VALUES ^† : A National Multicenter Study of Regional and Gender Differences in Frontotemporal Disease in Amyotrophic Lateral Sclerosis

Abstract

SUMMARY Aims: To investigate regional and gender differences in prevalence rates and the pattern of cognitive and behavioral impairment in amyotrophic lateral sclerosis. Materials & methods: One hundred and ten subjects (55 male) from 14 amyotrophic lateral sclerosis clinics were cross-sectionally evaluated with the Penn State Brief Exam of frontal and temporal dysfunction syndromes. Results: Prevalence rates of cognitive impairment and behavioral impairment were statistically equivalent among rural, suburban and urban subgroups. Females evidenced significant strengths in fluency and limitations in configurational processing. Patterns of regional findings suggested greater frontal cortical involvement in the rural sample. Females demonstrated more bihemispheric involvement in comparison to more left hemispheric involvement for males. Conclusions: Regional prevalence differences in frontotemporal disease prodrome appear insignificant and multifactorial, while being consistent with the toxicity model implicating pesticides in frontal lobe change. Female gender potentially masks the frontotemporal disease prodrome due to a bilateral distribution of language processing, which requires an assessment of right hemisphere-mediated capacities to detect.

Gender Differences in Frontotemporal Lobar Degeneration (FTLD) Support an Estrogenic Model of Delayed Onset

“…fMRI studies of Turner Syndrome adults provided with estrogen replacement to allow for physical maturation have implicated the parietal, amygdala and prefrontal areas in this condition, in association with tasks of working memory, as well as interpretation of facial emotional expressions, and mediation of arousal by affective stimuli. Provision of estrogen replacement to stimulate developmental maturation thus has the indirect effect of mitigating many cognitive and behavioral abnormalities also characteristic of cognitive and behavioral declines seen in emerging FTLD in females [23,24,150].…”