neuronal intermediate filament inclusion disease

Neuronal intermediate filament inclusion disease

Neuronal intermediate filament inclusion disease is a rare distinct variant, having inclusion bodies that are cytoplasmic and made up of type IV intermediate filaments. Neuronal intermediate filament inclusion disease has an early age of onset between 23 and 56. Symptoms can include behavioral and personality changes, memory and cognitive impairments, language difficulties, motor weakness, and extrapyramidal symptoms. It is one of the frontotemporal lobar degeneration–FUSproteopathies. Imaging commonly shows atrophy in the frontotemporal region, and in part of the striatum in the basal ganglia. Post-mortem studies show a marked reduction in the caudate nucleus of the striatum; frontotemporal gyri are narrowed, with widened intervening sulci, and the lateral ventricles are enlarged.