Biomedical subjects
J B Krijgsman
Publications and source records attributed to J B Krijgsman.
[Ventriculomegaly and its relation to skull circumference].
Since the possibility of brain visualisation with ultrasound ventriculomegaly can be found frequently in an early stage. Ventriculomegaly caused either by an increased intraventricular pressure or an atrophic process of cerebral tissue around the ventricles. An increase in headcircumference together with an increase in ventricular size will not give problems with the diagnosis increased intraventricular pressure. However, competition between brain tissue compliance and resistance of the skull will determine which will be the first one to increase. In this the age of the patient will play an important role. The histories of three patients demonstrate these problems. The follow up of headcircumference, ventricular diameter and knowledge of the intracranial pathology, given by transfontanellar sonography in infants will determine therapeutic management excellently, often because of absence of clinical signs. Finally, the effect of treatment given can be followed closely by these measurements in an easier way and more friendly to the patient than by CT-scans.
[The too rapidly growing head].
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[Ultrasonic determination of the normal values of the diameter of the lateral ventricles in newborn infants].
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Ultrasound detection of congenital arteriovenous aneurysm of the great cerebral vein of Galen.
Using combined echoencephalography and Doppler flow determination the diagnosis Arteriovenous aneurysm of the great cerebral vein of Galen could be made in two infants. Without vascular surgery one patient died, the other recovered completely. CT scanning confirmed the diagnosis. Invasive methods such as cerebral angiography were avoided. Case histories, neuropathological findings, ultrasound method and results are presented. Pathogenesis, clinical signs, treatment and prognosis are discussed. With the ultrasound method presented, the nature and location of the vascular anomaly were demonstrable, thus additional higher risk diagnostic methods could be avoided or planned more purposefully.
Sural nerve biopsy in the diagnosis of progressive cerebral degenerative disorders of childhood. A retrospective study.
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Congenital muscular dystrophy and cerebral dysgenesis in a Dutch family.
A Dutch sibship is described consisting of a girl and a boy affected by the same disease. Both suffered from hydrocephalus and severe generalized weakness with death at 2 days and 4 months respectively. Full autopsy was done on the boy and this revealed a lissencephalic, partly polymicrogyric, neocortex, a bridge of grey matter linking the cerebral hemispheres before and over the lateral ventricles, neocortical dysplasia with subcortical neuronal heterotopic masses, generalized white matter gliosis, also involving the long fibre tracts and generalized vascular proliferation. The cerebellum showed generalized polymicrogyria. Also true hydrocephalus was found presumably related to a malformed aqueduct. Muscle biopsy revealed severe changes, consistent with congenital muscular dystrophy. Representative sections from the girls autopsy revealed an identical pattern of abnormalities. The described pattern fits descriptions of Fukuyama's cerebromuscular dystrophy.
Off-response: its clinical incidence in very young children.
Off-responses to repetitive photic stimulation may be of various shapes. In particular, very young children with respiratory distress showed a typical off-response. This reaction is rather peculiar; it consists of a triphasic, very sharp wave followed by a depolarization of longer duration. The latency is about 200 msec. This off-response seems to be the reaction of an immature brain.
Echo-encephalographic measurements (A-scan) of the lateral ventricles in children; its reliability.
A positioning of the probe on the skull has been developed to increase the diagnostic accuracy of hydrocephalus. Echo-encephalography in the trigonum region of the ventricles is usually possible in children up to 13 years of age. In this region sources of error as the medial wall of the temporal lobe or the lateral wall of the temporal horn are eliminated. In hydrocephalic ventricles the ultrasound measurements in this region corroborate very well with the measurements found in the pneumo-encephalograms. This in contrast to the measurements found with the probe placed in the more conventional region. As the largest diameter of the skull, corresponds with the trigonum region, a very practical topographic mark for a reliable standardized method has been found. With more confidence the ventricle indices of 93 normal children, in the age of 1 to 13 years, were measured.
Infantile globoid cell leucodystrophy (Krabbe's disease). Some remarks on clinical, biochemical and sural nerve biopsy findings.
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[An 18-day-old infant with a syndrome of post-infectious polyradiculo-neuropathy with cyto-albumin dissociation].
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