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Biomedical subjects

G J Vielvoye

Publications and source records attributed to G J Vielvoye.

At least 19 recordsLinked to original sources
PubMed1999

Bilateral sensorineural deafness, partial agenesis of the corpus callosum, and arachnoid cysts in two sisters.

We describe two sisters (ages 10 and 3 years, respectively) with a normal development and a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, arachnoid cyst, and hydrocephalus. Neither girl has distinctive physical anomalies. In the oldest girl, there was a hearing loss of 80 dB bilaterally, and the most severe loss on audiogram was seen at 2,000-4,000 Hz. In the youngest girl, there was a hearing loss of 100 dB bilaterally. Above 2,000 Hz no neural reactions were seen. Cerebral magnetic resonance imaging in one girl and computed tomography in the other showed a partial agenesis of the corpus callosum and a cyst in the pineal region, causing an aqueduct stenosis by compression and consequent hydrocephalus. The parents have normal hearing, and brain magnetic resonance imaging showed no abnormalities. They are nonconsanguineous but from the same small village. This is the first report of a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, and an arachnoid cyst. The pattern of inheritance is probably autosomal recessive.

Adult↗
PubMed1996

Optic nerve glioma mimicking an optic nerve meningioma.

The case is described of an optic nerve glioma, mimicking an optic nerve meningioma in a man aged 41 years. CT, MRI and DSA revealed an enhancing tumor surrounding the optic nerve. Histopathologic examination of the removed optic nerve revealed a centrally located glioma surrounded by normal optic nerve fibres.

Adult↗
PubMed1995

Percutaneous computed-tomography-guided thermocoagulation for osteoid osteomas.

Current treatment for osteoid osteomas is usually surgical excision of the nidus. We treated 18 patients with osteoid osteoma by percutaneous thermocoagulation of the nidus under computed-tomography guidance. The procedure was technically successful in all cases and there were no complications. Patients were discharged on the following morning and resumed normal activities immediately. All patients but one remained pain free during follow-up (range 3-15 months). A second thermocoagulation treatment relieved the recurrent symptoms in this patient. Percutaneous thermocoagulation appears to be effective for osteoid osteomas, and is a minimally invasive alternative to surgical resection.

Bone Neoplasms↗
PubMed1995

Idiopathic isosexual central precocious puberty: magnetic resonance findings in 30 patients.

The purpose of this prospective study was to define the incidence of magnetic resonance imaging (MRI) abnormalities in the brain in patients with idiopathic central precocious puberty without any additional neurological signs and symptoms, and to evaluate the routine use of gadolinium contrast in these patients. 30 patients (29 girls, one boy; age range 1.9-11.9 years) with idiopathic central precocious puberty were studied. MRI of the brain in axial, coronal and sagittal planes was performed before and after administration of gadopentetate dimeglumine, with special attention to the region of the third ventricle. There are three major findings: (1) the height of the pituitary gland is increased up to adult size compared with normal individuals; (2) in four patients (13%) major structural abnormalities were found; three hamartomas of the tuber cinereum and one gliomatous process extending from the chiasm to the optic tract; and (3) the routine use of gadopentetate dimeglumine did not reveal new abnormalities although the lack of enhancement made a positive contribution to diagnostic certainty. We conclude that contrast enhanced MR examination is a safe and reliable method for the exclusion of abnormalities in children with precocious puberty and for the follow-up of those patients in whom abnormalities are present.

Adolescent↗
PubMed1993

Neuroradiological investigations in cervical root avulsion.

Cervical myelography in combination with CT myelography is not fully reliable to demonstrate a partial or complete cervical root avulsion. MRI scanning can demonstrate large traumatic meningoceles or additional lesions, such as intramedullary or extradural haematomas, but not a root avulsion. In experimental conditions MR microscopy enables visualization of the avulsed root separated from the spinal cord. The anterior funiculus shows transverse tracts left behind by the avulsion at the original site of the motor fibres. However, the small bore of the current high field magnets and the very long acquisition time makes this method, as yet, unsuitable for application in man.

Brachial Plexus↗
PubMed1992

Follow-up magnetic resonance imaging in Hallervorden-Spatz disease.

Bilateral high signal emitting areas in the globus pallidus surrounded by low signal emitting areas have been described as a typical MRI finding in Hallervorden-Spatz disease (HSD). We made a diagnosis of HSD in an 11-year-old girl with progressive dystonia of 4 years duration who showed these typical MRI abnormalities. An initial MRI at the age of 9 was normal. Pathological confirmation of these typical MRI findings has not yet been described, but earlier reports as well as our case suggest that MRI may be helpful in making a clinical diagnosis of HSD. This case further shows that MRI may be normal in an early stage of the disease.

Child↗
PubMed1992

Magnetic resonance imaging of the so-called cerebral cryptic angiomas.

Cerebral cryptic angiomas are vascular malformations with variable histological appearance, but with similar radiological features. Angiography does not usually visualize the lesion. Computer tomography often detects the malformation, but frequently fails to make classify correctly. With magnetic resonance imaging (MRI) the cryptic angioma is studied best on T2 weighted images. The pattern of signal intensities differs both among and within the lesions. Evidence of small hemorrhages of different ages and flow phenomena are seen in all malformations. A total of 51 angiomas were demonstrated in 38 patients by MRI (excluding one patient with a very large number of angiomas). Enhanced or plain CT scans depicted respectively 16 and 6 lesions. In one case angiography was the only way to detect an angioma. Currently, MRI is the most sensitive method for the detection of cerebral cryptic angiomas.

Adolescent↗
PubMed1992

Similar brain lesions in alcoholics and Korsakoff patients: MRI, psychometric and clinical findings.

MRI examination revealed similar brain lesions in 5 alcoholic Korsakoff patients and 5 chronic alcoholics without cognitive impairment. Not only cerebral atrophy and demyelination, but also lesions thought to be specific for the Wernicke-Korsakoff syndrome were equally prominent in both groups. The morphological abnormalities thought to be typical of Wernicke-Korsakoff syndrome are probably common features of chronic alcoholism and malnutrition. Marked atrophy of the operculae was found in all Korsakoff patients and in 3 out of 5 chronic alcoholics. Alcohol amnestic disorder may not exclusively result from diencephalic lesions, but also from temporal lesions.

Adult↗
PubMed1991

Lhermitte-Duclos disease and Cowden disease: a single phakomatosis.

Two unrelated patients with macrocephaly, seizures, and mild cerebellar signs had a dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). Both also had autosomal dominant Cowden disease as evidenced by facial, oral, and acral papules. In the two families, 9 sibs demonstrated the mucocutaneous lesions, thyroid disease, breast tumors, and ovarian tumors compatible with the diagnosis of Cowden disease. Some of the sibs also showed various degrees of neurological signs such as macrocephaly, mental retardation, seizures, tremor, and dysdiadochokinesia. Magnetic resonance imaging scans of sibs of one family demonstrated megalencephaly and other mild abnormalities. The occurrence of these two rare disorders in single patients is more than a coincidence, and the clinical findings in the combined condition establishes it as a new phakomatosis.

Abnormalities, Multiple↗
PubMed1991

Hereditary spastic dystonia: a new mitochondrial encephalopathy? Putaminal necrosis as a diagnostic sign.

A large kindred, in which either Leber's hereditary optic atrophy, or a hereditary spastic dystonia, or a combination of both manifested over many generations was restudied after the first report on it in 1964. NMR scans revealed bilateral, and, in two patients with hemidystonia, unilateral necrosis with shrinkage of the putamen, in one case associated with total disappearance of the head of the caudate nucleus. Except for age-appropriate cortical atrophy in one instance, no other changes were observed in the brain, brainstem, and cerebellum. The putaminal necrosis appears as typical "striatal slits" on the NMR scans. It is argued that this rare disease, since the princeps description in 1964 only reported in England (1986) and the U.S.A (1986), is most likely a singular type of mitochondrial encephalopathy: it is associated with Leber's optic atrophy, and the NMR changes observed have been signalled in other mitochondrial encephalomyelopathies, such as Leigh's disease and MELAS.

Adult↗
PubMed1991

Sciatic neuritis as initial symptom of spontaneous clostridial myonecrosis.

A 21-year-old woman is reported with aplastic anaemia, who presented with pain in the leg. Rapid loss of sciatic nerve function followed. MRI showed irregular streaks of low intensity in the muscles of the pelvic region. A diagnosis of sciatic neuritis as initial symptom of clostridial myonecrosis was made.

Adult↗
PubMed1991

Heredo-ataxia in a large Dutch pedigree. M.R.I. findings.

One segment of a large Dutch pedigree with heredo-ataxia is presented. The clinical and genetic features of the disease, together with the M.R.I. findings, indicate the diagnosis of autosomal dominant late onset olivo-ponto-cerebellar atrophy. The clinical picture remained invariant through successive generations. Emphasis is put on the diagnostic usefulness of M.R.I. technique in the absence of neuropathological data.

Adult↗
PubMed1990

Does very preterm birth impair myelination of the central nervous system?

Myelination of the central nervous system (CNS) can be demonstrated with magnetic resonance (MR) imaging. Myelin formation may be reduced in conditions of neonatal "undernutrition". Very preterm infants have a reduced postnatal growth rate when compared with intrauterine fetuses of the same gestational age. Using MR imaging, we studied qualitative myelination patterns in healthy preterm infants of less than 30 weeks gestation with an optimal nutritional intake and in term infants at 44 weeks postmenstrual age (PMA). At that age, preterm infants had a significantly lower mean body weight than term infants, but mean head circumference did not differ significantly. All preterm and term infants had reached myelination stage M3 (myelin in brainstem, internal capsule, and corona radiata) and M4 (myelin in brainstem, internal capsule, corona radiata, and centrum semiovale). There was no significant difference in myelination stage between the preterm and term infants. We conclude that adequate nutrition in the neonatal period leads to qualitatively adequate myelination of the CNS in very preterm infants.

Central Nervous System↗
PubMed1989

Optic glioma with intraocular tumor and seeding in a child with neurofibromatosis.

We treated a 3-year-old boy with neurofibromatosis who had an optic glioma, intraocular extension with seeding, and iris tumors. On the basis of results of ultrasonography, computed tomography, magnetic resonance imaging, and fine needle aspiration, other intraocular and orbital tumors were excluded. Because of the malignant intraocular aspect, the optic nerve glioma was extirpated. Histologic examination confirmed the diagnosis of an optic nerve glioma with intraocular extension, seeding, and iris nodules. In this juvenile pilocytic astrocytoma with secondary perineural fibrous hyperplasia, several mitoses were found in the orbital and intraocular parts. In the optic canal, three small islands were found that were compatible with the diagnosis of malignant astrocytoma, grade 3. The iris nevi appeared as iris pits and not like the Lisch nodules typical of neurofibromatosis. Café au lait spots were present on the skin. The family history was positive for neurofibromatosis. The results of this study demonstrate that optic gliomas are true astrocytomas and not hamartomas, and have a continuous scale from benign to malignant differentiation.

Child, Preschool↗
PubMed1989

MRI in eye tumors.

Thirty patients with intraocular tumors were examined with MRI. A clear demarcation was seen between the melanomas and other intraocular structures such as sclera, vitreous and ciliary body. Retinal detachment, hemorrhage or choroidal-retinal layer could be seen separated from the tumor. In all our patients investigated with MRI the absence of extraocular melanoma tissue was confirmed by histopathology or by the findings during surgery. The demarcation between tumor and sclera seemed more sensitive in MRI than in ultrasonography in establishing extrascleral extension. Our study showed that MRI can be a valuable addition to ultrasonography in detecting intraocular tumors or tumor simulating lesions.

Evaluation Studies as Topic↗