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PubMed2007

Evidence that congenital hydrocephalus is a precursor to idiopathic normal pressure hydrocephalus in only a subset of patients.

OBJECTIVE: To identify the proportion of patients in a large idiopathic normal pressure hydrocephalus (INPH) cohort with large head circumference (HC) who presumably have congenital hydrocephalus that has not become clinically apparent until late in life. METHODS: HC was measured in 158 patients diagnosed with communicating INPH and assigned to HC centile range adjusted for height and sex. The proportion of patients with INPH and HC above the 97th, 90th or 50th centiles was compared with the proportion expected in a normal population. RESULTS: The proportion of patients with HC >90th centile was significantly larger than would be expected in a normal distribution (19.6% vs 10%, p = 0.0001), as was the proportion of patients with HC >97th centile (8.9% vs 3%, p = 0.0001). The relative association between INPH and HC >97th centile was nearly tripled (relative association 2.95; CI 1.36 to 6.41), but the relative association between INPH and HC >50th centile was not significantly higher than predicted (relative association 1.13; CI 0.95 to 1.34). CONCLUSION: A significantly larger proportion of elderly adults with INPH have a HC greater than the 90th or 97th centile than predicted by population norms, supporting the concept that compensated congenital hydrocephalus that does not become symptomatic until late in life is one aetiology of INPH, but is not responsible for all INPH. The mechanism that leads to the development of INPH in most patients remains elusive.

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PubMed2001

[Incidence of surgery for hydrocephalus in adults surveyed: same number afflicted by hydrocephalus as by multiple sclerosis].

The incidence of surgical treatment for adult hydrocephalus (older than 18 years) in Sweden from 1996 to 1998 was surveyed. The number of operations was 891 and the average incidence 3.36 operations per 100,000 inhabitants and year, varying regionally from 2.3 to 6.3. The mean age was 60 years (range 18-92), with no sex difference. Normal pressure hydrocephalus (47%) was most common, followed by communicating high pressure hydrocephalus (27%) and aqueductal stenosis (11%). 804 shunt operations (90%) and 67 ventriculostomies (7.5%) were performed, 2% were unclassified.

Adult↗
PubMed1978

[Normal pressure hydrocephalus. Part 1. Dynamic study of CSF circulation in patients with normal pressure hydrocephalus (author's transl)].

To clarify the pathogenesis of normal pressure hydrocephalus (NPH), quantitative measurement of CSF dynamics was attempted using RI techniques. In this study results concerning RI ventriculography and RI transfer test from CSF to plasma were reported. "Barrier ratio" and regional cerebral blood flow study in NPH will be reported elsewhere. Fifty-three patients with chronic communicating hydrocephalus wer devided into two groups. Thus, twenty-seven cases were diagnosed as NPH according to clinical symptoms and signs, and others were considered as mere chronic communicating hydrocephalus without any NPH characteristics. Results are as follows: 1) 169Yb--DTPA ventriculography; In NPH group, there was longer retention of RI injected into lateral ventricle, and RI activity was not detected in the cisterna magna 60 minutes after the injection. These findings show remarkable delay of CSF flow in NPH patients. 2) Transfer test of 169Yb--DTPA from CSF to plasma; Immediately after the RI injection, Transfer ratio of RI activity from CSF to plasma was measured. It was revealed that intraventricular RI was more rapidly transfered to plasma in NPH group compared with in the control group, suggesting accelerated trans-ependymal absorption of RI in NPH group.

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PubMed1979

Ventricular differences between hydrostatic hydrocephalus and hydrocephalus ex vacuo by computed tomography.

Transaxial CT scans of 100 patients with hydrostatic hydrocephalus and 50 patients with hydrocephalus ex vacuo have been reviewed with respect to measurements of: frontal horn ratio, width of the temporal horns, width of the third ventricle, width of cerebral fissures and sulci. The diagnosis of hydrostatic hydrocephalus is probable when (a) both temporal tips are visualized and measure 2 mm or greater in width and the sylvian and interhemispheric fissures and cerebral sulci are not visible, or (b) there is visualization of temporal horn tips measuring 2 mm or greater in width and the lateral ventricles are symmetrically enlarged with the frontal horn ratio 0.50 or more.

Cerebral Ventriculography↗
PubMed2003

"Hydrocephalus-parkinsonism complex": progressive hydrocephalus as a factor affecting extrapyramidal tract disorder-an experimental study.

OBJECTIVE: To experimentally clarify the symptomatological and pathophysiological aspects of a newly proposed clinical entity, we produced an experimental rat "hydrocephalus-parkinsonism complex" model. METHODS: A total of 60 male Sprague-Dawley rats were used. Twenty rats were treated with only kaolin as controls. Hemiparkinsonism was induced in the other 40 rats by stereotactic injection of 6-hydroxydopamine (6-OHDA) directly into the right substantia nigra. Twenty of these 40 rats were then treated with an injection of kaolin into the cisterna magna 3 days after the induction of parkinsonism. Neurological features were assessed weekly for 1 to 6 weeks after 6-OHDA injection by an apomorphine-induced turning test. RESULTS: The mortality rate was 25% in the rats injected with kaolin and 10% in those with the 6-OHDA injection. The frequency of the induction of parkinsonism-like signs was minimal in the first 2 weeks and reached a maximum point 4 weeks after the 6-OHDA injection. In contrast, the rats injected with both 6-OHDA and kaolin developed hemiparkinsonism-like signs in the early stage of the progression of hydrocephalus, and the peak incidence was reached in the 2nd week after induction ( p<0.05 compared with the 6-OHDA group). The severity of the neurological disturbance was also significantly more prominent in the latter group. CONCLUSION: These results suggest that the hydrodynamic effect in the early period of ventriculomegaly is the mechanism for signs of parkinsonism in 6-OHDA-treated rats. It could be concluded that the hydrocephalic state aggravates parkinsonism, if any as the associated condition, and it may be a new clinical entity of hydrocephalus symptomatology with a specific pathophysiology.

Animals↗
PubMedDate not supplied

Glial reaction in periventricular areas of the brainstem in fetal and neonatal posthemorrhagic hydrocephalus and congenital hydrocephalus.

This immunohistochemical, neuropathological study was performed on the ventricular wall of the brainstem in children with fetal and neonatal posthemorrhagic and congenital hydrocephalus. In posthemorrhagic hydrocephalus (PHH), hemosiderin deposits, nodular gliosis, ependymal cell loss and subependymal rosette formation developed subventricularly after 2 weeks of age. Ferritin-positive reactive microglia were well stained until about 2 weeks of age and thereafter diminished as reactive astrocytosis occurred. In congenital hydrocephalus (CH), the damage to the ventricular wall was less than in PHH at all ages and was associated with only mild astrogliosis. These differences in the neuropathological findings of periventricular regions, consisting mainly of glial reactions between PHH and CH, are due to differences in pathophysiology, and the timing of the insult in both conditions. The differences may be due to the effects of intraventricular hemorrhage and/or rapidly increased intracranial pressure in PHH. These results may have implications for the neurological prognosis in children with PHH.

Biomarkers↗
PubMed2002

Is normal pressure hydrocephalus a valid concept in 2002? A reappraisal in five questions and proposal for a new designation of the syndrome as "chronic hydrocephalus".

The authors question the current validity of the traditional concept of normal pressure hydrocephalus (NPH) as it was described by Adams and Hakim in 1965. The classic features of the disease are addressed. It is concluded that most of the historical statements made three decades ago need to be revised. Especially, the term "normal pressure" hydrocephalus probably does not match the actual manometric profile of patients with NPH. Similarly, the terms"curable"and "reversible" dementia are inadequate to designate the mental alterations of NPH. That NPH is non-specific to the adult population is also stressed, since it may be not uncommonly encountered in paediatrics, especially in an implanted shunt malfunction. The term "chronic hydrocephalus" without reference to cerebrospinal fluid pressure and to the age of the patient is proposed instead of NPH, which seems out of step with current knowledge of the pressure profile and with the diagnosis and decision making context in patients with so called NPH.

Age Factors↗
PubMed1987

[What future is there for hydrocephalus children? Intellectual and visual neurological prognosis in series of 77 cases of non-tumor hydrocephalus].

We report the neurological, intellectual and visual outcome of 77 children with hydrocephalus of non tumoral origin. Neurological deficits and epilepsy were common, particularly in those with severe intellectual deficit and with acquired brain damages. Intellectual level prognosis was usually good, although lower than in a healthy population: 75% of children had full intellectual quotient (FIQ) higher than 70 but often, there was an heterogeneous pattern of the IQ with greater differences between verbal and performance results because of poor visual-spatial skills. Attention and syntax comprehension of language disabilities were also specifically encountered. Meningitis or toxoplasmosis secondary to hydrocephalus often resulted in a poor intelligence level but other causes did not influence intelligence level or pattern. Visual-spatial deficits, attention disorders and language disabilities did not seem related to the degree of hydrocephalus or the delay before shunting, but were especially encountered when ventricular dilatation persisted in spite of the operation. Functional amblyopias were frequent, certainly related to strabismus. Optic atrophy was rare and linked to direct compression of the optic nerves or to major and repeated episodes of intracranial hypertension.

Child↗
PubMed1983

[Fontanelle pressure in infantile hydrocephalus--with special reference to 6 cases of hydrocephalus with normal head circumference and without shunting operation].

Follow-up study on ICP was carried out in 6 cases of hydrocephalus within normal head circumference for 8-9 months after birth. There were no cases with shunting operation. Abnormal plateau-like wave was recorded in 2 cases with myelomeningocele during follow-up period. In other cases of postemeningitic hydrocephalus, almost normal baseline pressure without abnormal pressure wave was recorded and all showed favorable morbidity (function). These results showed the importance of follow-up study on ICP for 9 months after birth in cases of hydrocephalus without large head circumference and shunting operation should be recommended in cases showing abnormal pressure wave recorded during follow-up period.

Age Factors↗
PubMed1989

Symptomatic congenital hydrocephalus in the elderly simulating normal pressure hydrocephalus.

In a series of 30 older patients shunted for symptomatic hydrocephalus, we found 3 with a head circumference at or greater than the 98th percentile. In 2, we demonstrated deterioration over 6 and 12 months by serial videotaping of gait and neuropsychological testing. In the 3rd, serial lumbar punctures over a 6-month period gave temporary improvement. In each, CTs showed ventriculomegaly without transependymal flow. One patient had an Arnold-Chiari type I abnormality identified by MRI. All had systemic hypertension. CSF pressure monitoring showed CSF pressure greater than 15 mm Hg 39% of the time in 1 patient, and 100% in another. All improved with ventriculoperitoneal shunting. Patients with probable compensated congenital hydrocephalus who functioned well throughout most of their lives may become symptomatic as they age but improve with shunt surgery. The head circumference should be measured in all older hydrocephalic patients.

Aged↗
PubMed2006

Idiopathic normal pressure hydrocephalus may be a "two hit" disease: benign external hydrocephalus in infancy followed by deep white matter ischemia in late adulthood.

PURPOSE: 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). MATERIALS AND METHODS: 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"). RESULTS: 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. CONCLUSION: The electrical circuit analog and finding of increased periventricular ADC support the theory that NPH is a "two hit" disease.

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PubMed1993

"Z" flow hydrocephalus shunt, a new approach to the problem of hydrocephalus, the rationale behind its design and the initial results of pressure monitoring after "Z" flow shunt implantation.

The over-drainage of CSF after shunt implantation, particularly when the patient is in the vertical posture, is well documented. Problems associated with it are negative pressure syndrome (postural headache and postural irritability), abnormal skull size, craniosynostosis and subdural haematoma. The pressure gradient in the vertical position between the cranial cavity and the right atrium is 15 to 20 cm of water and between the cranial and peritoneal cavity it is 25 to 32 cm of water and both these are much higher than those needed for the optimum functioning of a conventional shunt. The need, therefore, has been for a self-regulating device that can adjust itself to any change of posture from horizontal to vertical and vice versa and regulate the flow of CSF from the cranial cavity. "Z" Flow hydrocephalus shunt system has this in-built self-regulating mechanism to prevent over drainage of CSF and the resultant abnormal low pressure in the cranial cavity. The rationale behind the functioning of "Z" Flow hydrocephalus shunt system has been discussed, its design has been described and illustrated and the results of pressure monitoring after Z Flow shunt implantation in five adult patients have been detailed. As the opening pressures of the "Z" Flow shunt system are 15-29 cm of water, it has been found to prevent over-drainage of CSF and maintain intraventricular pressure within normal limits.

Adult↗
PubMed1999

A clinical survey of hydrocephalus and current treatment for hydrocephalus in Japan: analysis by nationwide questionnaire.

We analyzed current treatment for hydrocephalus in Japan by means of a nationwide questionnaire. Ventriculoperitoneal (VP) shunting was the most common form of cerebrospinal fluid (CSF) shunting, and in 66% of cases the ventricular catheter was inserted via the anterior horn. Various types of flow-control valves were used in 46% of CSF shunting procedures, and the proportion of cases in which such valves are used seems to be increasing. The Codman-Hakim valve was used most frequently, followed by the Sophy valve. The initial pressure setting of programmable pressure valves was selected in 82% of institutes on the basis of the CSF pressure obtained during the shunt operation. Most flow-control valves were implanted in the retroauricular region; however, Sophy valves were implanted in the chest wall in a high percentage of cases. The rate of shunt complications was significantly lower among VP shunt cases with flow-control valves than in those without flow-control valves. The most common cause of shunt complications was trouble with the abdominal catheter. These results will contribute to progress in the treatment for hydrocephalus.

Adult↗
PubMed1984

CSF dynamics in hydrocephalus--with special attention to external hydrocephalus.

Cerebrospinal fluid (CSF) physiology, and the pathophysiology of hydrocephalus is briefly reviewed, with emphasis on the arachnoid villae and the CSF-venous interface. Five cases of benign "external hydrocephalus" are recorded and the literature of similar cases is reviewed. It is proposed that anatomical or functional obstruction of various etiologies at the level of the arachnoid villae accounts for the dilated subarachnoid pathways in these patients.

Absorption↗
PubMed1983

[Critical observations on the therapy of hydrocephalus using so-called "arrested" hydrocephalus as an example].

A comparative clinical and radiological follow-up study was conducted on 109 hydrocephalic children to study the criteria of shunt dependency. 50 children had a normal functioning shunt, 34 children had an ASD-shunt combination, 14 children had a non-functioning shunt without pressure symptoms and 11 children had the shunt previously removed. None of the cases showed pressure symptoms during examination. The following conclusions could be drawn: 1. The smaller the ventricular system, the higher the complication rate. 2. The standard of well-being did not correspond with the amount of the redevelopment of the compressed brain tissue. 3. A "well-drained" ventricular system was not necessarily a sign of an adequate hydrocephalic therapy, but rather a sign of a pathologic overdrainage. 4. The broader ventricular aspects of the suction-protected ASD shunt resembled the findings of the "arrested hydrocephalus" and seemed to be closer to the goal of hydrocephalus therapy, namely, shunt independency.

Adolescent↗
PubMed2002

Intracranial cerebrospinal fluid measurement studies in suspected idiopathic normal pressure hydrocephalus, secondary normal pressure hydrocephalus, and brain atrophy.

OBJECTIVE: To investigate intracranial cerebrospinal fluid (CSF) distribution in patients with a clinical diagnosis of idiopathic normal pressure hydrocephalus (INPH). METHODS: 24 patients with a clinical diagnosis of INPH were studied. Control groups comprised 17 patients with secondary normal pressure hydrocephalus (SNPH), 21 patients with brain atrophy, and 18 healthy volunteers. Ventricular volume (VV) and intracranial CSF volume (ICV) were measured using a magnetic resonance based method and the VV/ICV ratio was calculated. RESULTS: The SNPH group showed a marked increase in the VV/ICV ratio compared with the healthy volunteers (37.8% v 15.6%, p < 0.0001). The brain atrophy group showed a significant increase in ICV compared with the healthy volunteers (284.4 ml v 194.7 ml, p =0.0002). The INPH group showed an increase in ICV (281.2 ml, p = 0.0002) and an increase in the VV/ICV ratio (38.0%, p < 0.0001). Fifteen of 24 INPH patients underwent shunting; 11 improved and four did not. CONCLUSIONS: The results suggest that INPH patients have brain atrophy in addition to hydrocephalic features. This may help to explain the difficulties encountered in the diagnosis and the unpredictable response rate to shunt surgery in INPH patients.

Aged↗
PubMed1995

Morphological analysis of progressive hydrocephalus and shunt-dependent arrested hydrocephalus. An experimental study.

This experimental study was performed to determine if surgically treated feline hydrocephalus could produce any morphological and physiological changes in the periventricular tissue. The result was analyzed with clinical outcome, comparing two differently prepared models in which the biomechanical characteristics of the container property of the brain were altered. Craniectomies were performed in adult mongrel cats and the dura mater was left untouched in group A, while the dura was incised in crucial fashion in group B. Thirty-four animals underwent ventriculopleural shunt surgery 6-8 weeks after kaolin induction into the cisterna magna. In 19 animals, no shunt was implanted; they served as sham controls. These and 10 normal animals were subjected to transmission electron microscopic study or measurement of white matter water content. The regions studied were divided into three sections according to their depth from the ventricular surface (W1, W2 and W3). Considering the efficacy of shunting, the increased water content observed in preshunt animals decreased and was almost identical to normal controls after effective diversion surgery. On the other hand, the animals with ineffective shunt failed to normalize the water content and the figures were similar to the time-matched sham animals. These trends were preserved in groups A and B, but water content was much higher in group B. On histological observation, the chronological profile of subependymal extracellular space (W1, W2) in chronic condition did not correspond to the chronological changes in water content, while both changes observed after successful shunt were apparently linked. Subependymal glial proliferation was increased in volume as a function of time in all animals observed, but this was much more marked in group A than group B. Furthermore, gliosis was more evident in shunted animals than in the other group and was more prominent in cats with effective shunts than in those with ineffective shunts. These histological changes and clinical outcome were not closely related in this study. These results indicated that: (1) The shunting procedure itself could promote subependymal gliosis and this progresses unexpectedly even if CSF pressure is low enough after effective shunting. (2) This histological change is not necessarily a sufficient explanation for clinical improvement after successful shunting. (3) A biomechanical characteristic of the differently treated container property of the brain exerts an influence on the histological change and change in CSF dynamics in periependymal tissue mostly at an early stage of hydrocephalus rather than at a later stage. Therefore early treatment should be considered while avoiding an overindication for shunting.

Animals↗
PubMed2000

Indications for shunting in patients with idiopathic normal pressure hydrocephalus presenting with dementia and brain atrophy (atypical idiopathic normal pressure hydrocephalus).

The indications for shunt operation in patients with idiopathic normal pressure hydrocephalus accompanied by brain atrophy (atypical idiopathic normal pressure hydrocephalus: AINPH) were investigated in 25 patients who satisfied the diagnostic criteria and underwent ventriculoperitoneal (VP) shunting. All patients had no apparent history of intra- or extracranial disease; dementia and gait disturbance as the main complaints; moderate to severe cerebral atrophy and ventricular dilatation and at least periventricular low density around the anterior horn on computed tomography; normal cerebrospinal fluid (CSF) pressure and filling of ventricles or cortical surface space with contrast medium at 24 hours on cisternography. The 15 male and 10 female patients were aged 47-83 years (mean 60.4 years). VP shunting was effective in 12 improved patients and not effective in 13 unimproved patients according to NPH grading. Pathological pressure wave on epidural pressure monitoring was observed in eight of 12 improved patients, but none of 13 unimproved patients. CSF outflow resistance was 35.33 +/- 11.16 mmHg/ml/min in improved patients and 9.12 +/- 3.51 mmHg/ml/min in unimproved patients. Preoperative serum alpha-1-antichymotrypsin value (alpha-1-ACT) was 42.02 +/- 8.64 mg/dl in improved patients and 61.72 +/- 11.03 mg/dl in unimproved patients. Alpha-1-ACT over 55 mg/dl occurred only in unimproved patients. Cerebral arteriovenous difference of oxygen content value (c-AVDO2) before and after surgery was 6.34 +/- 0.9 ml% and 5.91 +/- 0.78 ml% in improved patients and 4.75 +/- 1.85 ml% and 4.81 +/- 1.73 ml% in unimproved patients, respectively. The two cases with preoperative c-AVDO2 value over 8.5 ml% were both unimproved. Mean cerebral blood flow value before and after surgery was 23.51 +/- 4.20 ml/100 g/min and 45.22 +/- 8.11 ml/100 g/min in improved patients and 21.77 +/- 5.12 ml/100 g/min and 24.82 +/- 4.97 ml/100 g/min in unimproved patients, respectively. Cerebral atrophy in improved patients is caused by a cerebral circulation disturbance defined as a cerebral blood flow of penumbra or more due to cerebral arteriosclerosis, etc. A flow-chart of indications of shunt surgery for AINPH was prepared based on the results of the present study.

Aged↗