Case Report

Neuropsychiatric Manifestations of Partial Agenesis of the Corpus Callosum: A Case Report and Literature Review

Table 1

Review of selected literature on cases of patients that presented with corpus callosum pathology.

Article GenderAgeSigns and SymptomsLab Findings/Imaging Studies

Agenesis of Corpus Callosum and Emotional Information Processing in Schizophrenia [7]F23(i) rituals of washing her hands and clothes out of fear of germs
(ii) collected issues from “People” magazine, predominantly of the American singer, Mariah Carey
(iii) megalomaniacal delusion in regard to Mariah Carey, with whom she believed was her friend and confidante
(iv) persecution belief: everyone around her was conspiring against her, wishing her ill, resulting in her to flee the city to escape her persecutors
(v) visual distortions and hallucinations
MRI - partial agenesis of the corpus callosum, above the genu and surrounding the midline, including the isthmus and anterior half of the splenium. Lateral ventricles were large, especially posteriorly, extending into the occipital lobe. fMRI - more lateralized and spatially localized activation, when the emotional content of the stimuli was considered

Delusional Disorder in a Patient with Corpus Callosum Agenesis [8]F23(i) paranoid and persecutory delusions against her brother and mother, speaking ill about her character and conspiring to kill her
(ii) delusion of reference
(iii) abusive and assaultive, e.g., started breaking expensive household items
(iv) poor appetite and difficulty falling asleep, sleeping about 3-4 hours
MRI of brain - agenesis of the corpus callosum, septum pellucidum with bilateral septal colpocephaly

Severe psychiatric disturbance and abnormalities of the corpus callosum: review and case series [5]Pt 1: F
Pt 2: M
Pt 3: M
Pt 4: M
Pt 5: M
Pt 6: M
Pt 7: F
Pt 1: 35
Pt 2: 29
Pt 3: 41
Pt 4: 33
Pt 5: 22
Pt 6: 12
Pt 7: 31
Pt 1: persecutory delusions, auditory and visual hallucinations, depression, and thought disorder
Pt 2: chronic schizophrenia, delusions, thought disorder, auditory and visual hallucinations, and epilepsy
Pt 3: manic depressive psychosis and epilepsy
Pt 4: acute anxiety state with auditory and visual hallucinations
Pt 5: schizoid personality
Pt 6: severe behavioral problems
Pt 7: personality disorder with depressive and conversion symptoms and epilepsy
Pt 1: CT - lipoma in the posterior corpus callosum. EEG performed twice at age 30; second showed some minor dysfunction
Pt 2: CT - agenesis of corpus callosum, left hemi-atrophy of anterior hemisphere due to a CSF-containing frontal lobe cyst, and widened third ventricle. EEG - abnormal with sharp waves over temporal regions
Pt 3: CT and MRI - agenesis of the anterior corpus callosum. EEGs - bursts of generalized rhythmic delta activity and left anterior quadrant dysfunction
Pt 4: CT - partial agenesis of the anterior corpus callosum. EEGs - normal
Pt 5: CT - lipoma in the splenium of the corpus callosum. EEG - slow activity
Pt 6: CT - partial agenesis of the corpus callosum with moderately large lipoma, small poorly defined caudate nuclei, and dilated temporal and posterior horns of the third ventricle. EEG - normal
Pt 7: CT - agenesis of the corpus callosum and the lateral ventricles were separated by an expanded third ventricle. EEG was normal.

Agenesis of corpus callosum and psychosis - review and case description [9]N/A43Neuropsychological investigation revealed mild mental retardationCT and MRI - partial agenesis of the corpus callosum
EEG - normal

Agenesis of the corpus callosum with associated inter-hemispheric cyst and right frontal pachygyria presenting with psychiatric symptoms in a Kenyan [10]M26(i) depression and recurrence of seizures
(ii) normal general and neurological examination apart from some frontal lobe signs on mental status examination
(iii) psychosis and labile mood
CT - agenesis of the corpus callosum with inter-hemispheric cyst and right frontal pachygyria. No fat density could be identified in the midline using Hounsfield Units.
CBC, EEG - normal

Agenesis of the Corpus Callosum [11]MMiddle
Aged
(i) paranoid delusions: doctors, nursing staff, and patients were against him
(ii) auditory hallucinations: voices, both male and female, warning him of the doctors, his wife was being unfaithful, and his death
Imaging - agenesis of the corpus callosum

Acute psychotic symptoms: a manifestation of antiphospholipid syndrome or infarction of corpus callosum [12]F25(i) sleeplessness, excessive talking
(ii) persecutory paranoid symptoms
(iii) auditory hallucinations, commentary voices and voices conversing with each other
Weighted T2 MRI - sagittal sections showed hyperintense lesions in the splenium of the corpus callosum due to an acute infarction from thrombosis due to antiphospholipid syndrome.

Corpus callosum atrophy and psychosis: a case report [13]M31(i) delusions of persecutionMRI - cerebral atrophy in the frontoparietal zone, non-specific gliotic white matter changes1, and atrophic corpus callosum.
Neuropsychological tests (e.g., Stroop test, clock-drawing test) - normal

Cognitive impairments associated with corpus callosum infarction: a ten cases study [14]10 Pts: M42-73(i) impairment of memory, attention, planning, ability to learn new things, orientation, calculation, language, and repetition; No psychiatric manifestationsMRI series (T2W1, T2W2, FLAIR, DWI) - corpus callosum infarction

Cognitive, Behavioral, and Psychiatric Symptoms in Two Children with Agenesis of the Corpus Callosum: Case Report [15]Pt 1: M
Pt 2: F
Pt 1: 11
Pt 2: 10
Pt 1: predominantly visual hallucinations over auditory hallucinations. Appeared tense and confused, with speech impairment. Lacked insight and cognition. Drawings exemplified the presence of anxiety and visual perceptual delays.
Pt 2: good personal hygiene, anxious, and mostly coherent speech. No delusions or hallucinations.
Pt 1: normal neurological examination, except for soft neurological signs. EEG - presence of partial complex seizures.
Pt 2: Psychological testing - full scale IQ of 102, ATRS score of 28. EEG - intermittent slowing, with occasional high amplitude sharp waves on the left occipital regions. No evidence of clinical seizures. MRI - partial ACC, predominantly the rostrum and the body, and colpocephaly. Thyroid function studies, urine metabolic, amino acid, and organic acid screens were normal.

Developmental Abnormalities of the Corpus Callosum in Schizophrenia [16]Pt 1: M
Pt 2: F
Pt 1: 39
Pt 2: 33
Pt 1: visual and auditory hallucinations, telling him to kill himself, since childhood.
Pt 2: Persecutory delusions that led her to attempt to kill herself before her persecutors “dismembered” her and displayed her on the “Johnny Carson Show”. Positive symptoms were refractory to treatment.
Pt 1: T1 weighted MRI - mid-sagittal image, almost complete absence of the corpus callosum
Pt 2: T1 weighted MRI - lipoma involving the entire corpus callosum

Agenesis of the corpus callosum and schizophrenia: A case report [17]M55(i) increasingly irritable with persecutory delusions towards his parents
(ii) withdrawn, believed to be persecuted as a “homosexual” at work
(iii) distressed by persistent auditory hallucinations of several “mumbling” voices which originated from within and outside his body
(iv) delusions of reference, believing that people were laughing and staring at him, and television programs referred to him
(v) no disturbance of language or mood
CT - absent corpus callosum, midline cystic space extending upward from the third ventricle, and dilated occipital horns of lateral ventricles. MRI - complete absence of the corpus callosum, present anterior commissure, and large CSF filled cavity in the left frontotemporal region, which extends to the vertex, apparently communicating with the ventricular system.

A case of schizophrenia with complete agenesis of the corpus callosum [18]F24(i) auditory hallucinations, voices of angels and the devil
(ii) erotic delusions, directed at a male school teacher
(iii) other delusions: losing a baby with every menstrual cycle
CT and MRI - complete agenesis of the corpus callosum, with colpocephaly, a sunburst gyral pattern and a high-riding third ventricle.

Corpus Callosum Agenesis and Psychosis in Andermann Syndrome [19]31 Pts: F
31 Pts: M
3-36(i) visual hallucinations, persecutory and paranoid type, auditory hallucinations, and paranoid delusionsUnspecified lab/imaging study - 40 of 62 patients presented with corpus callosum agenesis, 35 cases of total and 5 of partial agenesis. 10 of 40 patients presented with posterior fossa atrophy.

Corpus callosum shape alterations in individuals prior to the onset of psychosis [20]100 Pts:
M & F
14-30(i) 27 out of 100 patients developed a psychotic illnessT1 weighted MRI - Mid-sagittal slice showed reduced thickness of the anterior genu of the corpus callosum in the 27 symptomatic patients

Meta-analysis of magnetic resonance imaging studies of the corpus callosum in schizophrenia [21]M and F10-71(i) signs and symptoms related to disorders in the schizophrenia disorder spectrum or schizotypal personality disorderMRI - callosal areas of schizophrenic patients over the healthy unrelated control patients, course of the illness influenced the size

The corpus callosum in schizophrenia-volume and connectivity changes affect specific regions [22]12 pts: M
12 pts: F
29-49(i) chronic paranoid schizophrenia which was confirmed with structured clinical interviews.DTI brain image - mid-sagittal slices, corpus callosum segmentation in 3 patients. Volume and fractional anisotropy were positively correlated in both controls and patients. Fractional anisotropy, mean diffusivity, and volume were negatively correlated; however, all three measures were highly correlated with one another. The corpus callosum regions were not all affected equally or the same by the anatomical change.

Marchiafava-Bignami Disease Presenting as Acute Psychosis [23]M32(i) agitation, confusion, delirium, dysarthria, dementia, psychotic symptoms, and other neuropsychiatric manifestations.CT - hyperdense lesion of the corpus callosum. MRI - hyperintense lesion with restricted diffusion of the corpus callosum.

Clinical and radiological features of Marchiafava-Bignami disease [24]9 pts: M37-62(i) behavioral disorder, seizures, cognitive impairment, delirium, dysarthria and other neurological symptoms.Pt 1: MRI – extended lesions in the splenium of the corpus callosum.
Pt 2: Pt 1: MRI – circumscribed lesions in the splenium of the corpus callosum
Pt 3: Pt 1: MRI – extended lesions in the genu, body, and splenium of the corpus callosum.
Pt 4: Pt 1: MRI – extended lesions in the splenium of the corpus callosum
Pt 5: Pt 1: MRI – ovoid lesions in the splenium of the corpus callosum
Pt 6: Pt 1: MRI – extended lesions in the splenium of the corpus callosum
Pt 7: Pt 1: MRI - extended lesions in the splenium of the corpus callosum
Pt 8: Pt 1: MRI – lobulated lesions in the body, and splenium of the corpus callosum
Pt 9: Pt 1: MRI - extended lesions in the splenium of the corpus callosum

“M”, males; “F”, females; “Pt”, patient; “MRI”, magnetic resonance imaging; “fMRI”, Functional magnetic resonance imaging; “CT”, computed tomography; “DTI”, diffusion tensor imaging, “CBC”, complete blood count, “ATRS” abbreviated teacher rating scale, “ACC”, agenesis of the corpus callosum, “EEG”, electroencephalograph, and “IQ’, intelligence quotient.