Vestibular Nuclei in Jacob-Creutzfeldt Disease

What is it about?

The morphological alterations of the vestibular disease in a case of Jacob-Creutzfeld disease were studied by light and electron microscope. The most prominent histological finding s were the neuronal degeneration neuronal loss, resulting in a marked decrease of the total population, the increased reactive astrocytosis which demonstrated an extensive perivascular arrangement, the microgliosis and the interstitial spongiosis. At the ultrastructural level a marked dilatation of the astrocytic processes was seen. Most of them were completely transformed to sizeable cysts which were divided by membranous structures into smaller spaces. A large number of glycogen granules was accumulated into astrocytes and microglial cells. The neurons demonstrated numerous alterations such as (a) large accumulation of lysosomes, (b) fragmentation of the microtubules, (c) dilatation of the cisternae of the smooth endoplasmic reticulum. (d) mitochondrial abnormalities, ( e ) tremendous loss of the dendritic spines.(f) dilatation of the axonal terminals. (g) decrease of the number of the synaptic vesicles in the presynaptic terminal. (h) dilatation of the synaptic cleft,dilatation and lysosomal accumulation in the postsynaptic component, ( k ) numerous multivesicular bodies and coated vesicles in pre- and postsynaptic terminals. In the neuropile space there were plenty of degenerated dendritic profiles. The degeneration of the vestibular nuclei of the brain stem was more extensive than the degeneration of the nuclei of the other brain stem cranial nerves.

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Why is it important?

We should have thought that the synaptic alterations being the most impressive finding would precede the other morphological changes on one hand and could provoke the extensive phenomena of the brain dysfunction on the other hand. The neuronal loss and the reactive astrocytosis occur, as the pathologic process of the disease advances. Although the most prominent phenomena from the clinical point of view in Jacob-Creutzfeldt disease are the manifestations of the cortical dysfunction, the vestibular alterations contribute considerably in the neuropathological and the clinical spectrum of that disease.

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