Quantification of the pathological changes in the temporal lobe of patients with a novel neurofilamentopathy: neurofilament inclusion disease (NID).

OBJECTIVE

To quantify the densities of neurofilament inclusions (NI), swollen achromatic neurons, surviving neurons and glial cells in a novel neurofilamentopathy neurofilament inclusion disease (NID).

MATERIAL: Sections of temporal lobe from 4 cases of NID stained with an antibody raised to neurofilament proteins.

METHOD: Densities of the pathological changes were estimated in the various gyri of the temporal lobe, hippocampus and dentate gyrus.

RESULTS

Densities of the NI and swollen achromatic neurons (SN) were greater in the cerebral cortical gyri than in the hippocampus and dentate gyrus. Lesion density was relatively constant between gyri and between the CA sectors of the hippocampus. In cortical gyri, the density of the NI, SN and glial cell nuclei was greater in laminae II/III than laminae V/VI. Densities of the NI were negatively correlated with the surviving neurons and positively correlated with the glial cell nuclei. The density of the SN was positively correlated with that of the surviving neurons.

CONCLUSION

The pathology of NID morphologically resembles that of Pick's disease (PD) and corticobasal degeneration (CBD), but there are distinct differences between NID and these disorders supporting the hypothesis that NID is a novel and unique type of neurodegenerative disease.