Alexander disease


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Al·ex·an·der dis·ease

(al-ek-zan'dĕr), [MIM*203450]
a rare, fatal degenerative disease of the central nervous system of infants, characterized by psychomotor retardation, seizures, and paralysis; megaloencephaly is associated with widespread leukodystrophic changes, especially in the frontal lobes.

Alexander disease

A rare, progressive leukoencephalopathy (OMIM:203450) of the CNS, histologically characterised by widespread accumulation of Rosenthal fibres (cytoplasmic inclusions in astrocytes) and failure of central myelination. The most common form develops in infancy, and is accompanied by megalencephaly, developmental delay, spasticity, seizures and psychomotor retardation. Patients with juvenile or adult forms experience ataxia, bulbar signs and spasticity and slower progression.

Molecular pathology
Defects in GFAP, which encodes a major intermediate filament, cause Alexander disease.

Al·ex·an·der dis·ease

(al-ĕg-zan'dĕr di-zēz')
A rare, fatal central nervous system degenerative disease of infants, characterized by psychomotor retardation, seizures, and paralysis; megaloencephaly is associated with widespread leukodystrophic changes, especially in the frontal lobes.

Alexander,

William Stuart, 20th century New Zealand pathologist.
Alexander disease - a rare, fatal, central nervous system degenerative disease of infants.
References in periodicals archive ?
The 33-year-old and nine of his friends and family have raised PS21,000 so far on their Children's Hospitals Challenge to raise awareness of Alexander disease.
But Emilia also has Alexander disease - a nervous system disorder so rare there have only been 500 reported cases worldwide.
They describe the history of neuroscience; the morphology and interrelationships between glial cells and neurones in different parts of the nervous system; the cellular physiology of the different kinds of glial cells; the mechanisms of intra and inter-cellular signaling in glial networks and glial-neuronal communications; the role of glial cells in synaptic plasticity, neuronal survival, and the development of the nervous system; the cellular and molecular mechanisms of metabolic neuronal-glial interactions; and the role of glia in nervous system pathology, including the pathology of glial cells and associated diseases like multiple sclerosis, Alzheimer's, Alexander disease, and Parkinson's disease.
Based on these MR findings, a diagnosis of Alexander disease was made.
Four years later, seemingly healthy Alexandra was diagnosed with the extremely rare Alexander Disease.
Four years later seemingly healthy Alexandra was diagnosed with the extremely rare Alexander Disease.
X-linked inheritance strongly suggests adrenomyeloneuropathy, Fabry disease, pre-mutation X-fragile diagnosis; dominant inheritance suggests cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL), collagen type IV (Col IV) mutation, Nasu-Hakola disease (NHD), adult-onset autosomal dominant leucodystrophies (ADLD) and Alexander disease diagnosis.
Considered a cousin to Alzheimer's and Parkinson's disease, Alexander disease attacks the nervous system of children, usually before the age of 1.
In fact, it's so rare that just 500 cases of Alexander disease, a nervous system disorder, have been reported worldwide.
Alexander disease was originally described by Alexander in 1949.
The little girl, a pupil at Earlsdon Primary School, had been suffering from Alexander Disease, a rare brain disorder which caused tumours and severe sickness.
The Kathy Stokes School of Dance has donated pounds 500 to Alexandra Emery, a seven-yearold Earlsdon Primary School pupil who has Alexander Disease, a terminal disorder affecting the nervous system.

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