Compulsive behaviour in a patient with Aicardi syndrome. Agenesis of the corpus callosum

The corpus callosum, which is the largest white matter structure in the brain of all placental mammals, connects the left and right cerebral hemispheres. An alteration in its morphology, hypoconnectivity or hyperconnectivity is a common marker of various neuropsychiatric pathologies. One of these is Aicardi syndrome, which is characterized by a triad of callosal agenesis, infantile spasms and chorioretinal lacunae. Patients affected by Aicardi syndrome frequently display other malformations together with congenital defects of the eyes, ribs and vertebrae. Based on the current clinical knowledge, this syndrome is now recognized as a complex neurodevelopmental disorder that includes neurolog- ical and constitutional symptoms. However, literature data have not yet defined the presence of a particular set of symptoms in psychiatric patients with this condition. The present case is the first report in which an on- going compulsive behaviour focused on the insistence to order objects has been observed in Aicardi syndrome.