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Table of Contents    
Year : 2016  |  Volume : 64  |  Issue : 5  |  Page : 1057-1058

A report on the first case of Tourette syndrome associated with segmental agenesis of corpus callosum

1 Department of Neuroimaging and Endovascular Neurosrugery, Indo-American Hospital, Brain and Spine Centre, Vaikom, Kerala, India
2 Department of Neurology, Indo-American Hospital, Brain and Spine Centre, Vaikom, Kerala, India

Date of Web Publication12-Sep-2016

Correspondence Address:
Jeevan S Nair
Department of Neurology, Indo-American Hospital, Brain and Spine Centre, Vaikom, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.190302

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How to cite this article:
Harsha KJ, Nair JS. A report on the first case of Tourette syndrome associated with segmental agenesis of corpus callosum. Neurol India 2016;64:1057-8

How to cite this URL:
Harsha KJ, Nair JS. A report on the first case of Tourette syndrome associated with segmental agenesis of corpus callosum. Neurol India [serial online] 2016 [cited 2020 Sep 23];64:1057-8. Available from:


A boy with apparently normal birth and developmental history started having repeated abnormal involuntary movements at 5 years of age. As noticed by his parents, the abnormal movements were repeated blinking of eyes, deviation of the angle of mouth, adversive head movements to one side and sudden flinging movements of the hand. In addition, he had occasional temper tantrums. After 2–3 years of onset of these abnormal movements, the parents noticed that he could control these movements when asked to do so. At the age of 8 years, the boy started producing abnormal yelling sounds. He was able to control these abnormal sounds in crowded places or in school, but with significant discomfort. He had features of obsessive-compulsive disorder (OCD) in the form of indulging in excessive cleanliness, and frequent and compulsive hand washing. He was also previously diagnosed as having Attention Deficit Hyperactivity Disorder (ADHD). At the time of presentation to our institute, he was 13 years old, and was already on risperidone and clonidine medications. His abnormal movements and vocal sounds were still uncontrolled. He was average in his studies. He also had subtle learning disabilities, as noticed by his parents. He was satisfying all clinical criteria required for the diagnosis of Tourette syndrome (TS). As the previous literature had revealed grossly normal magnetic resonance imaging (MRI) in TS, MRI was not considered for many years. However, the patient's parents were apprehensive and were repeatedly insisting on conducting a brain imaging, and hence, an MRI was performed. MRI [Figure 1] revealed dysgenesis of corpus callosum (AgCC). He was started on tetrabenzoate and kept on a regular follow up.
Figure 1: Magnetic resonance imaging of the brain. (a) Axial T2-weighted image showing colpocephaly, unfused septum pellucidum. (b) Coronal T2-weighted image showing absence of genu of corpus callosum (Probst bundles). (c) Sagittal image showing segmental agenesis of corpus callosum, radial arrangement of hemispheric sulci, and an elongated straight sinus

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TS is an inherited disabling neuropsychiatric disorder occurring in the childhood and adolescence, presenting typically with motor and photic tics that are refractory to medical treatment. The quest for etiology and exact pathophysiology of TS remains elusive till date. Both genetic and environmental factors are believed to play a role in its etiology. Several studies have shown subtle brain structural changes using volumetric MRI of the basal ganglia, lateral frontal cortex, thalamus, midbrain and periaqueductal gray matter, cerebellum, hippocampus, and amygdala.[1] Subtle abnormality in interhemispheric connectivity,[2] as demonstrated by diffusion tensor imaging (DTI) technique, was hypothesized as the possible etiological factor for the occurrence of TS.[3] Functional MRI studies showed abnormalities in the basal ganglia and thalamus,[4] paralimbic areas, and sensorimotor areas.[5] However, no gross MRI abnormalities have been reported in the corpus callosum in TS till date.[3] We report the first case of TS associated with gross structural abnormalities in the corpus callosum. Our case also supports the long-standing hypothesis of TS developing due to an abnormal interhemispheric connectivity. The complete agenesis of splenium and posterior body of corpus callosum, hypoplasia of rostrum, genu and anterior body of corpus callosum, and segmental sparing of the central body of corpus callosum in our case may provide further insights into the segmental functioning of corpus callosum. As this is the first case demonstrating such an association between AgCC and TS, the possibility of the association being a chance finding cannot be completely ruled out, and future case studies/series with similar associations would strengthen the hypothesis that corpus callosal maldevelopment may be in some way responsible for the genesis of TS.

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  References Top

Felling RJ, Singer HS. Neurobiology of Tourette syndrome: Current status and need for further investigation. J Neurosci 2011;31:12387-95.  Back to cited text no. 1
Bäumer T, Thomalla G, Kroeger J, Jonas M, Gerloff C, Hummel FC, et al. Interhemispheric motor networks are abnormal in patients with Gilles de la Tourette syndrome. Mov Disord 2010;25:2828-37.  Back to cited text no. 2
Cavanna AE, Stecco A, Rickards H, Servo S, Terazzi E, Peterson B, et al. Corpus callosum abnormalities in Tourette syndrome: An MRI-DTI study of monozygotic twins. J Neurol Neurosurg Psychiatry 2010;81:533-5.  Back to cited text no. 3
Peterson BS, Skudlarski P, Anderson AW, Zhang H, Gatenby JC, Lacadie CM, et al. A functional magnetic resonance imaging study of tic suppression in Tourette syndrome. Arch Gen Psychiatry 1998;55:326-33.  Back to cited text no. 4
Bohlhalter S, Goldfine A, Matteson S, Garraux G, Hanakawa T, Kansaku K, et al. Neural correlates of tic generation in Tourette syndrome: An event-related functional MRI study. Brain 2006;129(Pt 8):2029-37.  Back to cited text no. 5


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