Alexander disease | [[Image:{{{Image}}}|190px|center|]] | | {{{Caption}}} | | ICD-10 | | | ICD-O: | {{{ICDO}}} | | ICD-9 | 331.89 | | OMIM | {{{OMIM}}} | | MedlinePlus | {{{MedlinePlus}}} | | eMedicine | {{{eMedicineSubj}}}/{{{eMedicineTopic}}} | | DiseasesDB | {{{DiseasesDB}}} | Alexander disease is a slowly progressing fatal neurodegenerative disease. The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ...
The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ...
The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ...
The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ...
MedlinePlus (medlineplus. ...
eMedicine is an online clinical medical knowledge base that was founded in 1996. ...
The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ...
Neurodegenerative disease is a condition which affects the brain function. ...
Synonyms
- Dysmyelogenic Leukodystrophy
- Dysmyelogenic Leukodystrophy-Megalobare
- Fibrinoid Degeneration of Astrocytes
- Fibrinoid Leukodystrophy
- Hyaline Panneuropathy
- Leukodystrophy with Rosenthal Fibers
- Megalencephaly with Hyaline Inclusion
- Megalencephaly with Hyaline Panneuropathy
Clinical features Delays in development of some physical, psychical and behavioral skills, progressive enlargement of the head (macrocephaly), seizures, spasticity, in some cases also hydrocephalus, dementia, clumsy movements. Cephalic disorders are congenital conditions that stem from damage to, or abnormal development of, the budding nervous system. ...
This article is about the medical condition. ...
Spasticity is a disorder of the bodys motor system in which certain muscles are continuously contracted. ...
For other senses of this word, see dementia (disambiguation). ...
This genetically based condition, affecting the central nervous system (mid brain and cerebellum)is caused by mutations in the gene for glial fibrillary acidic protein (GFAP) that maps to chromosome 17q21. It is inherited in an autosomal dominant manner. Alexander disease belongs to leukodystrophies, a group of diseases which affect growth or development of the myelin sheath. The destruction of white matter in the brain is accompanied by the formation of fibrous, eosinophilic deposits known as Rosenthal fibers. Pathology (from Greek pathos, feeling, pain, suffering; and logos, study of; see also -ology) is the study of the processes underlying disease and other forms of illness, harmful abnormality, or dysfunction. ...
Figure 1a: A human brain, with the cerebellum in purple. ...
This stylistic schematic diagram shows a gene in relation to the double helix structure of DNA and to a chromosome (right). ...
Intermediate filaments are one component of the cytoskeleton - important structural components of living cells. ...
Figure 1: Chromosome. ...
An autosomal dominant gene is an abnormal gene on one of the autosomal (non-sex determining) chromosomes. ...
Leukodystrophy refers to progressive degeneration of the white matter of the brain due to imperfect growth or development of the myelin sheath, the fatty covering that acts as an insulator around nerve fiber. ...
In neuroscience, myelin is an electrically insulating fatty layer that surrounds the axons of many neurons, especially those in the peripheral nervous system. ...
White matter is one of the two main solid components of the central nervous system. ...
Comparative brain sizes In animals, the brain, or encephalon (Greek for in the head), acts as the control center of the central nervous system. ...
CT shows: CT apparatus in a hospital Computed tomography (CT), originally known as computed axial tomography (CAT) and body section roentgenography, is a medical imaging method employing tomography where digital geometry processing is used to generate a three-dimensional image of the internals of an object from a large series of two...
- decreased density of white matter
- frontal lobe predominance
- +/- dilated lateral ventricles
Unknown. Etiology (alternately aetiology, aitiology) is the study of causation. ...
Occurrence and prevalence Very rare, occurs mostly in males. The infantile form (80% of all cases) starts usually at the age of six months or within the first two years. The average duration of the infantile form of the illness is usually about 3 years. Onset of the juvenile form (14% of all cases) presents usually between four to ten years of age. Duration of this form is in most cases about 8 years. In younger patients, seizures, megalencephaly, developmental delay, and spasticity are usually present. Neonatal onset is also reported. Onset in adults is least frequent. In older patients, bulbar or pseudobulbar symptoms and spasticity predominate. Symptoms of the adult form may also resemble multiple sclerosis. There are no more than 300 cases reported.
Treatment There is neither cure nor standard treatment for Alexander disease. All treatment is symptomatic and supportive, for example antibiotics for intercurrent infection and anticonvulsants for seizure control are usually used. An antibiotic is a drug that kills or slows the growth of bacteria. ...
The anticonvulsants, sometimes also called antiepileptics, belong to a diverse group of pharmaceuticals used in prevention of the occurrence of epileptic seizures. ...
Prognosis The prognosis is generally poor. With early onset, death usually occurs within 10 years after the onset of symptoms. Usually, the later the disease occurs, the slower its course is. |
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