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BACKGROUND Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), or Nasu-Hakola disease, is a presenile dementia associated with loss of myelin, basal ganglia calcification, and bone cysts. It is caused by recessively inherited mutations in two genes encoding subunits of a cell membrane-associated receptor complex: TREM2 and(More)
BACKGROUND AND PURPOSE Data on the prevalence of Fabry disease in patients with central nervous system pathology are limited and controversial. In this study, we assessed the prevalence of Fabry disease in young patients presenting with cerebrovascular disease in Belgium. METHODS In this national, prospective, multicenter study, we screened for Fabry(More)
Rare monogenic dementias have repeatedly exposed novel pathways guiding to details of the molecular pathogenesis behind this complex clinical phenotype. In this paper, we have studied polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), an early onset dementia with bone fractures caused by mutations in TYROBP (DAP12) and(More)
A 49-year-old man presented with episodic hypothermia many years after sustaining a contusional brain injury. Brain magnetic resonance imaging demonstrated the destruction of the anterior parts of the corpus callosum without hypothalamic lesions. Nevertheless, delayed hypothalamic dysfunction at the neurotransmitter level is the probable pathophysiological(More)
OBJECTIVE It is unclear whether carotid revascularization can improve the cognitive problems often observed in patients with carotid stenosis. We examined the presence of preoperative disturbances and the effects of different types of carotid revascularization on cognition. METHOD Forty-six patients treated for significant carotid stenosis [26 carotid(More)
BACKGROUND Seizures occur more frequently in patients with an intracerebral haematoma (ICH) than in those with a cerebral infarct. However, the risk factors for seizures in association with an ICH are less well known. PURPOSE The characteristics of medically treated patients with spontaneous ICHs, who developed seizures, were retrospectively compared to(More)
Fabry disease is an X-linked inborn error of glycosphingolipid metabolism caused by quantitative or qualitative defects in the lysosomal enzyme alfa-Galactosidase A (aGAL A), ultimately resulting in vital organ dysfunction. Mainly the kidneys, the heart, and the central nervous system are involved. While the classical phenotype of Fabry disease is readily(More)
BACKGROUND Postinterventional cerebral hyperdensities (PCHDs) are a common finding after endovascular stroke treatment. There is uncertainty about the extent to which PCHDs correspond to hemorrhage or contrast staining. Our aim was to evaluate the use of PCHD density on immediate postinterventional CT, and PCHD evolution on follow-up CT for differentiating(More)
PURPOSE To evaluate a series of patients treated electively with carotid endarterectomy (CEA), transfemoral carotid artery stenting with distal filter protection (CASdp), and transcervical carotid stenting with dynamic flow reversal (CASfr) monitored continuously with transcranial Doppler (TCD) during the procedure to detect intraoperative embolization(More)
There is ample evidence from randomized trials that for patients with stroke, stroke unit care is superior to care in general medical or neurological wards. This evidence, which has been adopted by international guidelines has to be implemented into daily stroke care. This consensus document prepared by the Belgian Stroke Council provides a set of minimum(More)