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Article
Open AccessAnti-nociceptive effect of Faecalibacterium prausnitzii in non-inflammatory IBS-like models
Visceral pain and intestinal dysbiosis are associated with Irritable Bowel Syndrome (IBS), a common functional gastrointestinal disorder without available efficient therapies. In this study, a decrease of Faecali...
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Article
Modèles expérimentaux de douleurs pelvi-périnéales
Les douleurs viscérales sont souvent associées à de nombreuses pathologies touchant les organes profonds. Face à l’hétérogénéité étiologique, clinique et physiopathologique de ces douleurs, la modélisation, ch...
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Article
Demyelinating peripheral neuropathy associated with hemophagocytic lymphohistiocytosis. An immuno-electron microscopic study
We report the case of an 11-year-old male who developed subacute diffuse polyradiculoneuropathy, associated with digestive symptoms and Epstein-Barr virus infection. Parental consanguinity was present. The lab...
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Article
Alexander disease: putative mechanisms of an astrocytic encephalopathy
Alexander disease (AXD) is the first primary astrocytic disorder. This encephalopathy is caused by dominant mutations in the glial fibrillary acidic protein (GFAP) gene, encoding the main intermediate filament...
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Article
Widespread distribution of β-hexosaminidase activity in the brain of a Sandhoff mouse model after coinjection of adenoviral vector and mannitol
Sandhoff disease is a severe inherited neurodegenerative disorder resulting from deficiency of the β-subunit of hexosaminidases A and B, lysosomal hydrolases involved in the degradation of GM2 ganglioside and rel...
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Article
Increased density of oligodendrocytes in childhood ataxia with diffuse central hypomyelination (CACH) syndrome: neuropathological and biochemical study of two cases
We report neuropathological, biochemical and molecular studies on two patients with childhood ataxia with diffuse central nervous system hypomyelination (CACH) syndrome, a leukodystrophy recently defined acco...
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Article
In vivo diagnosis of Kufs’ disease by extracerebral biopsies
In almost all of the earlier reported cases of Kufs’ disease, the adult form of ceroid lipofuscinosis, the diagnosis was ascertained by cerebral tissue examination, while peripheral biopsy examination reveale...
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Article
Developmental aspects of type II lissencephaly
We report neuropathological studies of five cases of type II lissencephaly from three fetuses and two infants. This comparative study allowed us to determine the developmental course of the cerebral lesions. T...