[Pathological findings in a case of normal pressure hydrocephalus (author's transl)].
Pathological findings in a case of normal pressure hydrocephalus are reported with a review of the literature.
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Pathological findings in a case of normal pressure hydrocephalus are reported with a review of the literature.
After reviewing the literature on psychic disturbance in normal pressure hydrocephalus, the author reports on 26 cases of CSF drainage. Impairment of memory and concentration were always present in normal pressure hydrocephalus; behaviour disorders were very frequent and characterized by both types of frontal behaviour; the mental picture also comprised difficulties of calculation, writing, visuoconstructive praxis and, less frequently, impaired speech and reading ability. CSF drainage brought clinical improvement in 73% of the cases. Disorders of consciousness, mental function and behaviour regressed generally progressively as soon as the operation had been performed. Two major factors affecting therapeutic efficacity appear to be the patient's age and the length of time between onset of the symptoms and start of therapy.
OBJECTIVE: We tried to identify indications for cerebrospinal fluid shunting in patients with normal pressure hydrocephalus. METHODS: We studied the cerebral blood flow (CBF) and vascular response to acetazolamide in the white matter, cortex, and thalamus of 21 patients with normal pressure hydrocephalus, comparing patients who improved clinically after shunting with those who did not. We used xenon-enhanced computed tomography for the CBF measurements. RESULTS: Preoperatively, both groups had globally reduced CBF, but the reduction was more pronounced in the unimproved patients. The vascular response was impaired only in the white matter of the patients who improved later. After shunting, restoration of CBF, more marked in the white matter, and recovery of vascular response in the white matter paralleled clinical improvement and a reduction in ventricular dilation and periventricular lucency on computed tomographic scans in nine patients. The CBF reduction, however, deteriorated in the 12 patients who did not improve clinically. CONCLUSION: We conclude that the underlying disease in the improved patients was ischemia, with a loss of autoregulatory capacity in the periventricular white matter caused by cerebrospinal fluid diffusion. Those who did not improve had irreversible brain damage in which the CBF reduction was secondary to metabolic depression and autoregulation was preserved. We also conclude that patients suspected of having normal pressure hydrocephalus will improve clinically after shunting if preoperative hemispheric CBF is greater than 20 ml/100 g per minute and the vascular response to acetazolamide is impaired only in the periventricular white matter. They will not improve, however, if the preoperative CBF is less than 20 ml/100 g per minute and the vascular response to acetazolamide is intact.
Regional cerebral blood flow (rcbf) was studied preoperatively and at 2 and 6 months postoperatively in 22 normal pressure hydrocephalus patients using xenon-133 inhalation and single photon emission computed tomography. Sixteen of the 22 patients improved (improved group) and six did not (unimproved group). The following comparisons were made: (1) preoperative rcbf in the improved group, to 14 normal elderly volunteers and to that in 59 SDAT (senile dementia of the Alzheimer type) patients; (2) preoperative rcbf in the improved and unimproved groups to determine if rcbf could predict surgical outcome; (3) pre- to postoperative rcbf in the improved group to see if increased cbf accounted for clinical improvement. The findings were: (1) preoperative rcbf in the improved group was lower than that in normal controls but was the same as that in SDAT; however, the ratios of rcbf values in anterior and posterior brain regions were significantly different between improved group and SDAT (p = 0.02); (2) an anterior/posterior ratio of 1.05 correctly classified surgical outcome in 19/22 patients; five of six in the unimproved group were above this cut off while 14/16 in the improved group were below; (3) in the improved group rcbf increased at 2 but not at 6 months after surgery without a corresponding reduction of clinical signs, supporting the notion that increase in cbf probably does not account for clinical improvement in normal pressure hydrocephalus.
The combination of cerebral blood flow measurement using (15)O-water positron emission tomography with magnetic resonance coregistration and CSF infusion studies was used to study the global and regional changes in CBF with changes in CSF pressure in 15 patients with normal pressure hydrocephalus. With increases in CSF pressure, there was a variable increase in arterial blood pressure between individuals and global CBF was reduced, including in the cerebellum. Regionally, mean CBF decreased in the thalamus and basal ganglia, as well as in white matter regions. These reductions in CBF were significantly correlated with changes in the CSF pressure and with proximity to the ventricles. A three-dimensional finite-element analysis was used to analyze the effects on ventricular size and the distribution of stress during infusion. To study regional cerebral autoregulation in patients with possible normal pressure hydrocephalus, a sensitive CBF technique is required that provides absolute, not relative normalized, values for regional CBF and an adequate change in cerebral perfusion pressure must be provoked.
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OBJECTIVE: To compare the lumbar infusion test and the cerebrospinal fluid (CSF) tap test for predicting the outcome of shunt surgery in patients with suspected normal pressure hydrocephalus. METHODS: 68 patients with suspected normal pressure hydrocephalus were studied. The absence of preceding history indicated idiopathic disease in 75% of these. All patients were assessed twice with walking and psychometric tests before lumbar infusion test and tap test assessments. The lumbar infusion test was done using a constant infusion rate (0.80 ml/min) and regarded as positive if the steady state CSF plateau pressure reached levels of > 22 mm Hg (resistance to outflow > 14 mm Hg/ml/min). The tap test was regarded as positive if two or more of four different test items improved after CSF removal. As the variability in baseline test results was large, the better of two evaluations was used in comparisons with the results after CSF removal, as well as to evaluate the outcome after shunt surgery. Only patients with a positive lumbar infusion test or a positive tap test had surgery. RESULTS: The results of the CSF tap test and the lumbar infusion test agreed in only 45% of the patients. Of the total cohort, 47 (69%) had positive test results and were operated on; 45 (96%) of these reported subjective improvement, and postoperative assessments verified the improvements in 38 (81%). Improvements were highly significant in walking, memory, and reaction time tests (p < 0.001). Most of the patients improved by surgery (84%) were selected by a positive lumbar infusion test, and only 42% by a positive tap test. Positive predictive values were 80% for lumbar infusion test and 94% for tap test. The false negative predictions in the operated group were much higher (58%) with the tap test than with the lumbar infusion test (16%). CONCLUSIONS: Both the lumbar infusion test and the tap test can predict a positive outcome of shunt operations in unselected patients with suspected normal pressure hydrocephalus. The two tests are complementary and should be used together for optimal patient selection.
Idiopathic intracranial hypertension (IDICH) and idiopathic normal pressure hydrocephalus (IDNPH) are disorders of the circulation of the cerebrospinal fluid (CSF) with opposite diagnostic features, obscure etiology but the same treatment: CSF drainage. In both diseases a large quantity of CSF exists: equilibrated by the brain edema in IdICH with high ICP and not equilibrated by brain pressure, and the ventricles are enlarged in IdNPH. The same anatomical structure involved in both diseases is the ventricular wall and this represents a true barrier, a glial-ependymal barrier and it acts as a barrier only in connection with the difference between the pressures. The spontaneous course of both diseases suggests that an open glial-ependymal barrier protects the brain parenchyma.
BACKGROUND: 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. METHOD: 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. RESULT: 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. CONCLUSION: 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.
The authors report ten cases of normal pressure hydrocephalus treated by ventricular drainage. They pay particular attention to the neuropsychological and psychometric aspects and endeavour to identify the most characteristic nosological features by comparing their own clinical observations with those reported in the literature.
Although extrapyramidal features in normal pressure hydrocephalus (NPH) are not uncommon, presentations with Parkinson's syndrome as the predominant feature are rare and may give rise to diagnostic difficulties. Failure of patients with parkinsonism to respond to therapy, should alert one to the possibility of NPH.
58 patients were investigated with a lumbar infusion test for normal pressure hydrocephalus in the department of neurology of the National Hospital between January 1991 and December 1996. We present a retrospective evaluation of the investigation protocol for these patients with suspected normal pressure hydrocephalus. The aim of the study was to find out whether specific prognostic factors could be identified by routine investigations. 20 patients had been referred to the department of neurosurgery and were subsequently shunted. A critical review of the criteria leading to surgical treatment (clinical symptoms, cerebral CT scan, infusion test, cisternography) is presented. There was no single variable which alone could discriminate normal pressure hydrocephalus from other types of hydrocephalus, or predict the outcome of shunting. A more systematic referral and clinical assessment of the patients together with a more precise evaluation of the findings and improved follow-up procedures are necessary for optimal selection of patients for surgery. The study also shows the importance of diagnosing these patients early, as delay in treatment appeared to worsen prognosis after shunting.
OBJECTIVE: To determine if patients with normal pressure hydrocephalus (NPH) have larger head size than normal controls. BACKGROUND: In 1989, Graff-Radford and Godersky noted that 3 of 30 patients diagnosed with NPH had large heads. They hypothesized that the cause of NPH in their patients was arrested congenital hydrocephalus becoming symptomatic later in life. METHODS: Participants included 31 newly diagnosed NPH patients (21 male, 10 female) seen by the same neurologist (N.R.G.-R.) at Mayo Clinic Jacksonville from January 1, 1995 through December 31, 2000, and 459 normal controls (226 male, 233 female) from the Charlotte County Healthy Aging Study. RESULTS: Head size was statistically larger for NPH males (median, 59.0 cm, range, 57.0-63.0 cm) compared with normal males (median, 57.8 cm, range, 53.3-62.8) (p < 0.01). Head size was also larger for NPH females (median, 6.3 cm, range, 53.5-58.0 cm) compared with normal females (median, 54.6 cm, range, 51.4-59.7 cm) (p < 0.02). CONCLUSION: Patients with NPH have larger head circumferences as a group than normal controls. This was found in both males and females. Results suggest that a significant proportion of patients with NPH may have congenital hydrocephalus that becomes symptomatic later in life.
5 patients are described in which normal pressure hydrocephalus was diagnosed. The criteria on which the diagnosis was based were a cerebrospinal fluid perfusion test and an air study. Pathological verification was additionally obtained in 2 patients. The clinical picture in all patients was characterized by dementia. 4 patients were incontinent and 3 were paraspastic.