A late autosomal dominant form of limb-girdle muscular dystrophy. A clinical, genetic, and morphological study.
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Biomedical subjects
Publications and source records attributed to J De Reuck.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
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Normal gastrocnemius muscle of Wistar rats at various ages to 2 1/2 years was studied. No light microscopic or qualitative electron microscopic changes were seen. However, stereologic determinations revealed significant reductions in volume and surface densities of sarcoplasmic reticulum, t-tubular system, and glycogen content. These alterations were considered to be the morphologic expression of the very early age-related changes in gastrocnemius muscle.
Cobalt-55 (55Co) is a positron emission tomography (PET) tracer used to demonstrate brain damage, possibly associated to calcium-mediated processes. The degree of 55Co accumulation correlates with the severity of ischaemia in stroke patients. It is still a matter of debate whether ipsilateral thalamic hypometabolism (ITH) and crossed cerebellar hypometabolism (CCH), occurring after middle cerebral artery (MCA) infarcts, represent only a metabolic depression of these remote regions or can lead to structural damage. The present study investigates whether an increased 55Co influx can be demonstrated with PET in ITH and CCH after MCA infarcts. About half of the patients with ITH and CCH had a significant degree of 55Co uptake in, respectively, the ipsilateral thalamus and the contralateral cerebellar hemisphere. It was observed in patients with severe signs of stroke on admission and poor clinical outcome, and correlated well with the degree of 55Co influx within the supratentorial infarct. The present study demonstrates that ITH and CCH after MCA infarction can represent structural damage in these remote areas that occurs during the second week after stroke onset.
Two patients presenting with progressive dysarthria as the single initial manifestation of a neurodegenerative condition are described. The nature of the dysarthria as well as the additional symptoms that developed in the course of the disorder are very different in these two cases. Nevertheless, neuroimaging findings are strikingly similar and suggest bilateral involvement of posterior inferior frontal lobe structures, mainly in the dominant cerebral hemisphere. The clinical syndrome of these patients can therefore be considered an example of frontotemporal degeneration presenting without dementia or compartmental alteration, at least in the early stages. This broadens the clinical spectrum of frontotemporal degeneration and demonstrates the need for a syndromal subclassification of this nosological entity.
A case of central pontine myelinolysis is described. The clinical, angiographic and post mortem findings suggest that among the associated disorders the oligemic shock combined with a local decreased flow in the basilar artery was the most important cause of this patholigical entity. The angioarchitecture findings in the brain stem, compared to these in 26 normal brains, confirm this hypothesis.
A 7-year-old mentally retarded girl died following subacute dermatomyositis. Muscle biopsies supported the clinical diagnosis and revealed paracrystalline inclusions on EM. The brain autopsy showed cerebral and cerebellar polymicrogyria. The clinico-pathological findings in this child are related to similar previously reported data in her older sister. The possibility of a new autosomal recessive syndrome involving both fetal brain development and childhood immunological function is discussed.
The efficiency of two types of steroid treatment was assessed in primary intracerebral haemorrhages. No significant difference in clinical evolution was observed between 57 cases treated with dexamethasone, 28 cases with methylprednisolone and 44 cases without corticosteroids. In a subgroup of 46 patients with putamino-capsular bleedings the non-steroid-treated group did significantly better than the steroid-treated one. Adverse reactions occurred at the same rate in the three groups.
A case of cervical myelopathy due to ossification of the posterior longitudinal ligament is presented. This disease is rather frequent among Japanese, but rare among non-Mongolian persons. To our knowledge, this is the first published Belgian patient with a myelopathy. The clinical and radiographic data are discussed together with a review of the literature.
The clinico-pathological findings in a 61-year-old man, who suffered from branchial and skeletal myoclonus, appearing six months after a brainstem infarction are reported. Of all the drugs which are usually thought to be effective in the treatment of myoclonus, only valproic acid brought some relief. The necropsy revealed bilateral hypertrophy of the olives, together with bilateral pontine tegmental and rubral infarctions, without involvement of the olivary pathway.
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A case of a primary intracerebral lymphoma, presenting with posterior uveitis, is reported together with its stepwise diagnostic approach. Various aspects of the diagnostic and therapeutic problems with these malignant tumours are discussed. The importance of indirect techniques for achievement of the diagnosis is emphasized.
A case of left hemiballism is presented. Besides multiple lacunar infarcts an haemorrhage in the contralateral subthalamic nucleus is shown. A short review of the literature is given with special attention to the etiology and the therapy.
A 61-year old man was admitted for sudden right hemiparesis. The first CT brain scan revealed a haemorrhage in the left frontal horn. The cause of the bleeding could not be discovered initially. Subsequent CT brain scans showed an isodense mass lesion in the left frontal horn. The patient died after a relapsing course of the hemiparesis. The postmortem examination demonstrated a large protoplasmic astrocytoma of the left basal ganglia with recurrent large bleedings.
A 64-year-old bilingual lawyer complained of an abrupt loss of comprehension of spoken words and much less severe loss of recognition of various sounds. An isolated partial auditory agnosia, due to a left-sided, temporal dysfunction was confirmed by the clinical neuropsychological and logopedic examination. Only a minimal left cortical, peri-insular hypodensity was suspected on CT-scan of the brain, while the NMR-scan was inconclusive. Arteriography showed a large coiling internal carotid artery on the left side, as a possible source of thrombo-embolism.