Neurodegenerative disease definition | List of neurodegenerative disease

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What is Neurodegenerative disease definition?

Neurodegenerative disease definition is an umbrella term for a range of conditions that primarily affect the neurons in the human brain.

Neurodegenerative diseases mainly strike people aged 65 and over. They are characterized by the progressive loss of neurons in more or less localized regions of the nervous system. Leading to the cognitive, motor, or perceptual complications. Ultimately, they can lead to death.

Their origin is not always precisely known while genetics seem to be involved in some cases. Most of the time they occur in isolation.

Chemicals (pesticides, heavy metals) or prions have also been identified as culprits in some patients however, there are other causes for the onset of the disease. But these have not been clearly established.

Some examples of neurodegenerative disease definition

List of neurodegenerative disease definition

Multiple system atrophy: neurodegenerative disease definition

System atrophy (AMS) is a group of neurodegenerative diseases sporadic, affecting several brain functions.

Spinocerebellar ataxia: 

The ataxia spinocerebellar are a group of neurodegenerative diseases clinically heterogeneous from the point of view that genetic and the outcome is often fatal. They are characterized by cerebellar syndrome which results in particular in walking and balance disorders.

These disorders are the consequence of the degeneration of the cerebellum. And more or less of its afferent and references Often. People are unaware that they carry the responsible gene until they have children.

Who begin to show clinical signs of the disease. Many forms of spinocerebellar ataxia remain little known, and studies are underway to better characterize them. This disease is still incurable (no treatment).

Alexander disease: neurodegenerative disease definition

The Alexander disease is a rare disease of the white matter of the brain. It occurs most often in the first years of life (infantile form). Sometimes later (juvenile form) but neonatal or adult forms exist.

Posterior cortical atrophy: 

Posterior cortical atrophy (PCA). Also called Benson syndrome is a rare and progressive neurodegenerative disease. Appearing mainly between the ages of 50 and 65 years.

It is a form of dementia sometimes considered an early atypical variant of Alzheimer’s disease. The causes or risk factors for this disease are unknown. Dr. Frank Benson first described this syndrome in 1988.

Alzheimer’s disease: neurodegenerative disease definition

Alzheimer’s disease is a neurodegenerative disease (progressive loss of neurons) incurable to date the fabric brain. Which causes progressive and irreversible loss of mental functions including memory. It is the record public effect of dementia in humans. It was originally defined by the German specialist and Doctor Alois Alzheimer in 1906.

The first symptom is often loss of memories (amnesia). Initially manifesting as minor distractions, which become more pronounced as the disease progresses. It is primarily short-term memory that is affected older memories are relatively well preserved, however.

Neurological damage subsequently extends to the frontal and tempura-parietal associative cortices. Resulting in more severe cognitive disorders (confusion, irritability, aggressiveness, mood and emotional disorders, executive and language functions). And long-term memory loss. The destruction of neurons continues until the loss of autonomic functions and death 2.

Neurodegenerative disease definition

Two types of nervous tissue damage characterize Alzheimer’s disease: senile plaques (or amyloid deposits) and neurofibrillary degeneration. The parts of these lesions are separately the amyloid peptide and the Tau protein. The causes of the aggregation of these proteins in amyloid deposits.

And neurofibrillary degeneration is still unknown but genetic and environmental factors contribute to their appearance.

The first risk factor is age, but there are also genetic risk factors with mutations in the amyloid peptide precursor gene or protein genes (presenilins ) involved in its genesis). Cardiovascular risk factors or poisoning by certain heavy metals or drugs.

neurodegenerative disease definition

The diagnosis of Alzheimer’s disease is essentially based on questioning, neuropsychological and psychomotor tests.  And on the demonstration of cortical atrophy which first affects the internal temporal lobe and in particular the hippocampus, regions important for memory.

The first signs of Alzheimer’s disease are often mistaken for the normal aspects of senescence. Depression, stress, or other neurological conditions such as vascular dementia. She was thus underdiagnosed even in the 1960s. The diagnosis improved with biological tests in cerebrospinal fluid and neuroimaging.

The speed and course of the disease vary depending on the individual, which makes any prognosis difficult.  Especially of life expectancy (from 3 to 8 years depending on the patient’s age at the time of diagnosis).

neurodegenerative disease definition

There is currently no cure for this disease. The treatments aim to limit its progression. The care offered is mainly palliative. Cognitive stimulation, exercise, and a balanced diet may delay the onset of cognitive impairment in older people 5 . Because Alzheimer’s disease is degenerative, the patient relies on others for help.

The role of the primary caregiver is essential Up to 70% of those affected suffer from psychological distress, defined as anxiety, depression and which can disrupt their daily functioning, decrease their quality of life and accelerate their cognitive decline.

In 2015, there were approximately 48 million people worldwide with Alzheimer’s disease 19. Most often, the disease begins in people over 65 years of age; only 4% to 5% of early Alzheimer’s cases start before this age 20. About 6% of people aged 65 and over are affected, but these figures differ by country.

In 2010, dementia caused by the disease caused an estimated 486,000 deaths worldwide 21. In developed countries, it is one of the most costly diseases for society 22, 23.

Faced with the prevalence of the disease, medical research efforts are aimed at developing drugs capable of stopping the neurodegenerative process. The main avenues are to attack the amyloid plaques that form between neurons during disease. And the aggregates of tau proteins that form neurofibrillary degeneration inside neurons 24.

Creutzfeldt-Jakob disease:

The Creutzfeldt-Jakob disease (CJD) is a degeneration of the central nervous system characterized by the accumulation of a prion (an abnormal form of a protein that can transmit the disease).

The incubation period is years or even decades before balance and sensitivity disorders appear, followed by dementia. The outcome is systematically fatal after approximately one year.

Lewy body dementia: neurodegenerative disease definition

The body disease Lewy ( MCL ) is also known as dementia with Lewy bodies.  DLB  is a type of dementia that shares characteristics with Alzheimer’s disease and Parkinson’s disease.

More specifically, it is Alzheimer’s disease and Parkinson’s disease that share characteristics with MCL, making it difficult to distinguish the diagnostic between the latter two diseases. A patient with Parkinson’s disease or Parkinson’s syndrome can also develop MCL in parallel (risk four times greater in this case).

MCL is manifested by progressive and fluctuating cognitive disorders associated with visual hallucinations and extrapyramidal syndrome.

Neurodegenerative disease definition

Of all the dementias listed in the medical profession, including those including hallucinations encountered in paranoid or schizophrenic delusions, MCL is the heaviest and the most severe. It also often combines the characteristics and manifestations of all other dementias.

It is also accompanied by many psychomotor disorders, and as with Parkinson’s disease or parkinsonian syndromes, patients are affected by dystonia as well as dysautonomia ( tachycardia, orthostatic hypotension, muscular hypertonia,  etc. ).

While the name of this disease draws attention to dementia, it is not the only manifestation of it. This disease is polymorphic and covers a number of bodily dysfunctions.

MCL is anatomically characterized by the presence of Lowy bodies, made up of clusters of alpha-syncline and ubiquitin, in neurons in the brain. Treatment is only symptomatic.

Parkinson disease: 

Parkinson’s disease as described by James Parkinson in 1817, is a chronic degenerative neurological disease (progressive loss of neurons) affecting the central nervous system responsible for progressive disorders: slow movements, shaking, stiffness, and cognitive disorders. It is another record communal neurodegenerative disorder 1.


Its causes are poorly understood. The clinical picture is the consequence of the loss of neurons of the Niger locus (or “black substance”) and of an attack of the Nigro-striated bundles (in). The sickness generally brings into being among the ages of 45 and 70.

It is the second most common neurodegenerative disease, after Alzheimer’s disease. Parkinson’s disease is distinguished from parkinsonian syndromes which are generally of various origins, more severe, and respond poorly to treatment.

Huntington’s disease: Neurodegenerative disease definition

Huntington’s disease (sometimes called Huntington’s disease) is an inherited disease and rarely, resulting in neurological degeneration causing major motor, cognitive and psychiatric, and progressing to loss of independence and death 1. Several treatment avenues are currently being tested.

The disease develops in people who are on average 40 to 50 years old. More rarely, it manifests itself in an early form with the appearance of the first symptoms between 15 and 25 years. We can classify these symptoms into three main families.

The latter, which are not all present in all patients, differ considerably depending on the case; some may be very discreet in some (or even completely absent) and more flamboyant in others:

Motor symptoms (irrepressible movements of the “chorea  ” type, balance disorders, difficulties with the pronator system, in particular speech and swallowing disorders);

cognitive symptoms (memory problems, difficulties in organizing multiple tasks, in manipulating acquired knowledge, slowing down of information processing, deterioration of cognitive faculties leading to dementia syndrome of the subcortical type);

Psychiatric symptoms (very wide variety of possible disorders: anxiety, depression, dis-inhibiting, aggressiveness, agitation).

Pick’s disease:

The Pick disease is dementia associated with deterioration of the lobes frontal and temporal brain. It is a proteinopathy of the front temporal lobar degeneration family.

Macrophage myofasciitis: Neurodegenerative disease definition

The myofasciitis macrophage (MFM) is a tissue entity characterized by muscle damage infiltrated by macrophages. These lesions are visualized by analyzing muscle tissue under a microscope during a muscle biopsy.

MFM is responsible for a flu-like syndrome: muscle and joint pain, fatigue, and a slight fever. Although it is not established that this histologist entity is associated with any clinical syndrome. some associate it with varying symptoms.

Progressive supranuclear palsy: definition

The progressive supranuclear palsy (PSP or disease of Steele-Richardson-Olszewski. The names of doctors who characterized in 1963) is a neurodegenerative disease due to the progressive destruction of neurons from various regions of the brain. The striatum, the formation cross-linked the brain stem, the Niger locus, the nuclei of the cranial nerves.

These lesions cause in particular difficulty in performing vertical eye saccades (supranuclear ophthalmologist) characteristic of PSP. But they also gradually affect the balance, sight, walking, swallowing, speaking, and more generally, movements performed in the sagittal plane.

PSP affects around six in 100,000 people, both men, and women. Without being able to identify any risk factor linked to ethnicity, living environment, or professional activity. Some of the symptoms of PSP are similar to those of front temporal degeneration, Parkinson’s disease, or multisystem atrophy.

But it is distinguished from it by coulometer damage. The pathological mechanisms of PSP remain poorly understood. It is sometimes described as tauopathy because neurofibrillary degeneration is associated with an accumulation of Tau protein in nerve cells.

 Amyotrophic lateral sclerosis: Neurodegenerative disease definition

Amyotrophic lateral sclerosis or ALS, also known in the francophone world Gehrig’s disease (Lou Gehrig’s disease in North America) is a neurodegenerative disease of the motor neurons of the adult. It is characterized by progressive degeneration of the motor neurons of the cerebral cortex with consequent destruction of the pyramidal tract (attack of the first motor neuron).

And of those of the anterior horn of the spinal cord with the destruction of the motor units. Associated (involvement of the second motor neuron). It causes progressive paralysis of the entire skeletal musculature of the limbs, the trunk (including the respiratory muscles), and the cephalic extremity.

The causes of ALS are considered to be multi-factorial. It affects both sexes and its incidence increases with age from 40 years.

In the United States and Canada, it is also known as “Lou Gehrig’s disease”. After a famous baseball player died of this disease in 1941. It should not be confused with a disease of similar name and symptoms. But of less serious course char-cot-Marie-Tooth disease.

Wolfram syndrome:

The Wolfram syndrome also known by its acronym English DIDMOAD (for ”  diabetes insipid us, diabetes mellitus, optic atrophy, and deafness  ” ) is a syndrome rare belonging to the group called orphan diseases. Which associates a type 1 diabetes, diabetes insipid us, severe vision impairment ( optic atrophy ), and neurological disorders.

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