Amyotrophic lateral sclerosis: A hormonal condition?

Abstract

Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disorder in adults. This fatal condition, due to degeneration of upper and lower motor neurons in spinal and bulbar myotomes, leads to death from respiratory failure after median disease duration of 36 months. ALS is sporadic in more than 90% of cases and familial in the remaining cases. Most studies show male predominance with a gender ratio of 3:2, but gender differences are age related. The phenotype of ALS is also different in males and females with a predominance of limb onset in males and bulbar onset in females. While age and site of onset impact survival rate, and are both related to gender, gender by itself has not clearly been shown to have an effect on survival. Given this complex relationship between gender and ALS, we developed a hypothesis about hormone involvement in ALS aetiology by suggesting protective effect of oestrogens and adverse effect of androgens.

Clinical, demographic and prognostic features of sporadic amyotrophic lateral sclerosis in Northern Turkey

“…The differences between males and females in the ability to repair damage could be relevant to possible differences in the clinical course of ALS. Moreover, the neuroprotective effect of estrogen in females and the probable toxic effects of androgens have been mentioned; late onset of the disease in women and convergence of disease ratios around the age of 80 were associated with the elimination of the protective effect of estrogen [4,36]. In a very recent study conducted on a rat model of ALS, it was determined that onset of the disease, progression, and survival were related to the gender; male rats lost weight earlier and died earlier [37].…”

Female gender doubles executive dysfunction risk in ALS: a case-control study in 165 patients

“…The first explicative hypothesis related to female gonadal hormone production, the most salient biochemical factor related to gender differences, can be linked to gender differences in the disease incidence, as recently proposed by Blasco et al 22. Oestrogen controls the differentiation and plasticity of distinct neuronal populations, enhances neurogenesis, regulates some neurotransmitter activity and may have neurotrophic effects.…”

The Impact on the Family Carer of Motor Neurone Disease and Intervention with Noninvasive Ventilation

“…The mean age of onset of MND varies; however it is reported to be between 55 and 65 in most studies. 25,26 Our sample contained a higher proportion of patients in an older age group than might be expected, with nine over 70 and only one below 60. We omitted collecting carer age data; however all appeared to be in the same over60s age range as the patients.…”