Emotional lability in MND: relationship to cognition and psychopathology and impact on caregivers

Emotional Lability (EL) is a well recognized symptom of cortico-bulbar pathway dysfunction in Motor Neuron Disease/Amyotrophic Lateral Sclerosis (MND/ALS), and is reported to occur in 19-49% of patients. The Emotional Lability Questionnaire (ELQ), is specifically designed to detect EL as reported by MND patients and as observed by their carers. The aims of this study were to 1) validate the Italian version of the ELQ; 2) investigate the relationship between EL and presence of cognitive dysfunction; 3) investigate the relationship between EL and presence of psychopathology. Forty one MND patients, 39 caregivers and respective control groups composed of 39 subjects and 39 partners/friends were tested. The Italian version of the ELQ was found to have good psychometric properties. Seventy-one per cent of patients reported suffering from EL. Correlations were found between bulbar involvement and EL, and between bulbar involvement and low performance on tests of fluid intelligence and working memory. However, the cognitive profile did not correlate with any aspect of EL. The findings suggests that damage to different neurological pathways underlie cognitive change and EL, which supports the concept of MND/ALS as a multisystem disorder. Moreover the outcomes suggest that EL affects patients' everyday life with the increased anxiety and emotional frailty. The findings suggest that those involved in the care of MND patients should be more aware of the effects of EL in the management of the disease