Neuropsychiatric symptoms in Friedreich's ataxia assessed by the Mild Behavioral Impairment Checklist (MBI-C)
Abstrakt
Background and aims: Neuropsychiatric symptoms (NPS) are common in hereditary ataxias. In Friedreich’s ataxia (FRDA), depressive symptoms were previously reported, but little is known about other NPS. The MBI-C is a questionnaire assessing NPS in early stages of neurodegenerative diseases. Using the MBI-C, we aimed to assess the presence and severity of NPS in FRDA and examine their relationship with disease severity.Methods: 27 FRDA patients and 37 healthy controls (HC) were recruited at the Centre of Hereditary Ataxias. Close informants of all participants filled in the MBI-C.
Disease severity was assessed by the Scale for the Assessment and Rating of Ataxia (SARA), and the scale of the Activities of Daily Living (ADL).Results: Mean MBI scores in FRDA and HC were 6.70 (SD=8.70) and 2.59 (SD=3.20) respectively. 25.9% of FRDA patients had at least one MBI symptom compared to 18.9% HC. Prevalence of specific NPS in FRDA was 25.9% (vs. 18.9% HC) for decreased motivation, 55.6% (vs. 32.4%) for emotional dysregulation, 48.1% (vs. 37.8%) for impulse dyscontrol, 18.5% (vs. 8.1%) for social inappropriateness and 11.1% (vs. 13.5%) for psychotic symptoms. Patients had significantly higher score only in the emotional dysregulation domain (p=0.026). ADL correlated with motivation (r=0.39, p=0.044) and emotional dysregulation (r=0.39, p=0.048). SARA did not correlate with any MBI-C score.Conclusion: NPS are common in FRDA, particularly in the affective domain, are linked to impairment of ADL but not ataxia severity. NPS should be addressed in clinical care due to their potential impact on quality of life and the possibility for therapeutic intervention.