Cognitive and behavioural characteristics in blind children with bilateral optic nerve hypoplasia.
OBJECTIVE
To describe cognitive and behavioural characteristics in a group of blind children with bilateral optic nerve hypoplasia (ONH).


METHODS
Data from records, parents, teachers, and repeated developmental assessments of 13 blind children with ONH born in 1988-1998 were analysed. All children had neuroimaging and/or hormonal evidence of midline malformations. They were all blind and able to communicate with speech.


RESULTS
Severe mood swings and temper tantrums were common, especially during the first years of life. Later in life, sluggish tempo, low frustration tolerance and a narrow range of interests were common. Autism had been diagnosed in 6/13 children, autistic-like condition (ALC) was found in another three. The behaviour of the remaining four children was not within the autism spectrum. Eight children had cognitive capacities within the normal or near-normal range; five had mental retardation. Autism/ALC was found in all cognitive subgroups. All children exhibited fluent speech and, of these, 12 had started to talk at the expected age, but had clear deficiencies in communicative ability.


CONCLUSIONS
These children had a common pattern of behavioural characteristics including autism spectrum disorders independent of intellectual capacities.
Ek
Ulla
U
Department of Psychology, University of Stockholm and Tomteboda Resource Centre, Stockholm. uek@psychology.su.se
Fernell
Elisabeth
E
Jacobson
Lena
L
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000453:epidemiology; D017677:Age Distribution; D001321:Autistic Disorder / Q000175:diagnosis* / Q000453:epidemiology; D001766:Blindness / Q000151:congenital / Q000453:epidemiology / Q000523:psychology*; D002648:Child; D002652:Child Behavior; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis* / Q000453:epidemiology; D015331:Cohort Studies; D002658:Developmental Disabilities / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009483:Neuropsychological Tests; D009900:Optic Nerve / Q000002:abnormalities; D009901:Optic Nerve Diseases / Q000151:congenital* / Q000453:epidemiology / Q000523:psychology; D015995:Prevalence; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D013548:Sweden / Q000453:epidemiology