Neurodegenerative disease
From Wikipedia, the free encyclopedia
| Neurodegenerative disease | |
| Classification and external resources | |
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| Para-sagittal MRI of the head in a patient with benign familial macrocephaly. | |
| MeSH | D019636 |
Neurodegenerative disease (Greek νέυρο-, néuro-, "nerval" and Latin dēgenerāre, "to decline" or "to worsen") is a condition in which cells of the brain and spinal cord are lost. The brain and spinal cord are composed of neurons that do different functions such as controlling movements, processing sensory information, and making decisions. Cells of the brain and spinal cord are not readily regenerated en masse, so excessive damage can be devastating. Neurodegenerative diseases result from deterioration of neurons or their myelin sheath which over time will lead to dysfunction and disabilities resulting from this.[1]
Some sources limit the term "degenerative" to conditions primarily affecting gray matter that are not associated with a obvious inciting event. [2]
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[edit] Classification
They are crudely divided into two groups according to phenotypic effects, although these are not mutually exclusive:
- conditions causing problems with movements, such as ataxia, and
- conditions affecting memory and related to dementia.
[edit] Pathophysiology
Misfolded proteins, or prions, were modified post-translationally, so that they no longer perform their cellular functions and instead trigger equivalent modifications in normal proteins, thus creating a cascade of damage that eventually results in significant neurodegeneration. In humans, this can cause Creutzfeldt-Jakob disease or variant CJD (Mad Cow Disease).
Normally, neurodegeneration begins long before the patient experiences any symptoms. It can be months or years before any effect is felt[citation needed]. Symptoms are noticed when many cells die or cease to function and a part of the Regulation, or production of microglia by the immune system, in a process of neuroinflammation, is currently being rigorously studied for its role in neurodegenerative diseases.[3][4][5][6]
[edit] Treatment
Initial treatment is dependent on diagnosis of underlying disorder. At present there are few therapies for the wide range of neurodegenerative diseases. Treatment with L-dopa can inhibit symptoms of Parkinson's Disease for a short time, but then causes acceleration of the symptoms. Efforts are being made to develop therapies for Alzheimer's Disease that will stabilize cognitive function at the level existing at time of diagnosis and treatment.
[edit] Epidemiology
Each specific kind of neurodegenerative disease has its own characteristics, but most affect middle-aged or older people. They usually worsen over time.
[edit] Research directions
Research is highly invested in stem cell technology and stem cell treatments, as well as Gene therapy.
Research is underway into biomarkers as part of an attempt to understand the progression of certain types of neurodegenerative disease. In theory, if relevant bio-markers were identified, people could be treated for such diseases prior to onset of symptoms, thus resulting in a significant extension of their normal functional lifespan. As yet, however, the science of bio-markers is in its infancy and consequently diagnosis of neurodegenerative disease tends to occur after the patient has already suffered the majority of the neural damages.
[edit] List of neurodegenerative diseases and conditions
- Adrenoleukodystrophy (ALD)
- Alcoholism
- Alexander's disease
- Alper's disease
- Alzheimer's disease
- Amyotrophic lateral sclerosis (Lou Gehrig's Disease)
- Ataxia telangiectasia
- Batten disease (also known as Spielmeyer-Vogt-Sjögren-Batten disease)
- Bovine spongiform encephalopathy (BSE)
- Canavan disease
- Cerebral palsy
- Cockayne syndrome
- Corticobasal degeneration
- Creutzfeldt-Jakob disease
- Familial fatal insomnia
- Frontotemporal lobar degeneration
- Huntington's disease
- HIV-associated dementia
- Kennedy's disease
- Krabbe's disease
- Lewy body dementia
- Neuroborreliosis
- Machado-Joseph disease (Spinocerebellar ataxia type 3)
- Multiple System Atrophy
- Multiple sclerosis
- Narcolepsy
- Niemann Pick disease
- Parkinson's disease
- Pelizaeus-Merzbacher Disease
- Pick's disease
- Primary lateral sclerosis
- Prion diseases
- Progressive Supranuclear Palsy
- Refsum's disease
- Sandhoff disease
- Schilder's disease
- Subacute combined degeneration of spinal cord secondary to Pernicious Anaemia
- Spielmeyer-Vogt-Sjogren-Batten disease (also known as Batten disease)
- Spinocerebellar ataxia (multiple types with varying characteristics)
- Spinal muscular atrophy
- Steele-Richardson-Olszewski disease
- Tabes dorsalis
- Toxic encephalopathy
[edit] See also
- Trinucleotide repeat disorders
- Proteopathy
- Brain Age: Train Your Brain in Minutes a Day!
- Central nervous system
- Central nervous system disease
[edit] References
- ^ Astrid Sigel, Helmut Sigel and Roland K.O. Sigel, ed (2006). Neurodegenerative Diseases and Metal Ions. Metal Ions in Life Sciences. 1. Wiley. ISBN 978-0-470-01488-2.
- ^ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. pp. 1385. ISBN 0-7216-0187-1.
- ^ Whitton PS (April 2007). "Inflammation as a causative factor in the aetiology of Parkinson's disease". British Journal of Pharmacology 150 (8): 963–76. doi:. PMID 17339843.
- ^ Turrin NP, Rivest S (December 2006). "Molecular and cellular immune mediators of neuroprotection". Molecular Neurobiology 34 (3): 221–42. doi:. PMID 17308354.
- ^ Sierra A, Gottfried-Blackmore AC, McEwen BS, Bulloch K (March 2007). "Microglia derived from aging mice exhibit an altered inflammatory profile". Glia 55 (4): 412–24. doi:. PMID 17203473.
- ^ Segura-Aguilar J, Kostrzewa RM (December 2006). "Neurotoxins and neurotoxicity mechanisms. An overview". Neurotoxicity Research 10 (3-4): 263–87. doi:. PMID 17197375.
[edit] External links
- Institute for Neurodegenerative Diseases
- National Center for Biotechnology Information
- Harvard NeuroDiscovery Center
- HOPES: Huntington's Disease Outreach Program for Education at Stanford]
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