Psychiatric and Cognitive Phenotype of Childhood Myotonic Dystrophy Type 1

Autor/inn/en	Douniol, Marie; Jacquette, Aurelia; Cohen, David; Bodeau, Nicolas; Rachidi, Linda; Angeard, Nathalie; Cuisset, Jean-Marie; Vallee, Louis; Eymard, Bruno; Plaza, Monique; Heron, Delphine; Guile, Jean-Marc
Titel	Psychiatric and Cognitive Phenotype of Childhood Myotonic Dystrophy Type 1
Quelle	In: Developmental Medicine & Child Neurology, 54 (2012) 10, S.905-911 (7 Seiten) PDF als Volltext Verfügbarkeit
Sprache	englisch
Dokumenttyp	gedruckt; online; Zeitschriftenaufsatz
ISSN	0012-1622
DOI	10.1111/j.1469-8749.2012.04379.x
Schlagwörter	Intelligence; Testing; Children; Anxiety Disorders; Hyperactivity; Attention Deficit Disorders; Patients; Intelligence Quotient; Symptoms (Individual Disorders); Spatial Ability; Speech Communication; Hospitals; France; Beck Depression Inventory + Suchen Sie Ihr Suchwort? Intelligenz; Klugheit; Testdurchführung; Testen; Child; Kind; Kinder; Hyperaktivität; Attention deficit hyperactivity disorder; ADHS; Aufmerksamkeits-Defizit-Hyperaktivitäts-Störung; Aufmerksamkeitsstörung; Patient; Intelligenzquotient; Psychiatrische Symptomatik; Räumliches Vorstellungsvermögen; Krankengymnast; Krankenhaus; Frankreich
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. (Contains 3 tables and 1 figure.) (As Provided).
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Erfasst von	ERIC (Education Resources Information Center), Washington, DC
Update	2017/4/10