|
NIH - Leukodystrophies
Alexander Disease Information Page
Alexander Disease -- GeneReviews -- NCBI Bookshelf
Disease characteristics. Alexander Disease is a disorder of cortical white matter that predominantly affects infants and children and usually results in death within ten years after onset. Most individuals present with nonspecific neurologic signs and symptoms. The two most common forms are infantile (about 63% of affected individuals) and juvenile (about 24%); however, neonatal and adult forms are also recognized. The infantile form initially presents in the first two years of life. Individuals typically have progressive psychomotor retardation with loss of developmental milestones, megalencephaly and frontal bossing, seizures, hyperreflexia and pyramidal signs, ataxia, and hydrocephalus secondary to aqueductal stenosis. Affected children survive a few weeks to several years. The juvenile form usually presents between four and ten years of age, occasionally in the mid-teens. Survival is variable, ranging from the early teens to the 20s-30s. Affected individuals can present with bulbar/ pseudobulbar signs, ataxia, gradual loss of intellectual function, seizures, megalencephaly, and breathing problems. The neonatal form is characterized by onset within the first month of life with rapid progression leading to severe disability or death within the first two years of life. Characteristics include seizures, hydrocephalus, severe motor and mental retardation, severe white matter abnormalities, involvement of the basal ganglia and cerebellum, and elevated CSF protein concentration. The adult form is the most variable and least common.
Alexander disease - Genetics Home Reference
Most cases of Alexander Disease begin before age 2 years (the infantile form). Signs and symptoms of the infantile form typically include an enlarged brain and head (megalencephaly), seizures, stiffness in the arms and/ or legs (spasticity), mental retardation, and delayed physical development. Less frequently, onset occurs later in childhood (the juvenile form) or adulthood. Common problems in juvenile and adult forms of Alexander Disease include speech abnormalities, swallowing difficulties, and poor coordination (ataxia).
OMIM - ALEXANDER DISEASE
OMIM - Alexander Disease
Alexander's disease
Alexander's Disease
ClinicalTrials.gov - Information on Clinical Trials and Human Research Studies: Trial List
Alexander Disease
Alexander Disease Organizations: National Institute of Neurological Disorders and Stroke (NINDS)
Alexander Disease Organizations: National Institute of Neurological Disorders and Stroke (NINDS)
Alexander technique
Alexander technique
Alexander Disease Information Page: National Institute of Neurological Disorders and Stroke (NINDS)
Alexander Disease is one of a group of neurological conditions known as the leukodystrophies, disorders that are the result
of abnormalities in myelin, the white matter that protects nerve fibers in the brain. Alexander Disease is a progressive
and usually fatal Disease. The destruction of white matter is accompanied by the formation of Rosenthal fibers, which are
abnormal clumps of protein that accumulate in non-neuronal cells of the brain called astrocytes. Rosenthal fibers are sometimes
found in other disorders, but not in the same amount or area of the brain that are featured in Alexander Disease. The infantile
form is the most common type of Alexander Disease. It has an onset during the first two years of life. Usually there are
both mental and physical developmental delays, followed by the loss of developmental milestones, an abnormal increase in head
size, and seizures. The juvenile form of Alexander Disease is less common and has an onset between the ages of two and thirteen.
These children may have excessive vomiting, difficulty swallowing and speaking, poor coordination, and loss of motor control.
Adult-onset forms of Alexander Disease are rare, but have been reported. The symptoms sometimes mimic those of Parkinson s
Disease or multiple sclerosis. The Disease occurs in both males and females, and there are no ethnic, racial, geographic,
or cultural/ economic differences in its distribution.
NWHIC Publication Catalog Record Details
This on-line publication reviews treatment, prognosis, research, and resources for Alexander Disease.
Health & Education
Health & Education
womenshealth.gov - Publication Catalog Search Results
NINDS Alexander Disease Information Page This on-line publication reviews treatment, prognosis, research , and resources for Alexander Disease. GENETIC DISORDERS,NEUROLOGICAL DISEASES AND DISORDERS details
Yellowstone InDepth - Troubled Waters Transcript
Yellowstone InDepth - Troubled Waters Transcript
Endo Organizations
Endo Organizations
CDC - Alexander the Great and West Nile Virus Encephalitis (Replies)
CDC - Alexander the Great and West Nile Virus Encephalitis (Replies)
MedlinePlus: Leukodystrophies
Genetics Home Reference: Alexander Disease
NIH - Neurologic Diseases (General)
Alexander Disease Information Page
Home - Public Health Image Library (PHIL)
Home - Public Health Image Library (PHIL)
Electrical Impulses Foster Insulation of Brain Cells, Speeding Communications, March 17, 2006 News Release - National Institutes of Health (NIH)
Dr. Fields explained that the finding has implications for disorders affecting myelination, such as Alexander Disease, which is a fatal neurological disorder of childhood caused by a genetic defect in astrocytes. The brains of children who have Alexander Disease also have severe myelin defects. The finding that astrocytes indirectly relay signals from neurons to oligodendrocytes provides a possible explanation for the lack of myelin characteristic of the disorder. Researchers may be able to provide treatment for demylinating diseases, such as multiple sclerosis, by developing drugs that mimic the actions of ATP and LIF on their target cells. Similarly, an understanding of how myelination takes place may offer insight into the learning process.
Brain and nervous system - Genetics Home Reference
fibrinoid degeneration of astrocytes see Alexander Disease
|