Almost one third of BMD patients have cognitive or neuropsychiatric disorders

An Italian team conducted a review of the literature published after 1995 on cognitive, behavioural and psychosocial disorders in people with Becker muscular dystrophy (BMD). The meta-analysis included 11 publications, included five focused on central nervous system disorders in BMD and five  multicentre or registry studies.

  • Across all studies, the distribution of intelligence quotient (IQ) values was wide, suggesting wide variability, and not correlated with disease severity. The IQ was below 70 in 7-23% of cases depending on the study.
  • The two studies that analysed selective IQ impairment in 56 patients showed that verbal IQ tended to be higher than performance IQ, with no significant difference between the two.
  • The only study to have carried out detailed neuropsychological assessments in 23 people with BMD, aged between 6 and 43 years, showed that long-term memory was better preserved than working memory and that visual-motor integration skills were weaker.
  • In four studies, attention deficit/hyperactivity disorder accounted for 32-36% of the total 126 cases.
  • Two studies (n=81) reported learning difficulties in one third of cases.
  • Emotional difficulties (most often depression) were found in 28 to 38.6% of cases in six studies.
  • Of the 121 people over 20 who responded to a self-questionnaire in a Japanese study in 2018, 45.6% reported having been harassed as a student and 73% had difficulties in the workplace.

 

Cognitive profile and neuropsychiatric disorders in Becker muscular dystrophy: A systematic review of literature. Ferrero A, Rossi M. Neurosci Biobehav Rev. 2022 Mar 31;137:104648.