Frontotemporal dementia and amyotrophic lateral sclerosis: Revisiting one of the first case reports with neuropathological examination

Demência frontotemporal e esclerose lateral amiotrófica: retornando a um dos primeiros relatos de caso com exame histopatológico

ABSTRACT

The occurrence of dementia in amyotrophic lateral sclerosis (ALS) was only widely recognized in the late 20^th century. Hitherto, it was believed that dementia was a rare event due to the fortuitous association with other diseases. In 1924, Kostantin Nikolaevich Tretiakoff and Moacyr de Freitas Amorim reported a case of dementia with features of frontotemporal dementia (FTD) that preceded the motor signs of ALS. Neuropathological examination confirmed ALS and found no signs of other dementia-causing diseases. The authors hypothesized that dementia was part of ALS and recommended the search for signs of involvement of motor neurons in cases of dementia with an ill-defined clinical picture, a practice currently accepted in the investigation of cases of FTD. This was one of the first descriptions of dementia preceding the motor impairments of ALS and was published in Portuguese and French in Memórias do Hospício de Juquery.

Key words:
dementia; frontotemporal dementia; amyotrophic lateral sclerosis; neuropathology; motor neuron disease