Academic journal article Hong Kong Journal of Psychiatry

Obsessive Patient with Agenesis of the Corpus Callosum

Academic journal article Hong Kong Journal of Psychiatry

Obsessive Patient with Agenesis of the Corpus Callosum

Article excerpt


Agenesis of the corpus callosum is an uncommon congenital anomaly. The following is a report of a patient with agenesis of the corpus callosum with cavum septum pellucidum and colpocephaly. This patient has also been diagnosed with anankastic personality disorder.

Keywords: Corpus callosum, Septum pellucidum, Personality disorders


The corpus callosum is the largest commissural connection between the 2 cerebral hemispheres. Anatomically, the corpus callosum is inferior to the longitudinal fissure and superior to the diencephalon. It is divided into 4 parts: the rostrum, body, genu, and splenium. Tumour, infarct, or injury to the corpus callosum can lead to interhemispheric transfer deficit, depending on the site and extent of involvement. (1) The so-called 'disconnection syndrome' was demonstrated by Sperry in 1974, in which split brain individuals whose corpus callosum was surgically sectioned were recruited for split brain experiments. (2) The experiment brought greater understanding of hemispheric function.

In psychiatric practice, agenesis of the corpus callosum (ACC) is an uncommon congenital malformation. It is usually detected in childhood, whereas in adults it is paucisymptomatic. (3) ACC accounts for 14% of central nervous system malformations. (4) Some patients with ACC are asymptomatic and ACC is only detected incidentally by neuroimaging such as computed tomography (CT) of the brain or magnetic resonance imaging (MRI). Nevertheless, some patients may present to a psychiatrist because of epileptic seizures, (4) mental retardation, developmental delay, or a psychotic experience. However, there is usually no significant relationship between ACC and psychosis, although some studies have suggested an association between posterior fossa atrophy or cerebellar anomalies and schizophrenia-like psychosis. (5) ACC can be a discrete and isolated entity but, occasionally, it is associated with Dandy-Walker cysts, hydrocephalus, meningomyelocele, microgyria, arachnoid cysts, interhemispheric cysts, porencephaly, Arnold-Chiari II malformations, skull deformities, facial dysmorphism, inborn errors of metabolism, neurocutaneous disease, or chromosomal microdeletion or aberrations (4,6-9) Associations with ACC have also been found with the following illnesses--asplenia syndrome, multiple congenital anomaly syndrome, X-linked mental retardation syndrome, Andermann syndrome, DiGeorge-velocardiofacial syndrome, Aicardi syndrome, and Coffin-Lowry syndrome. (5,8,10-13) X-linked recessive inheritance (14) and autosomal recessive inheritance (15) have been most commonly reported.

The prognosis depends on the presence of cerebral malformations that are involved in epilepsy and cognitive disturbances. There was a case report of a patient with ACC with strong anatomical and functional asymmetries. (16) Strong right hand superiority for hand skill and tactile object recognition indicated unusual left hemispheric dominance for these functions. (16) On the other hand, research has revealed that patients with ACC do not have disconnection syndrome as do individuals with split brain. Symptoms of interhemispheric disconnection are typically less severe for patients with ACC than after surgical section of the corpus callosum. It has been suggested that functional compensation is less efficient if ACC is partial rather than complete. (17) However, ACC is associated with specific problems in complex cognitive operations such as tests on reasoning and concept formation. In patients with ACC, significantly poor performance has been found for tests of social insight, proverb interpretation, social logic, self-perception, and interpretation of ambiguous stimuli. (18) The following is a report of an adult man with complete ACC, cavum septum pellucidum, colpocephaly, and concomitant anankastic personality disorder.

Case Report

A 34-year-old unmarried man, living with his parents, was referred to the South Kwai Chung Psychiatric Centre from the Medical Unit of the Princess Margaret Hospital due of worsening of work and social performance. …

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