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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 ...
Tomokatsu Yoshida
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Alexander disease: The story behind an eponym
Journal of the History of the Neurosciences, 2023In 1949, William Stewart Alexander (1919–2013), a young pathologist from New Zealand working in London, reported the neuropathological findings in a 15-month-old boy who had developed normally until the age of seven months, but thereafter had progressive
N. Anderson +2 more
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Journal of Child Neurology, 2023
Objective: To determine the ages at acquisition of developmental milestones, loss of motor function, and clinical symptoms in Alexander disease. Methods: Patients with confirmed cerebral Alexander disease were included.
Joshua Y Joung +8 more
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Objective: To determine the ages at acquisition of developmental milestones, loss of motor function, and clinical symptoms in Alexander disease. Methods: Patients with confirmed cerebral Alexander disease were included.
Joshua Y Joung +8 more
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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
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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
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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 J, Townsend +4 more
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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 J, Townsend +4 more
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Clinical and radiological characteristics of older‐adult‐onset Alexander disease
European Journal of Neurology, 2021Alexander disease (ALXDRD) affects a wide range of ages from infancy to adulthood. However, only a few cases involving patients with older‐adult onset over 65 years of age have been reported.
Tomokatsu Yoshida +3 more
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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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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.
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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.
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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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