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Biomedical subjects

R H Gooskens

Publications and source records attributed to R H Gooskens.

At least 19 recordsLinked to original sources
PubMed2003

Audit of prenatal and postnatal diagnosis of isolated open spina bifida in three university hospitals in The Netherlands.

OBJECTIVE: To audit the current Dutch policy of prenatal detection of isolated open spina bifida based on offering detailed ultrasound examination only on indication. METHODS: A retrospective analysis of prenatally diagnosed isolated spina bifida cases and of newborns diagnosed with this condition was carried out in three university hospitals. The data were collected from databases and clinical records of the departments of prenatal diagnosis, obstetrics, neonatology, child neurology and neurosurgery of the three centers. RESULTS: Between January 1996 and December 1999, 88 cases of isolated open spina bifida were diagnosed prenatally by ultrasound investigation. Thirty-eight cases (43%) were diagnosed before the 24th week of gestation. Of these, 35 (92%) ended in termination of the pregnancy at the parents' request. Of the remaining 50 cases (57%) diagnosed after the 24th week of gestation, eight (16%) pregnancies were terminated beyond the legal limit for termination due to the severity of the condition. Of the 88 cases of isolated spina bifida, 25 infants (28%) were still alive at the age of 4 years. In the same audit period 112 newborn infants with isolated open spina bifida were admitted to the neonatology, child neurology, or neurosurgery ward of the three centers. Of these cases, 47 (42%) had been diagnosed prenatally and 65 (58%) were an unexpected finding at birth. In 24 infants (21%) surgical treatment was withheld because of the severity of the condition and predicted poor outcome, whereas the remaining 88 infants (79%) underwent surgical repair. CONCLUSION: The current practice in The Netherlands of offering ultrasound screening to high-risk patients only leads to the early detection of a minority of cases of spina bifida. Most cases are diagnosed either after the 24th week of gestation or they remain undiagnosed until after birth. When spina bifida is diagnosed before the 24th week of gestation the vast majority of parents opt for termination. In order to reduce the birth prevalence of spina bifida in The Netherlands the introduction of a policy of routine ultrasound screening should be considered.

Child, Preschool↗
PubMed2001

[Aneurysm of the V. magna cerebri (vein of Galen), a cause of congestive heart failure in five neonates].

In five neonates (4 girls and 1 boy) with aneurysm of the V. magna cerebri, clinical signs of congestive heart failure were encountered. In three of the neonates the symptoms were detected postnatally and in two prenatally. In one of the prenatally detected cases the aneurysm was treated by embolisation during the first week of life. Upon follow-up at six years of age, this patient's situation was stable but hydrocephalus had led to poor visuomotor development, mild motor retardation and mental retardation. In the other infants, medicinal treatment of the congestive heart failure was discontinued in view of ischaemic brain damage, and these patients subsequently died. Congestive heart failure often develops in neonates with an aneurysm of the V. magna cerebri due to low cerebrovascular resistance. A continuous murmur over the skull is pathognomonic for this condition.

Cerebral Veins↗
PubMed2001

[Diagnostic value of prenatal MRI in fetus with intracranial anomalies diagnosed by ultrasonography].

OBJECTIVE: To determine the value of prenatal MRI in the third trimester in the foetus with intracranial lesions established by ultrasonography. DESIGN: Descriptive. METHOD: During a period of one year, prenatal MRI was performed in 12 patients after ultrasonography had revealed intracranial lesions in the foetus. Postnatally, ultrasonography, MRI or postmortem examination of the brain was performed in 11 children. The MRI examination was carried out in a 1.5 Tesla field. Rapid, T2-weighted images were obtained. To immobilize the child, the pregnant women were sedated. RESULTS: In 5 cases, prenatal ultrasonography and prenatal MRI resulted in the same diagnosis. In seven cases, MRI provided more information. Postnatal examination confirmed the prenatal diagnosis in 10 of the 11 cases. In one case, maceration rendered supplementary examination impossible. CONCLUSION: MRI may be regarded as a valuable alternative diagnostic aid if prenatal ultrasonography is incomplete or the findings are uncertain or limited.

Brain↗
PubMed2001

Medullary cone movement in subjects with a normal spinal cord and in patients with a tethered spinal cord.

PURPOSE: To compare movement of the normal medullary cone when the patient has changed from a supine to prone position with that in patients with known or suspected tethered spinal cord syndrome. MATERIALS AND METHODS: Fifty-six individuals divided into three groups were examined with lumbar spine magnetic resonance (MR) imaging performed with the patient in the prone and supine positions. Group 1 consisted of 15 healthy volunteers and six patients with a herniated disk; group 2, 25 patients clinically suspected of having a tethered cord; and group 3, 10 patients who previously had undergone tethered cord surgery. RESULTS: All group 1 subjects showed distinct and statistically significant medullary cone movement (range, 21%--41%); no patient in group 3 showed movement (Wilcoxon rank sum test, P <.001). In group 2, the 20 patients in whom a definite diagnosis of tethered cord syndrome was made on the basis of initial supine MR image findings showed no movement, whereas two of five patients with normal supine MR images had abnormal and decreased cone movement at prone imaging. CONCLUSION: Prone MR imaging has no additional value when the supine MR image has clearly shown the cause of tethering or in patients who have undergone tethered cord surgery, but it can provide additional information in patients clinically suspected of having a tethered cord and in whom supine MR imaging depicted no abnormalities.

Adolescent↗
PubMed2000

[Children with craniopharyngioma, a 'benign' brain tumor with a high morbidity].

The craniopharyngioma is a histologically benign tumour, mainly occurring in childhood. Neurosurgical treatment, in some of these patients in combination with external radiotherapy, results in a fair prognosis for children with craniopharyngioma. However, the central localisation of this tumour in the brain, damage of surrounding structures due to tumour growth, as well as the adverse effects of treatment will result in substantial morbidity in many patients. The first patient, a 13-year-old boy, presented with symptoms and signs of increased intracranial pressure. Following operation he was treated with radiotherapy, and up to 4 years after the operation there was no tumour recurrence. However, he experienced endocrine, visual, hypothalamic and intellectual disturbances due to the disease and its treatment. The second patient, a 6-year-old girl, presented with hydrocephalus due to the tumour, but also had growth retardation. After operation and radiotherapy, she had visual, endocrine, and intellectual dysfunction, but there were no signs of tumor recurrence. The third patient, an 12-year-old boy, already had growth retardation several years before he presented with neurological dysfunction. He received no postoperative radiation, as all tumour tissue could be removed. He had visual, hypothalamic, endocrine and intellectual disturbances but until nearly 6 years after the operation, there was no tumour recurrence. The morbidity due to the tumour and its treatment has a negative impact on the patient's quality of life. A multidisciplinary treatment and follow-up of these children cannot avoid this morbidity but may result in a timely observation of problems and thereby prevent unnecessary damage.

Adolescent↗
PubMed2000

[Acute encephalopathy in children: a serious illness after an inauspicious beginning].

Three young children, 2 girls of 6 and 3.5 years and 1 boy aged 3.5 years, had complaints like vomiting and fever of what initially was thought to be a minor infection. However, their condition deteriorated within two days to such an extent that they died while being treated on the intensive care department. The rapid drop in consciousness, sometimes accompanied by convulsions, is an expression of increased intracranial pressure resulting from diffuse brain oedema, which itself is probably caused by circulating toxins. The treatment of acute toxic encephalopathy is purely symptomatic.

Adenoviruses, Human↗
PubMed2000

NMR spectroscopic evaluation of cerebral metabolism in hydrocephalus: a review.

Cerebral ischemia contributes to cerebral damage in hydrocephalus. Many studies have reported changes in cerebral blood flow and metabolism, supporting this hypothesis. Magnetic resonance spectroscopy (MRS) enables us to investigate cerebral metabolism in a non-invasive and longitudinal manner, thereby providing a promising way of evaluating pathophysiological changes in experimental and clinical hydrocephalus. In this review, the potential of 1H (proton) and 31P (phosphorus) MRS in the assessment of cerebral metabolism will be summarized, and a synopsis of in vitro and in vivo MRS studies in experimental and human hydrocephalus will be presented. Changes in high-energy phosphate metabolism, intracellular pH and lactate production in several MRS studies are presumed to reflect cerebral ischemia. In vivo information on neuronal damage, maturational delay and membrane phospholipid metabolism may also be derived from 1H and 31P MRS data. Technical, methodological and pathophysiological considerations, which are important for a correct interpretation and comparison of different MRS studies, will be discussed. Finally, we will draw some conclusions on the significance of these MRS findings and the applicability of MRS in the diagnosis and evaluation of clinical hydrocephalus.

Animals↗
PubMed1999

Cerebral metabolism in experimental hydrocephalus: an in vivo 1H and 31P magnetic resonance spectroscopy study.

OBJECT: Brain damage in patients with hydrocephalus is caused by mechanical forces and cerebral ischemia. The severity and localization of impaired cerebral blood flow and metabolism are still largely unknown. Magnetic resonance (MR) spectroscopy offers the opportunity to investigate cerebral energy metabolism and neuronal damage noninvasively and longitudinally. Previous 1H MR spectroscopy studies have shown an increased lactate resonance that is suggestive of anaerobic glycolysis. The aim of this study was to assess cerebral damage and energy metabolism in kaolin-induced hydrocephalus in adult rats by using in vivo 1H and 31P MR spectroscopy. The presence of lactate was correlated with high-energy phosphate metabolism and intracellular pH. The measurement of relative concentrations of N-acetyl aspartate (NAA), choline (Cho), and total creatine (tCr) served to assess neuronal damage. METHODS: Hydrocephalus was induced in adult rats by surgical injection of kaolin into the cisterna magna. Magnetic resonance studies, using a 4.7-tesla magnet, were performed longitudinally in hydrocephalic animals at 1 (10 rats), 8 (six rats), and 16 weeks (six rats) thereafter, as well as in eight control animals. To evaluate ventricular size and white matter edema T2-weighted MR imaging was performed. The 1H MR spectra were acquired from a 240-microl voxel, positioned centrally in the brain, followed by localized 31P MR spectroscopy on a two-dimensional column that contained the entire brain but virtually no extracranial muscles. The 1H and 31P MR spectroscopy peak ratios were calculated after fitting the spectra in the time domain, intracellular pH was estimated from the inorganic phosphate (Pi) chemical shift, and T2 relaxation times of 1H metabolites were determined from the signal decay at increasing echo times. CONCLUSIONS: In hydrocephalic rats, ventricular expansion stabilized after 8 weeks. White matter edema was most pronounced during acute hydrocephalus. Lactate peaks were increased at all time points, without a decrease in phosphocreatine (PCr)/Pi and PCr/adenosine triphosphate (ATP) peak ratios, or pH. Possibly lactate production is restricted to periventricular brain tissue, followed by its accumulation in cerebrospinal fluid, which is supported by the long lactate T2 relaxation time. Alternatively, lactate production may precede impairment of ATP homeostasis. The NAA/Cho and tCr/Cho ratios significantly decreased during the acute and chronic stages of hydrocephalus. These changes were not caused by alterations in metabolite T2 relaxation time. The decreases in the NAA/Cho and tCr/Cho ratios implicate neuronal loss/dysfunction or changes in membrane phospholipid metabolism, as in myelin damage or gliosis. It is suggested that 1H MR spectroscopy can be of additional value in the assessment of energy metabolism and cerebral damage in clinical hydrocephalus.

Animals↗
PubMed1998

In vivo 1H MR spectroscopic imaging and diffusion weighted MRI in experimental hydrocephalus.

The severity and progression of ventricular enlargement, the occurrence of cerebral edema, and the localization of ischemic metabolic changes were investigated in a rat model of hydrocephalus, using in vivo 1H MR spectroscopic imaging (SI) and diffusion weighted MRI (DW MRI). Hydrocephalic rats were studied 1, 2, 4, and 8 weeks after injection of kaolin into the cisterna magna. Parametric images of the apparent diffusion coefficient (ADC) revealed a varying degree of ventriculomegaly in all rats, with different time courses of ventricular expansion. Extracellular white matter edema was observed during the early stages of hydrocephalus, most extensively in cases of progressive ventriculomegaly. In gray matter regions, ADC values were not changed, compared with controls. In case of fatal hydrocephalus, high lactate levels were observed throughout the whole brain. In all other rats, at all time points after kaolin injection, lactate was detected only in voxels containing cerebrospinal fluid. This suggests accumulation of lactate in the ventricles, and/or an ongoing periventricular production of lactate as a consequence of cerebral ischemia in experimental hydrocephalus.

Acute Disease↗
PubMed1998

Does CSF outflow resistance predict the response to shunting in patients with normal pressure hydrocephalus?

The value of the measurements of CSF outflow resistance (Rcsf) relative to predicting outcome after shunting was studied. In a group of 101 patients with mainly idiopathic normal pressure hydrocephalus (NPH) Rcsf was obtained by lumbar constant flow infusion. Gait disturbance and dementia were quantified using an NPH scale (NPHS) and disability by the Modified Rankin scale (MRS). Patients were assessed before and at 1, 3, 6, 9 and 12 months after surgery. Outcome measures were differences between the preoperative and last NPHS and MRS scores. Improvement was defined as a change of > or = 15% in NPHS and > or = 1 grade in MRS. Intention-to-treat analysis of all patients at one year yielded improvement of 57% in NPHS and 59% in MRS. Efficacy analysis, excluding comorbidity unrelated to NPH, revealed positive predictive values of around 80% at Rcsf < 18, and between 90% and 100% at Rcsf > or = 18 mm Hg/ml/min. For Rcsf > or = 18, the likelihood ratios were also higher. We conclude that the best predictor of the response to shunting is an Rcsf > or = 18 mm Hg/ml/min. Since two-thirds of the patients with Rcsf < 18 showed improvement as well, these patients should not be denied shunting.

Cerebrospinal Fluid Pressure↗
PubMed1998

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↗
PubMed1997

Cerebral ischemia and white matter edema in experimental hydrocephalus: a combined in vivo MRI and MRS study.

T2 and diffusion weighted MRI, as well as 31P and 1H MRS were performed in kaolin-induced hydrocephalic rats. Extracellular white matter edema was detected in the early stages of progressive hydrocephalus. Phosphocreatine (PCr)/inorganic phosphate (Pi) ratios in hydrocephalic animals were decreased compared to controls, and lactate was detected during the acute and chronic stages of hydrocephalus. These MR spectroscopic results are indicative of a compromised energy metabolism and suggest the occurrence of cerebral ischemia in experimental hydrocephalus.

Animals↗
PubMed1997

Dutch normal-pressure hydrocephalus study: prediction of outcome after shunting by resistance to outflow of cerebrospinal fluid.

The authors examined whether measurement of resistance to outflow of cerebrospinal fluid (Rcsf) predicts outcome after shunting for patients with normal-pressure hydrocephalus (NPH). In four centers 101 patients (most of whom had idiopathic NPH) who fulfilled strict entry criteria underwent shunt placement irrespective of their level of Rcsf obtained by lumbar constant flow infusion. Gait disturbance and dementia were quantified by using an NPH scale and the patient's level of disability was assessed by using the modified Rankin scale (mRS). In addition the Modified Mini-Mental State Examination was performed. Patients were assessed prior to and 1, 3, 6, 9, and 12 months after surgery. Primary outcome measures were based on differences between the preoperative and last NPH scale scores and mRS grades. Improvement was defined as a change measuring at least 15% in the NPH scale score and at least one mRS grade. Intention-to-treat analysis of all patients at 1 year yielded improvement for 57% in NPH scale score and 59% in mRS grade. Efficacy analysis, excluding serious events and deaths that were unrelated to NPH, was performed for 95 patients. Improvement rose to 76% in NPH scale score and 69% in mRS grade. Six cut-off levels of Rcsf were related to improvement in NPH scale score using two-by-two tables. Positive predictive values were approximately 80% for an Rcsf of 10, 12, or 15 mm Hg/ml/minute, 92% for an Rcsf of 18 mm Hg/ml/minute, and 100% for an Rcsf of 24 mm Hg/ml/minute. Negative predictive values were low. More important was the highest likelihood ratio of 3.5 for an Rcsf of 18 mm Hg/ml/minute. Extensive comorbidity was a major prognostic factor. Measurement of Rcsf reliably predicts outcome if the limit for shunting is raised to 18 mm Hg/ml/minute. At lower Rcsf values the decision depends mainly on the extent to which clinical and computerized tomography findings are typical of NPH.

Aged↗
PubMed1996

Relationship between anterior fontanelle pressure measurements and clinical signs in infantile hydrocephalus.

The treatment of choice in progressive hydrocephalus is drainage of cerebrospinal fluid in order to reduce elevated intracranial pressure (ICP). Defining the right moment for surgical intervention, however, in a hydrocephalic infant on the basis of clinical signs alone can be a difficult task. Clinical signs of raised ICP are known to be unreliable and sometimes even misleading. In the present study, the relationship between long-term anterior fontanelle pressure (AFP) measurements and clinical signs was investigated in 37 infants with hydrocephalus. The decision as to whether to operate or not was based on clinical signs alone; AFP values were not taken into account. There was an overall difference between the non-operated group and the preoperative measurements in the operated group, and also between the preoperative and the postoperative measurements in the latter, in regard to both AFP measurements and clinical signs. Almost all preoperative AFP values were increased. The direct correlation (phi) between most individual clinical signs and AFP levels, however, was low (phi = 0.15-0.41). The clinical sign "tense fontanelle" showed the best correlation with the AFP levels (phi = 0.75). Furthermore, using logistic regression analysis, no combination of clinical signs could be found which reliably predicted the AFP. The relationship between the AFP pressure variables and clinical signs was also examined. The pathological A-waves occurred only in the presence of raised (baseline) AFP, a situation in which considerably more frequent B-waves were observed as well. It was concluded that clinical signs of raised ICP in infantile hydrocephalus are not very reliable and AFP monitoring can therefore provide valuable information on intracranial dynamics in patients with dubious neurological manifestations of progressive hydrocephalus.

Female↗
PubMed1995

Non-invasive ICP monitoring in infants: the Rotterdam Teletransducer revisited.

Measurement of intracranial pressure (ICP) is important in patients at risk of raised ICP, as in hydrocephalus. Ideally, it should be non-invasive, thus avoiding the risk of infection and other complications: Such is provided by measurement of ICP through the anterior fontanelle. There are several methods of measuring anterior fontanelle pressure (AFP); those most frequently used are based on the applanation principle. An evaluation of AFP measurement devices resulted in the choice of the Rotterdam Teletransducer (RTT) to be used in our study of children with hydrocephalus. The literature contains little information on the accuracy or validation of the AFP measurements using the RTT. Therefore, the physical qualities of the RTT were reassessed, using a specially developed calibration device. The results of this study demonstrate that membrane temperature does not have any effect on the measured pressure. The thermal stabilization time of the RTT was found to be 3 h after switching on. Insufficient thermal stabilization results in a pressure underestimation of up to 3 mmHg. Furthermore, a maximum inaccuracy of 2.6 mmHg, after calibration and readjustment of the transducer, was calculated. Validation of the equipment was achieved by simultaneous AFP/ICP measurements in hydrocephalic patients showing high correlations (r = 0.96-0.98). The discussion suggests a measurement protocol as a means of increasing the reliability of RTT measurements.

Calibration↗
PubMed1995

Value of transcranial Doppler indices in predicting raised ICP in infantile hydrocephalus. A study with review of the literature.

Cerebral hemodynamic changes in infants with progressive hydrocephalus have been studied with the transcranial Doppler (TCD) technique. Several authors have referred to the correlation between the hemodynamic changes and increased intracranial pressure (ICP). Despite conflicting conclusions on the value of pulsatility index (PI) and resistance index (RI) measurements for monitoring infantile hydrocephalus, these pulsatility indices are the most commonly used for this purpose. Although clinical signs of raised ICP are highly variable and unreliable in infants, assumptions have been made in most of the studies about the presence of elevated ICP on the basis of the patient's clinical state. Few studies have reported on actual ICP values, however, and a direct relationship between ICP and TCD changes has never been adequately demonstrated. In the present study, this relationship was investigated in long-term simultaneous TCD/ICP measurements, in an attempt to develop a noninvasive method of monitoring the effect of ICP on intracranial hemodynamics. Two groups of data sets were established. Group I consisted of pre- and postoperative (shunt implantation) TCD/ICP measurements. Group II were long-term simultaneous TCD/ICP recordings showing significant ICP variations. In most of the postoperative measurements there was a decrease in the average PI and RI values. The correlation between PI or RI and ICP in the long-term simultaneous recordings, however, was generally poor. The risk of obtaining false positive or false negative PI or RI values in short-term measurements was also demonstrated. It can be concluded from our results, besides the wide range of reference values for the Doppler indices and extracranial influences upon them, that the present Doppler indices are inadequate for monitoring the complex intracranial dynamic responses in patients with raised ICP.

Adolescent↗