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Archives of Neurology, 1964
This is the sixth report of a disease first described by Alexander in 1949. 1 Several names have been given to this cerebral disorder (Table), but the eponym "Alexander's disease" will be used herein. The main neuropathological characteristic was granular, eosinophilic deposits at all interfaces of the central nervous system; that is, in the ...
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This is the sixth report of a disease first described by Alexander in 1949. 1 Several names have been given to this cerebral disorder (Table), but the eponym "Alexander's disease" will be used herein. The main neuropathological characteristic was granular, eosinophilic deposits at all interfaces of the central nervous system; that is, in the ...
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Neurology, 2006
Alexander disease has always been considered a classic leukodystrophy (the term literally meaning abnormal white matter nutrition, but usually used more broadly to indicate any hereditary or metabolic white matter abnormality). Although rare (the true incidence is not yet known), the disease is always included in the differential diagnosis when ...
A. James Barkovich, Albee Messing
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Alexander disease has always been considered a classic leukodystrophy (the term literally meaning abnormal white matter nutrition, but usually used more broadly to indicate any hereditary or metabolic white matter abnormality). Although rare (the true incidence is not yet known), the disease is always included in the differential diagnosis when ...
A. James Barkovich, Albee Messing
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Journal of Child Neurology, 2003
Alexander's disease, a rare and fatal disorder of the central nervous system, most commonly affects infants and young children but can also occur in older children and sometimes adults. In infants and young children, it causes developmental delay, psychomotor retardation, paraparesis, feeding problems, usually megalencephaly, often seizures, and ...
Anne B, Johnson, Michael, Brenner
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Alexander's disease, a rare and fatal disorder of the central nervous system, most commonly affects infants and young children but can also occur in older children and sometimes adults. In infants and young children, it causes developmental delay, psychomotor retardation, paraparesis, feeding problems, usually megalencephaly, often seizures, and ...
Anne B, Johnson, Michael, Brenner
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Alexander's Disease in an Adult
Archives of Neurology, 1968PREVIOUS descriptions of Alexander's disease have generally been of cases in young children.1-8The clinical picture most often associated with this disease has been one of developmental retardation and enlargement of the head. Vogel and Hallervorden,9however, described a case in a 15-year-old girl that began with weakness of the left leg and foot at ...
F J, Seil, S S, Schochet, K M, Earle
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Alexander's Disease: Clues to Diagnosis
Journal of Child Neurology, 1993The clinical, radiologic, neurophysiologic, and pathologic findings in 10 children with histologically proven Alexander's disease are described, and the presence of two broad clinical subgroups is confirmed. Macrocephaly, regression, and seizures are found in the infantile form, whereas bulbar signs predominate the so-called juvenile form. None of the
C L, Pridmore +5 more
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Alexander's Disease: Unique Presentation
Journal of Child Neurology, 1999Subacute necrotizing encephalomyelopathy (Leigh syndrome) refers to a nebulous disease entity characterized by lactic acidosis, a wide variety a clinical manifestations, and a consistent conglomeration of pathlogic findings. Several abnormalities in metabolism have been delineated in association with Leigh syndrome, but many cases have no identified ...
M K, Gingold +3 more
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Pediatrie, 1991
Alexander disease, or hypoproconvertinemia is a rare autosomic recessive coagulation disorder. The features include familial and/or personal history of bleeding, with an abnormal prothrombin period and a normal activated partial thromboplastin period. Coagulation and genetic studies allow subclassification with prognosis incidence for this disease. The
J C, Elian +4 more
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Alexander disease, or hypoproconvertinemia is a rare autosomic recessive coagulation disorder. The features include familial and/or personal history of bleeding, with an abnormal prothrombin period and a normal activated partial thromboplastin period. Coagulation and genetic studies allow subclassification with prognosis incidence for this disease. The
J C, Elian +4 more
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Clinical Characteristics of Alexander Disease
Neurodegenerative Disease Management, 2020Alexander disease (ALXDRD) is a primary astrocyte disease caused by GFAP gene mutation. The clinical features of ALXDRD vary from infantile-onset cerebral white matter involvement to adult-onset brainstem involvement. Several studies revealed that the level of GFAP overexpression is correlated with disease severity, and basic research on therapies to ...
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Computed tomography in Alexander's disease
Annals of Neurology, 1984AbstractComputed tomography demonstrated contrast‐enhancing lesions in the periventricular frontal regions, caudate nuclei, and thalami in an infant with Alexander's disease. The distribution of the enhancing lesions corresponded to the areas in which Rosenthal fibers were most prominent.
K, Farrell, S, Chuang, L E, Becker
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1989
Alexander’s disease (AD) is a rare, nonfamilial disorder of the nervous system which is also called dysmyelinogenetic leukodystrophy. The disease occurs sporadically without clear familial incidence. Three clinical subgroups of AD can be distinguished: infantile, juvenile and adult.
Jacob Valk, Marjo S. van der Knaap
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Alexander’s disease (AD) is a rare, nonfamilial disorder of the nervous system which is also called dysmyelinogenetic leukodystrophy. The disease occurs sporadically without clear familial incidence. Three clinical subgroups of AD can be distinguished: infantile, juvenile and adult.
Jacob Valk, Marjo S. van der Knaap
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