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Encyclopedia > Leukodystrophy
Leukodystrophy
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ICD-10 E71.3, E75.2
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ICD-9 330.0
OMIM {{{OMIM}}}
MedlinePlus {{{MedlinePlus}}}
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DiseasesDB {{{DiseasesDB}}}

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. Myelin, which lends its color to the white matter of the brain, is a complex substance made up of at least ten different chemicals. The leukodystrophies are a group of disorders that are caused by genetic defects in how myelin produces or metabolizes these chemicals. Each of the leukodystrophies is the result of a defect in the gene that controls one (and only one) of the chemicals. 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. ... 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), is the control center of the central nervous system. ... In neuroscience, myelin is an electrically insulating phospholipid layer that surrounds the axons of many neurons. ... Nerves (yellow)    Nerves redirects here. ... In neuroscience, myelin is an electrically insulating phospholipid layer that surrounds the axons of many neurons. ... This stylistic schematic diagram shows a gene in relation to the double helix structure of DNA and to a chromosome (right). ...

Contents


Types

Specific leukodystrophies include (ICD-10 codes are provided where available): The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ...

Adrenoleukodystrophy (ALD) is a degenerative disorder of the sheath covering nerve fibers, known as myelin. ... Metachromatic leukodystrophy (MLD) is the most common form of a family of genetic diseases known as the leukodystrophies, diseases which affect the growth and/or development of myelin, the fatty covering which acts as an insulator around nerve fibres throughout the central and peripherial nervous systems . ... Krabbe disease (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare, often fatal degenerative disorder that affects the nervous system. ... Pelizaeus-Merzbacher disease (PMD) is a rare central nervous system disorder in which coordination, motor abilities, and intellectual function are delayed to variable extents. ... Although Canavan disease may occur in any ethnic group, it affects persons of Eastern European Jewish ancestry more frequently. ... Alexander disease is a slowly progressing fatal neurodegenerative disease. ...

How do you get leukodystrophy?

Leukodystrophies are mostly inherited disorders, meaning that it is passed on from parent to child. They may be inherited in a recessive, dominant, or X-linked manner, depending on the type of leukodystrophy. The individual pages on each leukodystrophy will describe the particular pattern of inheritance for that disease. There is also a separate fact sheet describing the different genetic inheritance patterns available from the United Leukodystrophy Foundation.


There are some leukodystrophies that do not appear to be inherited, but rather arise spontaneously. They are still caused by a mutation in a particular gene, but it just means that the mutation was not inherited. In this case, the birth of one child with the disease does not necessarily increase the likelihood of a second child having the disease.


What is a leukodystrophy?

The word leukodystrophy comes from the Greek words leuko (meaning white), trophy (meaning growth), and dys (meaning ill). If you put these words together, the word leukodystrophy describes a set of diseases that affect the growth or maintenance of the white matter (myelin).


Symptoms

The most common symptom of a leukodystrophy disease is a gradual decline in an infant or child who previously appeared well. Progressive loss may appear in body tone, movements, gait, speech, ability to eat, vision, hearing, and behavior. There is often a slowdown in mental and physical development. Symptoms vary according to the specific type of leukodystrophy, and may be difficult to recognize in the early stages of the disease.


External links

  • This article incorporates public domain text from the National Institute of Neurological Disorders and Stroke.
  • Leukodystrophy Connections
  • Joey Johnson's Story about DHA and Leukodystrophy

The official color of Leukodystrophies awareness is Navy Blue.


More information on the Leukodystrophies, and research being supported, can be found at the The Stennis Foundation, located on the web at http://www.stennisfoundation.org.


  Results from FactBites:
 
Leukodystrophy: Encyclopedia of Genetic Disorders (1344 words)
Leukodystrophy describes a collection of about 15 rare genetic disorders that effect the brain, spinal cord and peripheral nerves.
Leukodystrophies covered as separate entries in this encyclopedia are adrenoleukodystrophy (ALD)/adrenomyeloneuropathy (AMN), Aicardi-Goutieres syndrome, canavan disease (spongy degeneration), Krabbe disease (globoid cell leukodystrophy), neonatal adrenoleukodystrophy, Pelizaeus-Merzbacher disease (X-linked spastic paraplegia), Refsum disease, and Zellweger syndrome.
Leukodystrophies are occasionally misdiagnosed as muscular dystrophy, since they all are neurological disorders involving white matter.
What is Leukodystrophy? - United Leukodystrophy Foundation (830 words)
The leukodystrophies are a group of rare genetic disorders that affect the central nervous system by disrupting the growth or maintenance of the myelin sheath that insulates nerve cells.
Leukodystrophies are mostly inherited disorders, meaning that it is passed on from parent to child.
However, the cause is different; whereas leukodystrophies are generally caused by a defect in one of the genes involved with the growth or maintenance of the myelin, MS is thought to be caused by an attack on the myelin by the body’s own immune system.
  More results at FactBites »

 
 

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