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Information about Neurodegenerative Disease

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. Aside from a small number of neural stem cells that are created daily, cells of the brain and spinal cord are not readily regenerated en mass, so excessive damage can be devastating. Neurodegenerative diseases result from deterioration of neurons which over time will lead to neurodegeneration and disabilities resulting from this. They are crudely divided into two groups according to phenotypic effects, although these are not mutually exclusive:
  1. conditions causing problems with movements, such as ataxia
  2. conditions affecting memory and conditions related to dementia


Following Stanley Prusiner, some hypothesize that infectious proteins called prions are pathogens that result in the degeneration of the central nervous system. Prions are proteins that suffer post-translational modification(s) that change their shape so that they can no longer perform their cellular functions. Worse, they are hypothesized to trigger equivalent modifications in normal proteins, thus creating a cascade of damage that eventually results in significant neurodegeneration. In humans, the result is Creutzfeldt-JaKob Disease (Mad Cow Disease).

Neurodegenerative diseases can result from stroke, heat stress, head and spinal cord trauma (blunt or infectious pathology), and bleeding that occurs in the brain, the pressure from which eventually causes the death of one or more neurons.

Many times neuronal death begins long before the patient will ever experience any symptoms. It can be months or years before any effect is felt. Symptoms are noticed when lots of cells have died and certain parts of the brain have been weakened to the point that they can no longer function properly.

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.[1][2][3][4]

In recent studies it has been suggested that Monosodium Glutamate (MSG), a food additive, can result in neurodegenerative diseases. MSG appears to cause lesions at specific locations in the brain of laboratory animals as well as concomitant proliferations of neuroendocrine dysfunction. Glutamic acid, where MSG is a source of, has not been identified as a neurotransmitter which transmits impulses. These neurotransmitters develop specific types of neurodegenerative diseases.

List of neurodegenerative diseases

These diseases have their own characteristics which usually affect middle aged or older people. They usually worsen over time.

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.

Research is highly invested in stemcell technology and stem cell treatments, as well as Gene therapy.

Research is underway into Bio-Markers 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 majority of neural damage has already been suffered by the patient.

References

1. ^ Whitton PS.Inflammation as a causative factor in the aetiology of Parkinson's disease. Br J Pharmacol. 2007 Apr;150(8):963-76.
2. ^ Turrin NP, Rivest S. Molecular and cellular immune mediators of neuroprotection. Mol Neurobiol. 2006 Dec;34(3):221-42.
3. ^ Sierra A, Gottfried-Blackmore AC, McEwen BS, Bulloch K. Microglia derived from aging mice exhibit an altered inflammatory profile. Glia. 2007 Mar;55(4):412-24.
4. ^ Segura-Aguilar J, Kostrzewa RM. Neurotoxins and neurotoxicity mechanisms. An overview. Neurotox Res. 2006 Dec;10(3-4):263-87.

External links

See also

Proteopathy
disease is an abnormal condition of an organism that impairs bodily functions. In human beings, "disease" is often used more broadly to refer to any condition that causes discomfort, dysfunction, distress, social problems, and/or death to the person afflicted, or similar problems
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Greek}}} 
Writing system: Greek alphabet 
Official status
Official language of:  Greece
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recognised as minority language in parts of:
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Regulated by:
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Latin}}} 
Official status
Official language of: Vatican City
Used for official purposes, but not spoken in everyday speech
Regulated by: Opus Fundatum Latinitas
Roman Catholic Church
Language codes
ISO 639-1: la
ISO 639-2: lat
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Neurons (also known as neurones and nerve cells) are electrically excitable cells in the nervous system that process and transmit information. In vertebrate animals, neurons are the core components of the brain, spinal cord and peripheral nerves.
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Neurodegeneration is progressive loss of structure or function of neurons, including death of neurons.

Neurodegenerative diseases are the subset of neurological disorders that include neuron pathologies, but which exclude diseases of the nervous system due to cancer, edema,
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Ataxia From Greek αν (used as a negative prefix) + τάξις (order), meaning "lack of order". It is a neurological sign and symptom consisting of gross incoordination of muscle movements.
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In psychology, memory is an organism's ability to store, retain, and subsequently retrieve information. Traditional studies of memory began in the realms of philosophy, including techniques of artificially enhancing the memory.
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Dimentia
Classification & external resources

ICD-10 F 00. -F 07.
ICD-9 290 - 294

DiseasesDB 29283
MedlinePlus 000739

Dementia (from Latin de- "apart, away" + mens (genitive mentis
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Stanley Prusiner

Born May 28 1942 (1942--) (age 65)
Des Moines, Iowa, United States
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Prion Diseases (TSEs)
Classification & external resources

ICD-10 A81
ICD-9 046

A prion (IPA: /ˈpriːɒn/[1]
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Microglia are a type of glial cell that act as the immune cells of the Central nervous system (CNS). Microglia, the smallest of the glial cells, can act as phagocytes, cleaning up CNS debris. Most serve as representatives of the immune system in the brain and spinal cord.
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immune system is a collection of mechanisms within an organism that protects against disease by identifying and killing pathogens and tumor cells. It detects a wide variety of agents, from viruses to parasitic worms, and needs to distinguish them from the organism's own healthy
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Alexander disease
Classification & external resources

ICD-9 331.89

OMIM 203450

Alexander disease is a slowly progressing and fatal neurodegenerative disease.
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MeSH D002549 Alpers' disease, also called Alpers' syndrome,[1] progressive neuronal degeneration of childhood,<ref name="naude2004" /> progressive sclerosing poliodystrophy, and progressive infantile poliodystrophy
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Alzheimer's disease
Classification & external resources

Histopathologic image of senile plaques seen in the cerebral cortex in a patient with Alzheimer disease of presenile onset. Silver impregnation.
ICD-10 G 30. , F 00.
ICD-9 331.0 , 290.
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Amyotrophic lateral sclerosis
Classification & external resources

ICD-10 G 12.2
ICD-9 335.20

OMIM 105400
DiseasesDB 29148
MedlinePlus 000688
eMedicine neuro/14   emerg/24 pmr/10
MeSH D000690 Amyotrophic lateral sclerosis
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Batten disease
Classification & external resources

ICD-10 E 75.4
ICD-9 330.1

OMIM 204200
DiseasesDB 31534

MeSH D009472 Batten disease
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Bovine spongiform encephalopathy (BSE), commonly known as mad-cow disease, is a fatal, neurodegenerative disease of cattle, which infects by a mechanism that surprised biologists upon its discovery in the late 20th century.
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Canavan disease
Classification & external resources

ICD-9 330.0

OMIM 271900
DiseasesDB 29780
MedlinePlus 001586

Canavan disease is an inherited disorder that causes progressive damage to nerve cells in the brain.
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MeSH D003057 Cockayne syndrome (or "Weber-Cockayne syndrome", or "Neill-Dingwall Syndrome") is a rare, autosomal recessive disorder characterized by growth failure, impaired development of the nervous system, abnormal sensitivity to sunlight (photosensitivity), and premature
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Corticobasal degeneration (CBD) is a sporadic progressive neurodegenerative disease associated with atrophy of the cerebral cortex and the basal ganglia. Onset is gradual. The first symptoms usually involve asymmetric changes in motor function, such as dystonia and myoclonus.
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MeSH D007562

Creutzfeldt-Jakob disease (CJD) is a very rare and incurable degenerative neurological disorder (brain disease) that is ultimately fatal. Among the types of transmissible spongiform encephalopathy found in humans, it is the most common.
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MeSH D006816 Huntington's disease (HD), also misspelt as Huntington disease and known historically as Huntington's chorea and chorea maior
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AIDS dementia complex (ADC)
Classification & external resources

ICD-10 B 22. , F 02.4
ICD-9 042

AIDS dementia complex (ADC; also known as HIV dementia, HIV encephalopathy and HIV-associated dementia
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Kennedy's disease (KD) or X-linked spinal-bulbar muscular atrophy (SBMA) is a neuromuscular disease associated with mutations of the androgen receptor (AR). Because of its endocrine manifestations related to the impairment of the AR, it can be viewed as a variation of the
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Krabbe disease
Classification & external resources

ICD-10 E 75.2
ICD-9 330.0

OMIM 245200
DiseasesDB 29468

eMedicine ped/2892  
MeSH D007965 Krabbe disease (also known as globoid cell leukodystrophy or
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MeSH D020961 Dementia with Lewy bodies is the second most frequent cause of hospitalization for dementia, after Alzheimer's disease. Current estimates are that about 60-to-75% of diagnosed dementias are of the Alzheimer's and mixed (Alzheimer's and vascular dementia) type,
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MeSH D020754 Spinocerebellar ataxia (SCA) is a genetic disease with multiple types, each of which could be considered a disease in its own right.

Symptoms


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Multiple sclerosis
Classification & external resources

MRI FLAIR sequence showing four bright spots (plaques) where multiple sclerosis has damaged myelin in the
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