Agenesis of the corpus callosum: a United Kingdom series of 56 cases

Abstract

A survey of cases of agenesis of the corpus callosum was carried out to examine its associations, utilising the British Neurological Surveillance Unit (BNSU). Fifty six cases were reported (36 male), 37 were adults. Nearly two thirds had epilepsy; half of the adult cases had intellectual impairment as estimated clinically, and a third a psychiatric disorder. Nine cases (five adults) were apparently normal neurologically, and may have escaped detection but for a coincidental or minor disorder leading to neurological investigation. The BNSU is a valuable aid in the study of rare disorders but in less severe conditions, such methods of ascertainment inevitably underestimate prevalence and are prone to selection bias towards patients with associated morbidity. (J Neurol Neurosurg Psychiatry 1998;64:131-134)

First-Episode ‘Coenesthetic’ Schizophrenia Presenting with Alien Hand Syndrome and Partial Agenesis of the Corpus Callosum

“…ACC may only be discovered at autopsy or on in vivo neuroimaging and may remain clinically asymptomatic during lifetime [9] . Also, although psychotic illness has been reported to be over-represented, ACC seems not to be specific to any disorder [10] . ACC has been associated with other neuropsychiatric disorders, including bipolar psychosis [37] , pervasive developmental disorders [38] and attention-deficit hyperactivity disorder [39] , and the commonest presentation is that of severe mental retardation and early focal seizures [40] .…”

“…Prevalence estimates of ACC in the general population are low, and range from 0.073 [5] to 2.2% [6] . ACC has been reported to be found in patients with alien hand syndrome (AHS), a phenomenon that is characterized by the subjective feeling that the hand does not belong to the patient [7] .A growing number of cases have described ACC in patients with schizophrenia [8][9][10] . We report a case of a young woman with the coenesthetic subtype of schizophrenia presenting partial ACC and AHS.…”

Can we predict drug response by volumes of the corpus callosum in newly diagnosed focal epilepsy?

“…Epilepsy is reported in up to two-thirds of patients with developmental corpus callosum agenesis (Taylor & David, 1998). In addition, microstructural changes of the corpus callosum were demonstrated in patients with acquired epilepsy by several studies using DTI and volumetric methods (Kim et al, 2008;Weber et al, 2007).…”

Ultrasound Diagnosis of Congenital Brain Anomalies

“…Hence, prognosis is determined primarily by the underlying or associated malformations. Studies of persons with isolated agenesis of the corpus callosum without other abnormalities show that some have normal intelligence, while others are developmentally delayed (Taylor et al, 1998). Complete agenesis has a worse prognosis than partial agenesis.…”