Results 271 to 280 of about 16,484,276 (296)
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Neurology, 1976
A boy with juvenile onset of symptoms of Alexander's disease had a clinical picture of pseudobulbar palsy, ataxia, and spastic paraparesis. Pathologic examination of the central nervous system revealed the diffuse presence of Rosenthal fibers and patchy demyelination.
L S, Russo, A, Aron, P J, Anderson
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A boy with juvenile onset of symptoms of Alexander's disease had a clinical picture of pseudobulbar palsy, ataxia, and spastic paraparesis. Pathologic examination of the central nervous system revealed the diffuse presence of Rosenthal fibers and patchy demyelination.
L S, Russo, A, Aron, P J, Anderson
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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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Alexander's disease: cranial ultrasound findings
Pediatric Radiology, 1988This is thought to be the first report of the recognition by cranial ultrasound of the abnormal pattern of cerebral tissues which occurs in Alexander's disease. This finding suggests that cranial ultrasound could be a useful adjunct in the diagnosis of this cerebral leukodystrophy, particularly in those infants presenting with megalencephaly.
M G, Harbord, G W, LeQuesne
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2014
Alexander's disease can occur at any age. Children typically present with delayed development, seizures, and macrocephaly, whereas older patients generally display bulbar symptoms. Myelin defects can be severe in younger patients, but subtle or absent in older patients.
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Alexander's disease can occur at any age. Children typically present with delayed development, seizures, and macrocephaly, whereas older patients generally display bulbar symptoms. Myelin defects can be severe in younger patients, but subtle or absent in older patients.
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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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Fetal-onset Alexander disease with radiological-neuropathological correlation
Pediatric Radiology, 2023J. Devos +6 more
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Computed tomography in Alexander's disease
Neuroradiology, 1980Two cases of biopsy-proven Alexander's disease are described with computed tomographic changes which, in our experience and on survey of the literature, have not occurred in any other condition. Such changes in a child with a progressive condition consistent with Alexander's disease. strongly support the diagnosis.
I M, Holland, B E, Kendall
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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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Adult-onset Alexander disease among patients of Jewish Syrian descent
Neurogenetics, 2023Saar Anis +11 more
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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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