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Alexander's disease

Acta Neuropathologica, 1985
This is the first pathologic report of an infant at 37 weeks' gestation with Alexander's disease. The findings demonstrate that the disease can arise in utero and be extensive at birth before myelination begins.
J F Wilson
exaly   +5 more sources

Neurocognitive Decline in Alexander Disease

Clinical Neuropsychologist, 2011
Alexander disease is a neurological condition associated with prominent white matter deterioration. Its rarity and relatively rapid disease course have provided limited understanding into the cognitive effects of the illness. We report the serial neuropsychological findings of a 21-year-old with normal development and no medical history until age 9 ...
June, Restrepo   +2 more
exaly   +3 more sources

GFAP mutations in Alexander disease

International Journal of Developmental Neuroscience, 2002
AbstractAlexander disease is a rare but often fatal disease of the central nervous system. Infantile, juvenile and adult forms have been described that present with different clinical signs, but are unified by the characteristic presence in astrocytes of Rosenthal fibers–protein aggregates that contain glial fibrillary acidic protein (GFAP) and small ...
Albee Messing, James E Goldman
exaly   +3 more sources

Computed tomography in Alexander's disease

Neuroradiology, 1980
Two 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
exaly   +3 more sources

Alexander disease

Journal of child neurology, 2018
Alexander disease is a rare and generally fatal disorder of the central nervous system, originally defined by the distinctive neuropathology consisting of abundant Rosenthal fibers within the cytoplasm and processes of astrocytes. More recently, mutations in GFAP, encoding glial fibrillary acidic protein, the major intermediate filament protein of ...
Ali, Tavasoli   +11 more
openaire   +4 more sources

Alexander's disease

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
openaire   +2 more sources

Alexander disease

European Journal of Paediatric Neurology, 2003
Alexander disease is a rare disorder with limited understanding of its cause, although it does seem to be a disorder of astrocytes rather than a leukodystrophy. It can be divided into three groups: infantile, juvenile, and adult. The infantile type shows enlargement of the head, retarded development and evidence of a severe neurological disorder.
openaire   +2 more sources

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