D Willekens

A study of the cognitive and psychological profile in 16 children with congenital or juvenile myotonic dystrophy

We report data on intelligence and on possibly associated psychopathology in 16 children and adolescents, between 7 and 18 years of age, with congenital or juvenile myotonic dystrophy. We found that all the subjects have an intelligence level below the population mean, four of them in the mentally retarded range. An unexpected number of subjects are in the clinical range on the Child Behavior Checklist. In nine of the 16 subjects, a child psychiatric diagnosis was found using a standardized psychiatric interview. The most frequent child psychiatric diagnosis was Attention Deficit Hyperactivity Disorder.

Marden-Walker syndrome versus isolated distal arthrogryposis: evidence that both conditions may be variable manifestations of the same mutated gene.

In this report we present evidence that Marden‐Walker syndrome and isolated distal arthrogryposis may be variable manifestations of the same entity.

Delusional and Psychotic Disorders in Juvenile Myotonic Dystrophy Type-1

We investigated the clinically derived hypothesis of a relatively high incidence of delusional and psychotic disorders in adolescents with juvenile Myotonic Dystrophy type‐1 (DM1). Twenty‐seven subjects of age 16–25 with juvenile DM1 and their parents were invited to have a clinical psychiatric interview, and to complete an ASEBA behavior checklist (YSR, ASR, CBCL, and ABCL). We diagnosed a Delusional Disorder in 19% of our patients and a Psychotic Disorder not otherwise specified in another 19%. These two groups of patients had a significantly worse level of clinically defined general functioning. It is clinically relevant to investigate in patients with juvenile DM the symptom of delusions and the presence of a delusional and psychotic disorder, and to consider the presence of juvenile DM in youngsters presenting with such a thought disorder. These disorders compromise the general functioning of the subjects and are often to some extent treatable.