The Experts below are selected from a list of 15 Experts worldwide ranked by ideXlab platform
M Chung - One of the best experts on this subject based on the ideXlab platform.
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familial Binswanger Encephalopathy in the absence of hypertension a new family with the cadasil syndrome
American Journal of Human Genetics, 1994Co-Authors: N C Schanen, P Glusker, M ChungAbstract:Binswanger Encephalopathy is a clinicopathologic entity characterized by deep white matter ischaemic lesions associated with hypertension and arteriosclerosis; but a rare inherited form, termed CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and LeukoEncephalopathy) has been recently described with earlier onset in the absence of hypertension. This syndrome has been linked to markers in chromosome band 19q12 in of two unrelated French families. We have identified a family of Central American heritage with a similar syndrome also demonstrating an autosomal dominant pattern of inheritance. Clinically, the onset of symptoms is between 19-53 years of age with variable progression in loss of neurologic function. Numerous lesions are visible on MRI involving the deep white matter including the corpus callosum as well as basal ganglia. None of the family members has had significant hypertension. Extensive evaluations of metabolic, infectious and inflammatory origin of disease have been noncontributory. In the two patients whose brains were examined pathologically, multiple cystic lesions, frequently centered around blood vessels, were present in the deep white matter and basal ganglia with diffuse loss of myelin and relative preservation of the neurons. The media of large and small parenchymal arteries shows hyaline and hydropic degeneration with replacement of themore » smooth muscle by a slate-grey amorphous substance on trichrome staining, corresponding to granular osmiophilic material on ultrastructural examination. There was no evidence of amyloid or atherosclerosis in the vessels. Phenotypically, this family appears to have the CADASIL syndrome. We are currently pursuing linkage analysis with chromosome 19 markers to investigate the genetic homogeneity of the disorder. Thirty-three individuals spanning three generations and including seven clinically affected patients are potentially available for study.« less
N C Schanen - One of the best experts on this subject based on the ideXlab platform.
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familial Binswanger Encephalopathy in the absence of hypertension a new family with the cadasil syndrome
American Journal of Human Genetics, 1994Co-Authors: N C Schanen, P Glusker, M ChungAbstract:Binswanger Encephalopathy is a clinicopathologic entity characterized by deep white matter ischaemic lesions associated with hypertension and arteriosclerosis; but a rare inherited form, termed CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and LeukoEncephalopathy) has been recently described with earlier onset in the absence of hypertension. This syndrome has been linked to markers in chromosome band 19q12 in of two unrelated French families. We have identified a family of Central American heritage with a similar syndrome also demonstrating an autosomal dominant pattern of inheritance. Clinically, the onset of symptoms is between 19-53 years of age with variable progression in loss of neurologic function. Numerous lesions are visible on MRI involving the deep white matter including the corpus callosum as well as basal ganglia. None of the family members has had significant hypertension. Extensive evaluations of metabolic, infectious and inflammatory origin of disease have been noncontributory. In the two patients whose brains were examined pathologically, multiple cystic lesions, frequently centered around blood vessels, were present in the deep white matter and basal ganglia with diffuse loss of myelin and relative preservation of the neurons. The media of large and small parenchymal arteries shows hyaline and hydropic degeneration with replacement of themore » smooth muscle by a slate-grey amorphous substance on trichrome staining, corresponding to granular osmiophilic material on ultrastructural examination. There was no evidence of amyloid or atherosclerosis in the vessels. Phenotypically, this family appears to have the CADASIL syndrome. We are currently pursuing linkage analysis with chromosome 19 markers to investigate the genetic homogeneity of the disorder. Thirty-three individuals spanning three generations and including seven clinically affected patients are potentially available for study.« less
P Glusker - One of the best experts on this subject based on the ideXlab platform.
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familial Binswanger Encephalopathy in the absence of hypertension a new family with the cadasil syndrome
American Journal of Human Genetics, 1994Co-Authors: N C Schanen, P Glusker, M ChungAbstract:Binswanger Encephalopathy is a clinicopathologic entity characterized by deep white matter ischaemic lesions associated with hypertension and arteriosclerosis; but a rare inherited form, termed CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and LeukoEncephalopathy) has been recently described with earlier onset in the absence of hypertension. This syndrome has been linked to markers in chromosome band 19q12 in of two unrelated French families. We have identified a family of Central American heritage with a similar syndrome also demonstrating an autosomal dominant pattern of inheritance. Clinically, the onset of symptoms is between 19-53 years of age with variable progression in loss of neurologic function. Numerous lesions are visible on MRI involving the deep white matter including the corpus callosum as well as basal ganglia. None of the family members has had significant hypertension. Extensive evaluations of metabolic, infectious and inflammatory origin of disease have been noncontributory. In the two patients whose brains were examined pathologically, multiple cystic lesions, frequently centered around blood vessels, were present in the deep white matter and basal ganglia with diffuse loss of myelin and relative preservation of the neurons. The media of large and small parenchymal arteries shows hyaline and hydropic degeneration with replacement of themore » smooth muscle by a slate-grey amorphous substance on trichrome staining, corresponding to granular osmiophilic material on ultrastructural examination. There was no evidence of amyloid or atherosclerosis in the vessels. Phenotypically, this family appears to have the CADASIL syndrome. We are currently pursuing linkage analysis with chromosome 19 markers to investigate the genetic homogeneity of the disorder. Thirty-three individuals spanning three generations and including seven clinically affected patients are potentially available for study.« less
Richard Yanagihara - One of the best experts on this subject based on the ideXlab platform.
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Amyloid β-protein in cerebral amyloid angiopathy, senile plaques, and preamyloidotic lesions in subcortical arteriosclerotic Encephalopathy (Binswanger disease)
Neuroscience Letters, 1991Co-Authors: Don C. Guiroy, Andrzej Bogucki, Wielisław Papierz, Pawel P. Liberski, Richard YanagiharaAbstract:Abstract Cerebral vascular amyloid deposits, senile plaques and neurofibrillary tangles have been found in subcortical arteriosclerotic Encephalopathy (Binswanger disease). A mouse antiserum, prepared against a 43-amino acid synthetic peptide homologous to the amyloid β-protein of Alzheimer disease (anti-SP43), revealed immunoreactive amyloid deposits in meningeal and intracortical blood vessels, senile plaques, intraneuronal amyloid and preamyloid in a neuropathologically confirmed case of Binswanger disease previously reported to have cerebral vascular amyloid deposits. These lesions contained sulfated glycosaminoglycans as determined by the Alcian blue/critical electrolyte concentration method. Similar findings were not observed in a case of Binswanger Encephalopathy without cerebral amyloid deposits. Our study indicates that amyloidotic lesions in Binswanger Encephalopathy with cerebral amyloid deposits contain amyloid β-protein and sulfated glycosaminoglycans.
Don C. Guiroy - One of the best experts on this subject based on the ideXlab platform.
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Amyloid β-protein in cerebral amyloid angiopathy, senile plaques, and preamyloidotic lesions in subcortical arteriosclerotic Encephalopathy (Binswanger disease)
Neuroscience Letters, 1991Co-Authors: Don C. Guiroy, Andrzej Bogucki, Wielisław Papierz, Pawel P. Liberski, Richard YanagiharaAbstract:Abstract Cerebral vascular amyloid deposits, senile plaques and neurofibrillary tangles have been found in subcortical arteriosclerotic Encephalopathy (Binswanger disease). A mouse antiserum, prepared against a 43-amino acid synthetic peptide homologous to the amyloid β-protein of Alzheimer disease (anti-SP43), revealed immunoreactive amyloid deposits in meningeal and intracortical blood vessels, senile plaques, intraneuronal amyloid and preamyloid in a neuropathologically confirmed case of Binswanger disease previously reported to have cerebral vascular amyloid deposits. These lesions contained sulfated glycosaminoglycans as determined by the Alcian blue/critical electrolyte concentration method. Similar findings were not observed in a case of Binswanger Encephalopathy without cerebral amyloid deposits. Our study indicates that amyloidotic lesions in Binswanger Encephalopathy with cerebral amyloid deposits contain amyloid β-protein and sulfated glycosaminoglycans.
