Mitsuyama variant in the association ALS-dementia

Mitsuyama variant in the association ALS-dementia. We report a case characterized by the association of an early-onset, rapidly progressive and severe dementia with the subsequent appearance of a motoneuron disease (bulbar ALS). The neuropsychological evaluation showed a global cognitive impairment, with a more profound impairment of executive, linguistic and mnestic functions, as in fronto-temporal dementia. SPECT and PET investigations demonstrated a bilateral reduction of blood perfusion and glucose metabolism in the frontal and temporal lobes. Brain MRI showed a diffuse cortical atrophy, more pronounced at the level of the frontal lobes. The characteristics of the present case are in agreement with the clinical variant first described by Mytsuyama in eastern patients. However, our case differs for the extremely rapid onset and progression of cognitive decline and this could suggest that the complexity of the association between fronto-temporal dementia and ALS is even more heterogeneous than reported up-to-date.