Temporal Changes in 18 F-FDG PET/CT in Familial Neuronal Intranuclear Inclusion Disease, Based on the Clinical Course.

We report temporal changes in 18 F-FDG PET/CT in neuronal intranuclear inclusion disease (NIID). In a patient with encephalitis-like episodes, PET showed hypermetabolism correlating with EEG alterations. Affected sites later became hypometabolic and showed diffusion changes on MRI. In another patient, hypermetabolic regions correlated well with the EEG, whereas MRI showed changes after only 1 month. One chronic patient had diffuse hypometabolism, which correlated with atrophy on MRI and cerebral dysfunction on EEG. This is the first report of temporal changes in PET in NIID and suggests that it reflects the disease activity of NIID while correlating well with EEG.