Brain and Behavior Indicators in Myotonic Dystrophy (Autistic)

A new study from France gives some additional information for children that have myotonic Dystrophy. While not as comprehensive and the studies done in Sweden this gives good basic information on the condition. This information is very help to set up IEP goals and to know what issues for parents and professionals to expect. The study did not specifically mention Autism but many of the items described would be considered autistic in the USA
CONCLUSION: Children and adolescents with childhood DM1 have frequent diagnosis on the DSM-IV axis 1 with internalizing disorders being the most common type of disorder. They also have borderline low intelligence and frequent impairments in attention and visual-spatial construction abilities.
The study had 28 individuals in it, 15 females and 13 males. They were generally an older population of individuals with a mean age of 17 and a range of 7-14 years. There was no breakdown on the classification of the type of myotonic dystrophy. The age range may mean that juvenile or early childhood and moderately severe myotonic dystrophy are underrepresented in this study.
Results
Mean Full scale IQ =      73.6
Verbal IQ Mean               80.2
Performance IQ Mean   72.95
Issues Identified
Phobias (7)
Mood Disorder (6)
anxiety Disorder (5)
ADHD (8)
Other Issues
19 Patients had to repeat a grade
Alexithymia  12 of 22  (inability to express feeling with words
and to share emotional states)
Severe impairments in visual attention 4 of 18
Severe impairments in visual spacial construction 14 of 24
Dev Med Child Neurol. 2012 Oct;54(10):905-911. doi: 10.1111/j.1469-8749.2012.04379.x. Epub 2012 Aug 3.

Psychiatric and cognitive phenotype of childhood myotonic dystrophy type 1.

Source

Department of Child and Adolescent Psychiatry, Groupe-Hospitalier Pitié-Salpêtrièr, Paris  Institute of Myology and Department of Genetics, Groupe-Hospitalier Pitié-Salpêtrièr, Paris  Institute of Intelligent Systems and Robotics, Université Pierre et Marie Curie, Paris  Paediatric Neurology, Hôpital Roger Salengro, Centre Hospitalier Régional Universitaire de Lille, Lille  Child and Adolescent Psychiatry, Centre Hospitalier Universitaire d’Amiens, Université d’Amiens, Amiens, France.

Abstract

Aim   To investigate the psychiatric and cognitive phenotype in young individuals with the childhood form of myotonic dystrophy type 1 (DM1). Method  Twenty-eight individuals (15 females, 13 males) with childhood DM1 (mean age 17y, SD 4.6, range 7-24y) were assessed using standardized instruments and cognitive testing of general intelligence, visual attention, and visual-spatial construction abilities. Results  Nineteen patients had repeated a school grade. The mean (SD) Full-scale IQ was 73.6 (17.5) and mean Verbal IQ was significantly higher than the mean Performance IQ: 80.2 (19.22) versus 72.95 (15.58), p=0.01. Fifteen patients had one or more diagnoses on the DSM-IV axis 1, including internalizing disorders (phobia, n=7; mood disorder, n=6; other anxiety disorders, n=5) and attention-deficit-hyperactivity disorder, inattentive subtype (n=8). Twelve out of 22 patients had alexithymia (inability to express feelings with words and to recognize and share emotional states). Cognitive testing found severe impairments in visual attention and visual-spatial construction abilities in four out of 18, and 14 out of 24 patients respectively. No diagnosis was correlated with the transmitting parent’s sex or with cytosine-thymine-guanine (CTG) repeat numbers. Patients with severe visual-spatial construction disabilities had a significantly longer CTG expansion size than those with normal visual-spatial abilities (p=0.04). Interpretation  Children and adolescents with childhood DM1 have frequent diagnoses on DSM-IV axis 1, with internalizing disorders being the most common type of disorder. They also have borderline low intelligence and frequent impairments in attention and visual-spatial construction abilities.

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