.043; P = 0.014) as well as the plateau pulse amplitude (Rs = 0.043; P = 0.014) as measured with the lumbar infusion test. The data from this study show no evidence that cine phase-contrast magnetic resonance imaging measurements of SV in the cerebral aqueduct are useful for selecting patients with normal pressure hydrocephalus symptoms to shunt surgery.

Idiopathic normal pressure hydrocephalus may be a “Two Hit” disease: Benign external hydrocephalus in infancy followed by deep white matter ischemia in late adulthood

To determine if normal pressure hydrocephalus (NPH) could result from decreased resorption of cerebrospinal fluid (CSF) by the arachnoidal villi, leading to benign external hydrocephalus [BEH] in infancy, followed by deep white matter ischemia (DWMI) in late adulthood (the more hydrophilic environment increasing resistance to CSF flow through the extracellular space (ECS) of the brain). CSF outflow via the fourth ventricle and the ECS of the brain was mathematically modeled using a parallel electrical circuit analog. The apparent diffusion coefficient (ADC) was measured as a surrogate of the amount of water in the ECS in normals, patients with symptomatic NPH, and patients with dilated ventricles without symptoms of NPH ("pre-NPH"). The electrical circuit model demonstrates increasing ventricular volume with increasing resistance to flow through the ECS of the brain. ADC measurements performed in the centrum semiovale are significantly higher in patients with NPH and "pre-NPH" than in age-matched controls (P<0.05), controlling for the same degree of DWMI indicating increased fluid in the ECS of the brain. The electrical circuit analog and finding of increased periventricular ADC support the theory that NPH is a "two hit" disease.

Radionuclide Shuntogram Demonstrating Migration of Distal Ventriculoperitoneal Shunt Tubing out of the Peritoneal Cavity

An 81-year-old man with a history of ventriculoperitoneal (V-P) shunt placement for symptomatic normal pressure hydrocephalus presented with recurrence of confusion and gait disturbance. Radionuclide cisternography demonstrated loculation of In-111 DTPA in the abdominal wall. A soft tissue mass palpated at the location of tracer accumulation was confirmed to be extraperitoneal pooling of fluid at the site of coiling of the distal shunt tip. After surgical revision, with repositioning of the distal tube in the peritoneal cavity, the patient's symptoms promptly improved. When symptoms of hydrocephalus recur after successful V-P shunt placement, shunt malfunction should be suspected and evaluation for mechanical failure pursued. Catheter migration should be recognized as a correctable cause of shunt malfunction.

Slight elevation of baseline intracranial pressure after fluid infusion into CSF space in patients with hydrocephalus

Objective: To investigate the elevation of resting cerebrospinal fluid (CSF) pressure recorded after a CSF infusion test in patients with hydrocephalus.Material and methods: Fifty patients (30 men and 20 women, mean age 68 ± 13 years) with ventriculomegaly and clinical symptoms of normal pressure hydrocephalus have been studied. Lumbar (56%) or intraventricular (44%) computerized infusion studies were performed to investigate the hydrodynamics of CSF. After infusion, the fall in ICP was recorded until a steady-state level was achieved and the difference between pre- and post-infusion resting ICP was calculated (ΔICP).Results: A positive difference (>2 mm Hg) between post- and pre-infusion resting ICP was identified in 31 infusion tests (62%). The mean value of the difference was 6.7 with an SD of 3.5 mm Hg. The patients who demonstrated this phenomenon had a greater elastance coefficient (p>0.05); ΔICP was positively correlated with age (R=0.27; p=0.03), with the size of the brain's ventricles (R=0.63, p=0.03) and inversely with the severity of clinical impairment (Stein-Langfitt score R=–0.61, p=0.02; normal pressure hydrocephalus score: R=0.54; p<0.05). ΔICP was independent of the site of infusion (lumbar or ventricular).Conclusion: In patients with a 'stiffer' brain, ICP returns to the resting level after the infusion test at a slightly higher level than before the test. The magnitude of this increase is greater when ventricles are more dilated and clinical symptoms are less severe.

Multi-modal MRI in normal pressure hydrocephalus identifies pre-operative haemodynamic and diffusion coefficient changes in normal appearing white matter correlating with surgical outcome

Magnetic resonance techniques were used to investigate haemodynamic abnormalities and their consequences in normal pressure hydrocephalus (NPH) and to assess changes in these parameters following surgery. Eleven patients with NPH were studied pre- and post-operatively using perfusion and diffusion weighted imaging and compared with ten age-matched controls. Pre-operative periventricular relative cerebral blood volume (rCBV) was reduced in patients (0.76±0.11) compared with control (1.16±0.16, P<0.01). There was no difference between outcome groups and no change in haemodynamic parameters following surgery. The periventricular apparent diffusion coefficient (ADC) was elevated in the poor outcome group (1.67±0.3×10 −3 mm 2 s −1) compared with both controls (1.04±0.4×10 −3 mm 2 s −1, P<0.05) and the good outcome group (0.99±0.3×10 −3 mm 2 s −1, P<0.05) despite appearing normal on conventional imaging. In white matter hyperintensities (WMH), rCBV was reduced (0.70±0.12 vs. 1.00±0.10, P<0.01), and the ADC was increased (1.98±0.6 vs. 1.04±0.4×10 −3 mm 2 s −1, P<0.05) compared with the same anatomical location in controls. As low rCBV and high ADC is characteristic of chronic infarction, the findings in WMH regions suggest they are irreversibly damaged. Normal appearing periventricular tissue rCBV was reduced, implying that significant haemodynamic consequences contribute to symptoms in NPH. The elevated pre-operative ADC of the same region, was correlated with poor outcome, and may, therefore, be useful in selecting patients for surgery.

Reversal of Normal Pressure Hydrocephalus Symptoms by Subdural Collections

The pathogenesis of symptoms in normal pressure hydrocephalus (NPH) is unclear. One theory is that in the presence of enlarged lateral ventricles, the ventricular fluid exerts increased force on the ventricular walls, the cerebrospinal fluid pressure remaining normal. This is in accordance with Pascal's principle of F = PxA. It has not been possible to obtain direct evidence for this view. The opportunity to gain insight into the matter arose when it was observed that two patients with symptomatic NPH were relieved of their symptoms when they developed bilateral subdural collections, one after head trauma, the other after ventriculo-peritoneal shunting for NPH. In each case, concomitant with the subdural collections and the reversal of symptoms, the ventricular system became smaller. In the first patient, symptoms returned when the subdural collections were resorbed. In the second patient, symptoms returned when the subdural collections became excessive. Although documentation was less thorough than desired, it was concluded that relief of symptoms was related to the decrease in the ventricular size rather than lowering of the intraventricular pressure, thus providing evidence for operation of the principle F = PxA as the mechanism of symptoms in NPH.

Clinical features of late-onset idiopathic aqueductal stenosis

BACKGROUND Although late-onset idiopathic aqueductal stenosis (LIAS) is considered a good indication for endoscopic third ventriculostomy (ETV), the characteristics of this clinical entity have seldom been reported in the magnetic resonance (MR) imaging era. The authors reviewed their patients with LIAS who were treated by ETV to confirm its clinical features and response to treatment. METHODS The study group was composed of 31 patients diagnosed with LIAS who were previously untreated. Both anatomic and cine phase-contrast MR studies were used to detect the obstruction of the aqueduct canal. The preoperative symptoms were statistically analyzed in relation to the age of the patients and the degree of preoperative ventriculomegaly. RESULTS The overall success rate of ETV was 83.9% with mean follow-up duration of 26.2 months. Only two patients had acute onset (within one month) without any predisposing chronic symptoms. The patients with chronic symptoms (longer than 6 months [ n = 25]) can be divided into two major groups: a headache group ( n = 12) and a normal pressure hydrocephalus (NPH)-symptom group ( n = 11), Two patients had both headaches and NPH symptoms. The patients in the headache group were significantly younger and had smaller ventricles than the patients in the NPH group. With multivariate regression analysis, age independently affected the type of chronic symptoms. CONCLUSIONS LIAS typically presents with chronic onset, with younger patients tending to have headaches, and older patients tending to have NPH symptoms. ETV is considered the best surgical option, with more than an 80% success rate.

Spinal leptomeningeal metastases of giant cell glioblastoma associated with subarachnoid haemorrhage: case report

A case of subarachnoid haemorrhage (SAH) due to spinal leptomeningeal metastases of a giant cell glioblastoma is described. A 51 year old male presented with a four week history of headache. Neurological examination was normal except for a slight left h emiparesis. Computed tomography (CT) revealed a large cyst with a mural nodule in the right temporal lobe. The tumour was removed followed by 60Gy of radiation therapy. Thirty-two months later he developed headache and shoulder pain with symptoms of normal pressure hydrocephalus. Head CT showed ventriculomegaly and SAH. Magnetic resonance imaging showed spinal leptomeningeal metastases at the C4–5, T12, and L2 levels, but no local recurrence or tumour dissemination in the brain. He died 34 months after surgery. Autopsy revealed diffuse SAH over the whole brain and spinal cord, a ssociated with spinal leptomeningeal metastases, but no cerebral aneurysms. Spinal radiotherapy and ventriculoperitoneal shunting could possibly have extended survival in this patient.

Intracranial pressure B-waves precede corresponding arterial blood pressure oscillations in patients with suspected normal pressure hydrocephalus

B-waves are rhythmic oscillations of intracranial pressure (ICP) with a wavelength of 0.5-2 min. These oscillations are accompanied by corresponding fluctuations of arterial blood pressure (BP). There is controversy in the literature whether the ICP oscillations precede or follow the BP oscillations. This relationship, however, is important for the understanding of the origin and the interactions of ICP B-waves with other parameters. ICP and BP were monitored continuously for one night in nine patients with suspected symptomatic normal pressure hydrocephalus (NPH). Forty-five B-waves were identified and the time delays between the peaks and the onsets of B-waves and their BP-equivalents were measured. The mean (± standard deviation) of the time delays between the peaks of ICP B-waves and corresponding BP-oscillations was 4.2 ±2.4 sec and between the onsets of the oscillations 7 0.3 ±6.6 sec. In all but two B-waves, the ICP peak preceded the BP peak, in all the 45 B-waves the onset of the ICP increase preceded the BP increase (p < 0.004). Our study demonstrates that ICP B-waves precede concomitant changes in BP by several seconds. [Neurol Res 1999; 21: 627-630]

Psychophysiology, signs and symptoms of disease the course of normal pressure hydrocephalus

Between May 1982 until January 1997 we investigated 200 patients suspected for normal pressure hydrocephalus (NPH) by performing an intrathecal infusion test. According to our experiences gait ataxia is the leading symptom of NPH. Regarding dementia we could not find a significant difference compared to cerebral atrophy. Bladder incontinence can be characterised as a symptom of late-stage NPH. The complete Hakim trias should not be overestimated in differential diagnostic considerations. Graduation of NPH and cerebral atrophy after the results of the infusion test in an early and late stage enables prognostic evaluation of the course of disease. Patients with NPH in an early stage report in the follow-up on an improvement of their symptoms after shunt operation (65 percent of patients), whereas 50 percent of the patients with late stage NPH were improved. The computer-aided infusion test allows secure differentiation between patients with NPH and those with cerebral atrophy.

Movement disorders in adult hydrocephalus.

In a prospective series of symptomatic adult hydrocephalus characterized by gait disturbance, cognitive impairment, and/or urinary incontinence, 88 of 118 patients (75%) had additional akinetic, tremulous, hypertonic, or hyperkinetic movement disorders. Their prevalence was highest in patients with idiopathic normal pressure hydrocephalus (NPH) of the elderly (56/65 patients, 86%), and they were less frequent in patients with secondary NPH (10/15, 66%), with nonhydrodynamic atrophic/other hydrocephalus (20/33, 61%), and with obstructive hydrocephalus/aqueductal stenosis (2/5, 40%). Akinetic symptoms were found in 73 of 118 patients (62%), and the most frequent movement disorder was upper extremity bradykinesia (55%). Akinetic, tremulous, hypertonic, and hyperkinetic movement disorders were exclusively secondary to causes not related to hydrocephalus in 24 of 118 patients (20%). The proportion of patients with movement disorders not attributable to only such causes was highest in the idiopathic NPH group (44/65, 68%). Thirteen of 118 patients (11%) presented with a parkinsonian syndrome. There was evidence for coexistent Parkinson's disease in four of these patients. Parkinsonism was found to be secondary to NPH in five patients and was found improved after shunting. Akinetic symptoms in patients with NPH generally responded favorably to CSF diversion, which was evident in 80% of a subset of this group. Various other movement disorders did not show definite improvement. The high prevalence of bradykinesia and other akinetic symptoms in NPH and the beneficial effect of shunting on such symptoms suggest that NPH may cause a more generalized disorder of motor function.

Intracisternal schwannoma of the spinal accessory nerve presenting as a normal pressure hydrocephalus syndrome. Case report and review of the literature

Schwannomas of the spinal accessory nerve are rare lesions. They can be characterized by their locations as either intrajugular or intracisternal schwannomas, most of them being intrajugular. Only five cases arising in the cisterna magna are reported in literature. Clinical symptoms generally consist of 11th cranial nerve palsy associated with cerebellar signs and myelopathy. An additional case, studied by MRI, is described. The patient showed a mild cerebellar syndrome and normal pressure hydrocephalus symptoms. He was operated on and the mass completely removed with good postoperative results. The role of MRI for diagnosis of lower cranial nerve schwannomas is stressed, even though does not detect the exact nerve of origin. Total removal of these lesions is recommended, since their benign nature and unavoidable recurrence in case of partial excision.

Probabilistic diagnosis of normal pressure hydrocephalus and other treatable cerebral lesions in dementia

Clinical profiles of dementia patients, differing with respect to age, presence of gait abnormalities and urinary incontinence are discussed. Epidemiological data, subjective probabilities and clinical reasoning are used to predict a treatable cerebral lesion, i.e., an intracranial space occupying lesion or normal pressure hydrocephalus (NPH). Our calculations help in distinguishing clinically between demented patients who are, and who are not likely to benefit from computed tomography (CT) investigation for treatable lesions and, eventually, from cerebrospinal fluid shunting for NPH. Utility calculations show that shunting can be recommended only for a patient with the full triad of symptoms of NPH, and CT evidence of NPH, when no other prognostic information is available. Future clinical research should address the long-term prognosis of (treated) NPH patients, and the mortality of shunting, because these two factors are critical to the shunting decision.

Simultaneous recording of cerebrospinal fluid pressure and middle cerebral artery blood flow velocity in patients with suspected symptomatic normal pressure hydrocephalus.

CSF pressure (intracranial pressure, in one patient lumbar pressure) was monitored continuously for one night in 23 patients with suspected symptomatic normal pressure hydrocephalus (NPH) to identify patients who might benefit from subsequent shunt surgery. In 20 patients middle cerebral artery (MCA) blood flow velocity by means of transcranial Doppler sonography (TCD) and CSF pressure were recorded simultaneously. In three patients transcranial Doppler signals were insufficient. Spontaneous changes in CSF pressure always paralleled changes in the TCD signal. Equivalents of B-waves as well as intermediate waves (in between B- and A-waves), and C-waves could be identified easily and always appeared in phase. The Doppler signal, however, could not be used to evaluate the absolute changes in CSF pressure. Fast Fourier Transform of the Doppler signal was a useful tool to indicate the relative frequency of B-wave equivalents. In five patients the injection of 10ml saline into the ventricle raised intracranial pressure considerably, but hardly affected the MCA blood flow velocity. Continuous TCD monitoring might be useful as a noninvasive screening procedure in patients with suspected symptomatic NPH before continuous invasive CSF pressure measurements are performed.

Der Normaldruck - Hydrozephalus

A quarter of a century after the first description of a condition known as "normal pressure hydrocephalus", there no longer exists serious doubt about the existence of this disease nor about the possibility of treating it surgically with success. Nevertheless, there is still no general agreement on the exact definition of this condition, nor is there confirmed knowledge regarding its pathogenesis. Approximately half of the cases still are designated "idiopathic" in as much as physicians have no clear concept of its etiology. Some progress has been reached concerning the indication for operation: the "resistance to outflow" of cerebrospinal fluid can be measured now exactly by fluid infusion tests and seems to be a valuable predictor of outcome after shunting procedures. Reviewing world literature of the past 25 years reveals: the most important symptom of normal pressure hydrocephalus is not dementia, but disturbances of gait. Patients suffering from dementia, but not from gait-disturbances, do not gain from cerebrospinal fluid shunting and do not need to be investigated by invasive diagnostic procedures. In patients who suffer from gait-disturbances with or without dementia, with or without urinary incontinence, and in whom computed tomography reveals enlargement of ventricles without pronounced cortical atrophy, there should be a measurement of intracranial pressure and of the resistance to cerebrospinal fluid outflow.

Klüver-Bucy Syndrome Preceding Ventricular Enlargement in Normal-Pressure Hydrocephalus

Symptoms of normal-pressure hydrocephalus (NPH) and the complete Klüver-Bucy syndrome (KBS) developed in a previously healthy woman before the appearance of ventricular dilatation on computerized tomography. Ventricular enlargement typical of NPH occurred several months later. Following ventriculoperitoneal shunting, gait abnormalities and KBS resolved, while incontinence, memory, and intellect improved and have since remained stable. An associated sleep disturbance characterized by early onset of rapid-eye movement (REM) sleep periods and sleep spindles throughout REM sleep was also improved by treatment. NPH may impair periventricular function before ventricular dilatation becomes apparent.

The role of cerebrospinal compensatory parameters in the estimation of functioning of implanted shunt system in patients with communicating hydrocephalus (preliminary report).

Twelve patients presenting with communicating hydrocephalus were studied. In 9 cases where the mean ICP level remained below 10 mmHg the symptoms of normal pressure hydrocephalus were observed. All of the patients were treated by shunt system implantation. The clinical and psychological state, cranial computerized tomography and parameters of the cerebrospinal compensatory mechanisms, evaluated using the constant rate infusion test were compared before and after treatment. In most of the patients (11) the pathologically enlarged ventricles persisted. Only in three cases no clinical improvement was noticed. In this group the resistance to the cerebrospinal fluid absorption and the fluid formation rate were estimated as normal. In the group with improvement the normalization of the resistance (4), decrease in the cerebrospinal fluid formation rate (4) and decrease in the cerebrospinal system elasticity (1) can be pointed out as factors responsible for improvement manifested after shunting. Therefore the resorption resistance and the formation of cerebrospinal fluid should be considered as predictive factors in the shunt implantation in hydrocephalic patients, and play an important role in the diagnosis of this entity.

Normal-pressure hydrocephalus in rheumatic patients. A diagnostic pitfall.

In our rheumatologic clinics the diagnosis of idiopathic hydrocephalus has been made in six patients during the past three years. This disorder is characterized by the triad of gait disturbance, urinary incontinence, and dementia, all variable in severity.1 2 3 Since this diagnosis had not been made previously in our department of rheumatology, we assume that it has hitherto been overlooked. The mean period between the development of the first symptoms of normal-pressure hydrocephalus in our patients and the time when the disorder was diagnosed was 3.3 years; symptoms and signs were at first ascribed to arthritis and age. The main . . .

Posttraumatic hydrocephalus.

Posttraumatic hydrocephalus is a treatable complication of head injury and can present with several different clinical syndromes. These include obtundation; simple failure to improve; a tetrad of psychomotor retardation, memory loss, gait trouble, and incontinence; and unusual symptoms including emotional disorder. Posttraumatic hydrocephalus seems to result from a blockage of cerebrospinal fluid (CSF) flow around the cerebral convexities. When the lumbar CSF pressure is consistently above 180 mm H2O, the hydrocephalus should be treated unless a contraindication to operation exists. Underlying brain injury from the trauma itself, however, may prevent recovery. When the typical symptoms of normal pressure hydrocephalus (NPH) are present, a shunt is also indicated. Decision-making is difficult when the patient has normal pressure, but is too injured to display the symptoms of NPH or has atypical symptoms. Overnight pressure recording, lumboventricular infusion testing, or cisternography may clarify whether a shunt is needed in this situation, but there is still considerable uncertainty about shunt prediction in this setting.