Dementias Specific Diseases

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ALZHEIMER'S DISEASE

This is the commonest cause of dementia with an estimated half million sufferers in the UK. The disorder rarely occurs under the age of 45 years. The incidence increases with age. Up to 30% of cases are familial.

Pathology

(i) Neuritic plaque: a complex extracellular __ lesion of 15-100(tm. Aggregates of filaments . with a central core of amyloid. Found in the hippocampus "and parietal lobes tf3C

(ii) Neurofibrillary tangle: an intracellular lesion. Paired helical strands of protein close to nuclei of neurons. Mainly affecting pyramidal cells of cortex

These lesions are associated with neuronal loss and granulovacuolar degeneration

The brain is small with atrophy most evident in the superior and middle temporal gyri.

Subcortical origins of cholinergic projections are also involved.

(ii) Neurofibrillary tangle: an intracellular lesion. Paired helical strands of protein close to nuclei of neurons. Mainly affecting pyramidal cells of cortex

Diagnosis may be established during life by the early memory failure and slow progression and by excluding other causes.

Recent claims that (3 amyloid deposition also occurs in skin and intestine raises hopes of biopsy as a diagnostic marker.

CT scanning: aids diagnosis by excluding multiple infarction or a mass lesion.

(Through ventricular system)

Large basal cisterns and large Sylvian fissure

Shrunken walnut appearance to moderate ventricular enlargement

Enlarged cortical sulci

(High 'cut' over cortex)

Large basal cisterns and large Sylvian fissure

(Through ventricular system)

Causation

The cause of Alzheimer's disease is not known. An association with Down's syndrome suggests a disease locus on chromosome 21 (on which amyloid precursor protein is coded), and this has been confirmed in familial cases. The role of environmental toxins, especially aluminium, is uncertain. Early research suggested selective lesions of neurotransmitter pathways occurred and a disorder of cholinergic innervation was postulated. It is now-known that many neurotransmitter pathways are defective. Treatment

No effective treatment exists. Transmitter augmentation therapy seems unlikely to be effective in view of the many neurotransmitters involved.

Shrunken walnut appearance

Enlarged cortical sulci to moderate ventricular enlargement

(High 'cut' over cortex)

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Responses

  • leon
    What neurodegenerative disease can cause enlargement of the sulcus and sylvian fissure?
    8 years ago

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