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Neurodegenerative disease
 
 
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Neurodegenerative disease
Disease

A disease or medical condition is an abnormal condition of an organism that impairs bodily functions, associated with specific symptoms and Medical signs....
 (Greek
Greek language

Greek is an Indo-European languages native to the southern Balkan peninsula, the language of the Greek people. It forms an independent branch within Indo-European....
 ?????-, néuro-, "nerval" and Latin
Latin

Latin is an Italic language, historically spoken in Latium and Ancient Rome. Through the Military history of the Roman Empire, Latin spread throughout the Mediterranean and a large part of Europe....
 degenerare, "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.






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Neurodegenerative disease
Disease

A disease or medical condition is an abnormal condition of an organism that impairs bodily functions, associated with specific symptoms and Medical signs....
 (Greek
Greek language

Greek is an Indo-European languages native to the southern Balkan peninsula, the language of the Greek people. It forms an independent branch within Indo-European....
 ?????-, néuro-, "nerval" and Latin
Latin

Latin is an Italic language, historically spoken in Latium and Ancient Rome. Through the Military history of the Roman Empire, Latin spread throughout the Mediterranean and a large part of Europe....
 degenerare, "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 neuron
Neuron

Neurons are responsive cell in the nervous system that process and transmit information by electrochemical Signal . They are the core components of the brain, the vertebrate spinal cord, the invertebrate ventral nerve cord, and the peripheral nerves....
s or their myelin sheath which over time will lead to dysfunction and disabilities resulting from this. 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
    Ataxia

    Ataxia is a neurology sign and symptom consisting of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of parts of the nervous system that coordinate movement, such as the cerebellum....
    , and
  • conditions affecting memory
    Memory

    In psychology, memory is an organism's mental ability to store, retain and recall information. Traditional studies of memory began in the fields of philosophy, including techniques of mnemonic....
     and related to dementia
    Dementia

    Dementia is the progressive decline in cognition due to damage or disease in the body beyond what might be expected from normal aging. Although dementia is far more common in the geriatric population, it may occur in any stage of adulthood....
    .


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
Creutzfeldt-Jakob disease

Creutzfeldt–Jakob disease is a very rare and incurable degeneration neurology that is fatal. Among the types of transmissible spongiform encephalopathy found in humans, it is the most common....
 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. Symptoms are noticed when many cells die or cease to function and a part of the brain begins to fail.

Regulation, or production of microglia
Microglia

Microglia are a type of glial cell that acts as the first and main form of active immune defense in the central nervous system . Microglia constitute 20% of the total glial cell population within the brain....
 by the immune system
Immune system

An immune system is a collection of biological processes within an organism that protects against disease by identifying and killing pathogens and tumour cells....
, in a process of neuroinflammation, is currently being rigorously studied for its role in neurodegenerative diseases.

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.

Epidemiology

Each specific kind of neurodegenerative disease has its own characteristics, but most affect middle-aged or older people. They usually worsen over time.

Research directions

Research is highly invested in stem cell
Stem cell

Stem cells are Cell found in most, if not all, multi-cellular organisms. They are characterized by the ability to renew themselves through Mitosis cell division and Cellular differentiation into a diverse range of specialized cell types....
 technology and stem cell treatments
Stem cell treatments

Stem cell treatments are a type of cell therapy that introduce new cells into damaged tissue in order to treat a disease or injury. Many medical researchers believe that stem cell treatments have the potential to change the face of human disease and alleviate suffering....
, as well as Gene therapy
Gene therapy

Gene therapy is the insertion of genes into an individual's cell and Biological tissues to treat a disease, such as a hereditary disease in which a deleterious mutant allele is replaced with a functional one....
.

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.

List of neurodegenerative diseases

  • Adrenal Leukodystrophy (ALD)
  • Alcoholism
    Alcoholism

    Alcoholism is a term with multiple and sometimes conflicting definitions to describe the detrimental effects of alcohol intake.In common and historic usage, alcoholism refers to any condition that results in the continued consumption of alcoholic beverages despite health problems and negative social consequences....
  • Alexander's disease
  • Alper's disease
  • Alzheimer's disease
    Alzheimer's disease

    Alzheimer's disease , also called Alzheimer disease, Senile Dementia of the Alzheimer Type or simply Alzheimer's, is the most common form of dementia....
  • Amyotrophic lateral sclerosis
    Amyotrophic lateral sclerosis

    Amyotrophic Lateral Sclerosis is a progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement....
     (Lou Gehrig's Disease)
  • Ataxia telangiectasia
    Ataxia telangiectasia

    Ataxia-telangiectasia is a rare, neurodegenerative, inherited disease which affects many parts of the body and causes severe disability. Ataxia refers to poor coordination and telangiectasia to small dilated blood vessels, both of which are hallmarks of the disease....
  • Batten disease
    Batten disease

    Batten disease is a rare, fatal autosomal recessive neurodegenerative disorder that begins in childhood. Also known as Spielmeyer-Vogt-Sj?gren-Batten disease, it is the most common form of a group of disorders called neuronal ceroid lipofuscinosis ....
     (also known as Spielmeyer-Vogt-Sjögren-Batten disease)
  • Bovine spongiform encephalopathy
    Bovine spongiform encephalopathy

    Bovine Spongiform Encephalopathy , commonly known as Mad-Cow Disease , is a fatal, neurodegenerative disease in cattle, that causes a spongy degeneration in the brain and spinal cord....
     (BSE)
  • Canavan disease
    Canavan disease

    Canavan disease, also called Canavan-Van Bogaert-Bertrand disease, aspartoacylase deficiency or aminoacylase 2 deficiency, is an autosome dominance degenerative disorder that causes progressive damage to nerve cells in the brain....
  • Cerebral palsy
    Cerebral palsy

    Cerebral palsy is an umbrella term encompassing a group of non-progressive illness, non-Infectious diseases conditions that cause physical disability in Human development ....
  • Cockayne syndrome
    Cockayne syndrome

    Cockayne syndrome is a rare autosomal recessive congenital disorder characterized by growth failure, impaired development of the nervous system, abnormal sensitivity to sunlight , and premature aging....
  • Corticobasal degeneration
    Corticobasal degeneration

    Corticobasal degeneration is a rare progressive neurodegenerative disease characterized by marked disorders in movement and cognitive dysfunction....
  • Creutzfeldt-Jakob disease
    Creutzfeldt-Jakob disease

    Creutzfeldt–Jakob disease is a very rare and incurable degeneration neurology that is fatal. Among the types of transmissible spongiform encephalopathy found in humans, it is the most common....
  • Frontotemporal lobar degeneration
    Frontotemporal lobar degeneration

    Frontotemporal lobar degeneration is the name for a group of clinically, pathologically and genetically heterogeneous disorders associated with atrophy in the frontal lobe and temporal lobe of the brain, with sparing of the parietal lobe and occipital lobes....
  • Huntington's disease
    Huntington's disease

    Huntington's disease, also called Huntington's Chorea , chorea major, or HD, is a genetics Neurodegenerative disease characterized after onset by uncoordinated, jerky body movements and a decline in some mental abilities....
  • HIV-associated dementia
  • Kennedy's disease
  • Krabbe's disease
  • Lewy body dementia
  • Neuroborreliosis
    Lyme disease

    Lyme disease, or borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia....
  • Machado-Joseph disease
    Machado-Joseph disease

    Machado-Joseph disease is a type of spinocerebellar ataxia that causes ophthalmoplegia and mixed sensory and cerebellar ataxia.Based on DNA studies, MJD is the most widespread, autosomal dominant inherited form of ataxia in the world....
     (Spinocerebellar ataxia
    Spinocerebellar ataxia

    Spinocerebellar ataxia is a genetic disease with multiple types, each of which could be considered a disease in its own right....
     type 3)
  • Multiple System Atrophy
    Multiple system atrophy

    Multiple system atrophy is a rare disease, degenerative neurological disorder. MSA is associated with the degeneration of nerve cells in specific areas of the brain....
  • Multiple sclerosis
    Multiple sclerosis

    Multiple sclerosis is an autoimmune disease in which the immune system attacks the central nervous system, leading to demyelinating disease. Disease onset usually occurs in young adults, and it is more common in females....
  • Narcolepsy
    Narcolepsy

    Narcolepsy is a chronic sleep disorder characterized by overwhelming daytime drowsiness and sudden attacks of sleep. The condition is most characterized by excessive daytime sleepiness , in which a person experiences extreme tiredness and possibly falls asleep during the day at inappropriate times, such as at work or school....
  • Niemann Pick disease
  • Parkinson's disease
    Parkinson's disease

    Parkinson's disease is a degenerative disorder of the central nervous system that often impairs the sufferer's motor skills and speech, as well as other functions....
  • Pelizaeus-Merzbacher Disease
    Pelizaeus-Merzbacher disease

    Pelizaeus-Merzbacher disease is a rare central nervous system disorder in which coordination, motor abilities, and intellectual function are delayed to variable extents....
  • Pick's disease
    Pick's disease

    Pick's disease, also known as Pick disease and PiD, is a rare neurodegenerative disease. While the term Pick's disease was once used to represent a specific group of clinical syndromes with symptoms attributable to frontal and temporal lobe dysfunction, it is now used to mean a specific pathology that is just one of the causes of...
  • Primary lateral sclerosis
    Primary lateral sclerosis

    Primary lateral sclerosis is a rare neuromuscular disease characterized by progressive muscle weakness in the voluntary muscles. PLS belongs to a group of disorders known as motor neuron diseases....
  • Prion diseases
  • Progressive Supranuclear Palsy
    Progressive supranuclear palsy

    Progressive supranuclear palsy is a rare degenerative disease involving the gradual deterioration and death of selected areas of the brain.The sexes are affected approximately equally and there is no racial, geographical or occupational predilection....
  • Refsum's disease
    Refsum's disease

    Refsum's disease , named after Norwegian neurologist Sigvald Bernhard Refsum , is neurological disease that results in the malformation of myelin sheaths around neuron....
  • Sandhoff disease
    Sandhoff disease

    Sandhoff disease, also called Hexosaminidase A and B deficiency, is an autosomal recessive lysosomal storage disorder that affects lipid storage and causes progressive destruction of the central nervous system....
  • Schilder's disease
    Schilder's disease

    Schilder's disease or diffuse myelinoclastic sclerosis is a very infrequent neurodegenerative disease that presents clinically as tumor Multiple sclerosis lesions, that make its diagnosis difficult....
  • Subacute combined degeneration of spinal cord
    Subacute combined degeneration of spinal cord

    Subacute combined degeneration of spinal cord, also known as Ludwig Lichtheim's disease, refers to degeneration of the posterior and lateral columns of the spinal cord as a result of vitamin B12 deficiency....
     secondary to Pernicious Anaemia
  • Spielmeyer-Vogt-Sjogren-Batten disease
    Batten disease

    Batten disease is a rare, fatal autosomal recessive neurodegenerative disorder that begins in childhood. Also known as Spielmeyer-Vogt-Sj?gren-Batten disease, it is the most common form of a group of disorders called neuronal ceroid lipofuscinosis ....
     (also known as Batten disease)
  • Spinocerebellar ataxia
    Spinocerebellar ataxia

    Spinocerebellar ataxia is a genetic disease with multiple types, each of which could be considered a disease in its own right....
     (multiple types with varying characteristics)
  • Spinal muscular atrophy
    Spinal muscular atrophy

    Spinal Muscular Atrophy is a term applied to a number of different disorders, all having in common a Genetics cause and the manifestation of weakness due to loss of the motor neurons of the spinal cord and brainstem....
  • Steele-Richardson-Olszewski disease
  • Tabes dorsalis
    Tabes dorsalis

    Tabes dorsalis is a slow degeneration of the nerve cells and nerve fibers that carry sensory information to the brain. The degenerating nerves are in the dorsal columns of the spinal cord and carry information that help maintain a person's sense of position, vibration, and discriminative touch....


External links



See also

  • Trinucleotide repeat disorders
    Trinucleotide repeat disorders

    Trinucleotide repeat disorders are a set of genetic disorders caused by wikt:trinucleotide repeats in certain genes exceeding the normal, stable, threshold, which differs per gene....
  • Proteopathy
    Proteopathy

    Proteopathy is the abnormal accumulation and toxicity of proteins in certain disease states. The proteopathies comprise more than 30 diseases that affect a variety of organs and tissues, including Alzheimer's disease, Parkinson's disease, type 2 diabetes, amyloidosis, selective hyperproteolytic diseases , and a wide range of other disorde...