A long surviving case of amyotrophic lateral sclerosis with atrophy of the frontal lobe: a comparison with the Mitsuyama type.

A female patient with amyotrophic lateral sclerosis (ALS) showing psychiatric symptoms during her last 2 years of life is reported. Although pseudobulbar signs were seen at the onset of ALS and no respirator was used, the period from onset to death was rather long (9 years). The spinal lesions showed features common to ordinary ALS, while the marked atrophy with destructive changes throughout the frontal lobe seemed to be considerably more severe than that seen with either ordinary ALS or the Mitsuyama type of ALS. Since the clinical manifestations and histological characteristics are apparently different from those of the Mitsuyama type, our case may be a new nosological variant of ALS with psychiatric manifestations.