小林 良太

Few studies have longitudinally evaluated Hashimoto's encephalopathy with anti-NH2-terminal α-enolase (anti-NAE) antibodies using detailed imaging and neuropsychological assessments. We present the case of a man in his 50s who presented with acute hallucinations, catatonia, seizures, and cognitive decline. Initial MRI revealed diffuse white matter hyperintensities, and SPECT revealed widespread hypoperfusion. These symptoms improved with immunotherapy, but progressive frontal and temporal atrophy and residual hypoperfusion appeared over 33 months. His cognitive function improved, but he remained impaired, with persistent disinhibition and perseveration. This case suggests that Hashimoto's encephalopathy with anti-NAE antibodies can cause lasting structural and functional brain abnormalities and cognitive impairments, requiring long-term neuroimaging and neuropsychological follow-up.

Frontotemporal lobar degeneration (FTLD) encompasses frontotemporal dementia and related neurological disorders including motor neuron disease and movement disorders. During the 21th century, analyses of aggregative proteins suggested powerful hypotheses of gain-of-neurotoxicity or loss-of-function for aggregation-related proteins. However, recent translational researches in collaboration of basic studies and human pathology indicate that FTLD arises from more complex molecular mechanisms than dyshomeostasis of single molecules. Additionally, accumulation of clinicopathological evidences from various countries, genetic backgrounds or clinical specialties (e.g. neurology and psychiatry), suggests diverse phenotypes of FTLD, which are indicative of future paradigm-shift in the concept of FTLD. In this paper, we discuss FTLD pathomechanism on the basis of human pathology.