neurodegenerative disease

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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
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