Dimitri Hemelsoet

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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)
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)
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)
OBJECTIVE In the Belgian Fabry Study (BeFaS), the prevalence of Fabry disease was assessed in 1000 young patients presenting with stroke, unexplained white matter lesions or vertebrobasilar dolichoectasia. The results of the BeFaS suggested that Fabry disease may play a role in up to 1% of young patients presenting with cerebrovascular disease. However, the(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)
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)