A Temporal Pole Lesion in Neuronal Intranuclear Inclusion Disease: A Case Report

Tomoya Inaba1,2, Yoshinori Hirata1, Hidehiro Ishikawa1

  • 1Department of Neurology, Mie Graduate School of Medicine, Japan.

An 81-year-old woman was transferred to our hospital due to a consciousness disorder. MRI of her cranium revealed white matter lesions and lesions in the cerebellar paravermis, splenium of the corpus callosum, and a left temporal pole on fluid-attenuated inversion recovery (FLAIR) sequences. Diffusion-weighted imaging (DWI) revealed hyperintensity at the left frontal corticomedullary junction of the brain. She was diagnosed with neuronal intranuclear inclusion disease (NIID) via a skin biopsy and genetic analysis. These lesions were evident before the DWI hyperintensity was clear. When a lesion in the temporal pole is observed, then NIID should be considered in the differential diagnosis.