neurodegenerative disease

(redirected from CNS Disorder)

neurodegenerative disease

Any of a group of hereditary and sporadic conditions characterised by progressive dysfunction, degeneration and death of specific populations of neurones which are often synaptically interconnected.

Neurodegenerative diseases
Akinetic-rigid
Examples
Parkinson’s disease, progressive supranuclear palsy, corticobasal degeneration, multisystem atrophy with striatonigral degeneration.

Clinical findings
Bradykinesia, rigidity.

Location
Substantia nigra, basal ganglia.

Hyperkinetic
Examples
Huntington’s disease, dentatorubropallidoluysial atrophy.

Clinical findings
Choreoathetosis, dystonia.

Location
Basal ganglia.

Ataxic
Examples
Friedrich’s ataxia, spinocerebellar degeneration, dentatorubropallidoluysial atrophy.

Clinical findings
Cerebellar ataxia.

Location
Cerebellum and connecting tracts.

Motor neuron disease
Examples
Amyotrophic lateral sclerosis, spinobulbar muscle atrophy (Kennedy’s disease), spinal muscular atrophy.

Clinical findings
Motor weakness.

Location
Motor system.

Temporoparietal degeneration
Examples
Alzheimer’s disease, dementia with Lewy bodies.

Clinical findings
Memory changes, parietal lobe dysfunction.

Location
Hippocampus, cortical neurons.

Frontotemporal degeneration
Example
Frontotemporal lobar degeneration.

Clinical findings
Apathy, disinhibition, memory changes.

Location
Frontal and temporal cortex.

Multifocal degeneration
Example
Prion disease.

Clinical findings
Variable cortical and subcortical changes.

Location
Cortical, subcortical neurons.

Neurodegenerative disease

A disease in which the nervous system progressively and irreversibly deteriorates.
Mentioned in: Amnesia
References in periodicals archive ?
AADC Deficiency is a rare CNS disorder that is caused by mutations in the dopa decarboxylase gene.
Comorbid conditions included convulsions, CNS disorder, organic brain syndrome, severe mental retardation, substance-related disorder, and congenital heart defects.
Second, a mild CSF lymphocytic pleocytosis is consistent with an infectious or inflammatory CNS disorder, though rare occurrences have been reported in pathologically confirmed Creutzfeldt-Jakob Disease (CJD) (12).
physicians typically look for a positive result from the somewhat unreliable blood-antibody test, coupled with the presence of telltale symptoms of Lyme disease (SN: 3/25/89,p.184), to infer that the Lyme-causing bacterium led to a CNS disorder, Halperin says.
AADC Deficiency is a rare CNS disorder arising from reductions in the enzyme AADC that result from mutations in the dopa decarboxylase gene.
Upon closing of the acquisition, PTC acquires GT-AADC, an adeno-associated virus (AAV) gene therapy that treats Aromatic L-Amino Acid Decarboxylase (AADC) Deficiency, a rare CNS disorder that is caused by mutations in the dopa decarboxylase (DDC) gene.
In mice, an age-related CNS disorder characterized by agitation, neophobia, seizures, inactivity, diminished cerebral glucose utilization, cortico-limbic gliosis, and death, develops.
is a biopharmaceutical company developing the xB3 platform, a proprietary technology for the delivery of therapeutics across the BBB and the treatment of CNS disorders in areas of high unmet medical need.
In the new role, Dr Everhart will help accelerate the application of Signant's intelligent eCOA, rater training, predictive analytics and other data quality monitoring products outside of CNS disorders.
Cell, gene and antisense therapies are not only innovative treatments for CNS disorders but also involve sophisticated delivery methods.