neuropathologic classifiaction of dementias

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교과서를 정리한 내용입니다. (overview)

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Introduction
Unspecific lesions
2. Synaptic loss
3. Laminar spongiosis and astrocytosis
Inclusions and deposits
Tauopathies
1. Different types of tau accumulation
α-Synuclein accumulation

본문내용

Introduction
Dx. of dementia : topography, nature of the lesions
Anatomy of neuronal loss, depends on the identification of inclusion bodies
Loss of neurons, synpses, astrocytosis
non-specific lesions
Accumulation of proteins in the cells or in the extracellular space
most specific signs
Unspecific lesions
1. Neuronal loss : topography of neuronal loss
Huntington’s disease : neuronal loss in the head of the caudate nucleus
Neuronal loss : limited, unspecific, final comman pathway
Mechanism of neuronal death apoptosis in AD
n AD 1/1000 on a slide, not higher
1/1100 1/5000 apoptotic neurons, compatible with survival of the patients
Quantitative evaluation of neuronal loss is difficult
1. density of neuronal profiles is modified by size of cells, overestimated
Atophy density in slide decreases still alive, being smaller, less numerous on microscope
2. masked by atrophy of cortex or of nucleus
structure is smaller
These difficulties explain why a remarkably small proportion of dementing disorders have been subjected to a quantitative analysis of the neuronal loss which occurs during their course.




Cortical neurons or induce leukodystrophy
Mutations of two genes(NPC1, NPC2) cause Niemann-Pick type C disease(NPCD)
Progressive dementia, vertical supranuclear ophthalmoplegia, asphasia, ataxia
Hepato-splenomegaly, storage cell in bone marrow, occuring at younger age
lesterol esterification is low in fibroblast, accumulate free cholesterol
Frontal lobe atrophy, with NPC2 mutation
PAS-positive material pushes nucleus to the periphery
Tau-positive neurites, neurofibrillarytween cholesterol metabolism and tangle formation
Cognitive dysfunction in GM2 gangliosidosis due to mutation in beta subunit of hexosaminidase, ataxia, dystonia, choreoathetosis, peripheral neuropathy
Ballooned neurons, abundant in basal ganglia, luxol fast blue, PAS, sudan black positive
Ceroid lipofuscinosis(Kufs disease), AR, accumulation of lipofuscin, autofluorescent and insoluble lipopigment

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