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Biomedical subjects

J Willemse

Publications and source records attributed to J Willemse.

At least 37 records · Page 2Linked to original sources
PubMed1989

Ischaemic stroke in the basal ganglia and internal capsule in childhood.

Ischaemic stroke in childhood is rare. In particular, there have only been a few reports of lacunar infarcts in children. In this study, four children with true lacunar infarcts and four with larger subcortical infarcts are described. We compare the clinical features and the possible pathogenesis of ischaemic strokes in the basal ganglia and internal capsule in childhood with those in adults, and discuss diagnostic and therapeutic management.

Basal Ganglia↗
PubMed1989

Macrocephalies--a differentiated approach.

We present a clinical study of 43 macrocephalic children with a normal rate of headgrowth. The aims of the study were an evaluation of the usual criteria of macrocephaly and the drafting of a differentiated approach to the investigation of macrocephalics. Firstly, the value of head measurement in evaluating intracranial volume was assessed. As a golden standard for the intracranial volume the volume obtained by means of a CT-scan technique was used. It appeared that only about 30% of the variance of the intracranial volume was determined by the occipito-frontal circumference (OFC). A better estimation of the intracranial volume of the macrocephalic children was obtained by means of the "Utrecht Head Measure", the product of OFC-squared and the head height. Based on length measurements taken from CT-scans, the macrocephalic children were divided into three subgroups: megalencephaly (n = 15), extraventricular obstructive hydrocephalus (n = 5) and communicating hydrocephalus (n = 23). The clinical pictures of the megalencephalic and hydrocephalic children appeared to differ substantially. The megalencephalic subgroup had less neurological symptoms and physical abnormalities and showed a higher intelligence or a more advanced development or both. On the basis of our experience and theoretical considerations guidelines were developed for the assessment of patients with macrocephaly and a normal rate of head growth with a differentiated approach for megalencephalic and hydrocephalic patients.

Brain↗
PubMed1988

The diagnostic management of newborns with congenital contractures: a nosologic study of 75 cases.

A prospective clinical study is presented of 75 patients with multiple congenital contractures. With the data from medical history, child neurologic examinations, laboratory tests including chromosome and dermatoglyphic analysis, and neuropathology in 23 cases with perinatal death, a nosological or syndromal diagnosis was made in 61 cases. These cases were classified by localization of causal pathology in the categories "cerebral dysgenesis", spinal cord defects, neuromuscular disorders and miscellaneous disorders without muscle weakness. Following this concept, the various modes of inheritance of specific disorders presenting with congenital contractures, as well as possibilities for prenatal diagnosis by ultrasonography are discussed. A guideline for the child neurologic evaluation of infants with congenital contractures is proposed. It is concluded that: 1) specification of the causal lesion and proper classification of disorders with congenital contractures is crucial for genetic counseling, (prenatal) diagnosis and management; 2) the analysis of dermatoglyphics in differential diagnosis of congenital contractures should be restudied; and 3) more study and experience is required in the observation of abnormal or decreased fetal movements by ultrasonography.

Brain↗
PubMed1988

The value of estimating pressure-volume index in childhood macrocephaly. The relationship between pressure-volume index and the volumes of intracranial structures.

In this study on cerebrospinal fluid (CSF) dynamics in childhood macrocephaly, we investigated the relationship between the pressure volume index (PVI) and the volume of various brain structures. The most important variables of CSF dynamics are the outflow resistance (Ro) and the PVI. We applied a constant CSF infusion rate to measure the PVI. The volumes of intracranial structures were calculated by means of computed tomography. Our study revealed no significant correlation between PVI and CSF volume or between PVI and the intracranial volume, as suggested by earlier studies. We did, however, find a significant correlation between PVI and ventricle volume. These results indicate that in order to estimate PVI for clinical purposes, the ventricle volume rather than CSF volume or head circumference should be used.

Brain↗
PubMed1988

Megalencephaly: definition and classification.

The various definitions and classifications of megalencephaly are reviewed, and numerous diseases and syndromes associated with megalencephaly are listed. A new definition of megalencephaly based on quantitative radiographic features is proposed. We define megalencephaly as a brain volume which exceeds the mean by more than twice the standard deviation. Furthermore, a modified etiopathogenic classification of megalencephaly results in three main groups, viz anatomic, metabolic and dynamic megalencephaly. The clinical pictures in these main groups of megalencephaly, and the largest subgroup of anatomic megalencephaly, familial anatomic megalencephaly, appear to be quite different.

Brain↗
PubMed1988

Neuro-imaging of cerebral visual disturbances in children.

The present study comprises the neuroradiological examination (computertomography++, magnetic resonance imaging) of 26 children--9 of them were premature, 16 were full-term and in one patient, no details of the pregnancy were known--all suffering from: a) visual disturbance, not caused by ocular disease or afflictions of the optic nerve b) infantile encephalopathy The examination revealed characteristic lesions of the retrochiasmatic part of the visual system. These consisted of white matter abnormalities located periventricularly and just below the striate area, and of grey matter abnormalities of the striate cortex. The demonstration of these abnormalities may advance the diagnosis of the visual handicap and may facilitate early adjustment of developmental stimulation.

Adolescent↗
PubMed1988

Intracranial spaces in childhood macrocephaly: comparison of length measurements and volume calculations.

CT scanning was done to calculate the volume of intracranial spaces in children with the purpose of developing a reliable method of differentiating the various causes of macrocephaly. The technique has been applied to the CT scans of 60 apparently normal children, and the resulting graphs show the normal relationship between intracranial volumes and age from birth to 15 years. The measurements for 25 children with macrocephaly and normal rate of growth of head circumference were then compared with the reference values. It was possible to make accurate differentiations between megalencephaly, extraventricular hydrocephalus and communicating hydrocephalus. The advantage of this technique in relation to length measurements on CT scans is discussed. The authors advocate the estimation of the product of head circumference and head height as a much more reliable indication of normal and deviant head-size than head circumference alone.

Brain↗
PubMed1988

Treatment of intact familial intracranial aneurysms: a decision-analytical approach.

Clinical decision analysis is applied to the treatment decisions for four patients with unruptured familial aneurysms. The surgical treatment was uneventful in all patients except one with mild mixed aphasia and facial weakness postoperatively; these deficits disappeared in less than 2 years. In the decision analysis, discounted Quality Adjusted Life Years are used as an outcome measure. Probability estimates are extracted from the literature when available. It is concluded that the decision to treat the aneurysm neurosurgically in three of the four patients was correct. In two of these three patients, the decision cannot be altered by plausible changes in estimated data. For the third patient, only the combination of a low probability of rupture, a high surgical mortality and morbidity, and high discount favors conservative treatment. In the fourth patient, a toss-up situation exists. More knowledge of the probability of rupture, the probability of the development of other aneurysms, and the results of operation on intact intracranial aneurysms would have made the analysis more accurate. Clinical research should address these issues.

Adult↗
PubMed1987

Detection of unruptured familial intracranial aneurysms by intravenous digital subtraction angiography. Screening of two affected families.

The authors discuss the detection of intracranial aneurysms (IA) by means of intravenous digital angiography (ivDSA) in (a)symptomatic first degree relatives of families in which two or more individuals have IA. ivDSA is an almost noninvasive and low-risk diagnostic procedure. Screening, by means of ivDSA, of two affected families is described. In family I which includes 7 members with proven IA, ivDSA has been carried out in 36 asymptomatic individuals: in one, a 6 X 15 mm aneurysm was found at the left posterior communicating artery (PCoA). In family II, including one member with a proven IA and another with a subarachnoid hemorrhage, ivDSA has been carried out in 4 members: one aneurysm with a diameter of 6 mm was found at the left PCoA. Conventional cerebral angiography (CCA) confirmed both IA's. Neurosurgical treatment followed. The advantages and disadvantages of ivDSA vs. CCA as elective screening procedure in such cases are discussed. Screening of asymptomatic first degree relatives of cases with familial IA by means of ivDSA is strongly advocated.

Adult↗
PubMed1987

The pathogenesis of fetal hypokinesia. A neurological study of 75 cases of congenital contractures with emphasis on cerebral lesions.

A comprehensive prospective clinical study is presented of 75 cases of fetal hypokinesia and congenital contractures of various causes, with neuropathological investigation in 23 cases. With the data of medical history, neurological examination, laboratory tests and neuropathology an exact or probable nosological or syndromal diagnosis could be made in 61 cases. These cases were categorized by localisation of causal pathology in the subsequent levels of the developing motor system. In 14 of 61 cases developmental brain disorders (f.i. hydrocephalus, hydranencephaly, microcephaly) were the cause of fetal hypokinesia, often with perinatal death, whereas in 7 cases both cerebral and/or spinal cord lesions were found. Besides cerebral involvement was frequently present in cases with congenital contractures of other origin, concomitant or due to perinatal complications. In a large number of cases clinical evidence of spinal cord lesions, especially anterior horn cell degeneration was present. Myopathic disorders occurred in only four cases, whereas congenital myasthenia and congenital neuropathy were present in one case each. In cases without muscle weakness miscellaneous disorders including congenital skin anomalies and probably primary connective tissue disorders were encountered. The etiologic role of intrauterine viral infection is discussed.

Adolescent↗
PubMed1987

The heterogeneity of the Pena-Shokeir syndrome.

There is evidence that the Pena-Shokeir syndrome is not a specific phenotype but should be regarded as a "fetal akinesia deformation sequence". A neuropathological study of six random new cases was performed to evaluate this theory. Brain pathology observed included persistent fetal meningeal vascularization (two cases), agenesis of the septum pellucidum (one case) and hydranencephaly (one case). Investigation of the spinal cord (in two cases) revealed no abnormalities. Muscle histology (in four cases) was indicative of neurogenic atrophy in two cases. These findings are compared with the data of the 28 cases previously described. It is concluded that the Pena-Shokeir syndrome is a heterogeneous syndrome in which cerebral lesions may play an important role in the pathogenesis. The cerebral malformations may also indicate the time of origin and contribute in the perinatal death of this syndrome.

Abnormalities, Multiple↗
PubMed1987

Familial occurrence of intracranial aneurysms in childhood: a case report and review of the literature.

This report describes three individuals belonging to one family, who were affected with ruptured intracranial aneurysms (IAs) at a rather young age, 4, 15 and 23 years respectively. Familial IAs at this young age have not been described before. The evidence for a developmental, eventually inherited origin of familial IA in childhood is discussed with reference to the low occurrence of non-familial IA in childhood, IA-features in non-familial cases (localisation, multiplicity, size) and the association with (other) congenital malformations in the 0-5 years group.

Adolescent↗
PubMed1986

Familial association of intracranial aneurysms and multiple congenital anomalies.

The familial occurrence of intracranial aneurysms and the possible relationship with connective tissue disease are discussed. We studied a large family in which seven members presented with aneurysms. Another family member presented with a subarachnoidal hemorrhage. Two other family members each presented with Marfan's syndrome and an unclassified multiple congenital anomalies syndrome, respectively. The multiplicity of the aneurysms in four members is in excess of that found in sporadic or familial cases with intracranial aneurysms. We suggest a common cause, eg, a connective tissue disorder for both the intracranial aneurysms, the Marfan's syndrome, and the unclassified syndrome.

Abnormalities, Multiple↗
PubMed1986

Benign idiopathic dystonia with onset in the first year of life.

Four infants suffering from benign non-fluctuating, idiopathic dystonia with onset in the first year are presented. The discussion includes the relationship to childhood-onset dystonia, the significance of the tremor, the neurotransmitters in the CSF, spontaneous recovery, the risk of recurrence and the preference not to intervene orthopaedically.

Age Factors↗
PubMed1985

The Pena-Shokeir syndrome: report of nine Dutch cases.

We report on nine individuals with the Pena-Shokeir syndrome. Clinical findings are compared with data on patients from the literature. Emphasis is made on genetic background, neuropathological findings, and (in two cases) on prenatal data. Possible pathogenetic mechanisms are discussed.

Abnormalities, Multiple↗
PubMed1985

Conservative and surgical management of focal cerebral infection.

After the introduction of CT-scanning the management of focal cerebral infections has been modified. Based on the data of two patients and on the literature the authors discuss the choice between fully conservative, immediately neurosurgical or delayed neurosurgical treatment. It is the author's opinion that by closely following the infectious process too early neurosurgical intervention can be avoided and thus unnecessary sacrifice of viable neural tissue.

Adolescent↗
PubMed1985

A cerebral cause of arthrogryposis: unilateral cerebral hypoplasia.

The authors describe a case of predominantly right-sided arthrogryposis in an infant with contralateral congenital cerebral hypoplasia and associated unilateral hydrocephalus. Diagnosis was made by prenatal ultrasonography and confirmed by postnatal cerebral CT-scanning. In addition to a variety of congenital neuromuscular disorders, arthrogryposis may be caused by disorders of the central nervous system. Hence, in the diagnostic approach of newborns with congenital contractures, cerebral malformations must be considered.

Abnormalities, Multiple↗