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NEUROIMAGES
Year : 2019  |  Volume : 67  |  Issue : 1  |  Page : 325-326

Congenital unilateral peri-Sylvian syndrome in a patient with recurrent seizures, left hemiparesis, and cognitive impairment


1 Department of Radiology, Yashoda Hospital, Hyderabad, Telangana, India
2 Department of Radiology, Vijaya Diagnostic Centre, Hyderabad, Telangana, India

Date of Web Publication7-Mar-2019

Correspondence Address:
Dr. Ankit Balani
61, Shyam Nagar, Pal Link Road, Jodhpur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.253642

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How to cite this article:
Chatur C, Balani A, Murthy MG, Vadapalli R. Congenital unilateral peri-Sylvian syndrome in a patient with recurrent seizures, left hemiparesis, and cognitive impairment. Neurol India 2019;67:325-6

How to cite this URL:
Chatur C, Balani A, Murthy MG, Vadapalli R. Congenital unilateral peri-Sylvian syndrome in a patient with recurrent seizures, left hemiparesis, and cognitive impairment. Neurol India [serial online] 2019 [cited 2019 Mar 20];67:325-6. Available from: http://www.neurologyindia.com/text.asp?2019/67/1/325/253642




A 13-year-old female patient presented to neurology outpatient department of our hospital with complaints of recurrent episodes of seizures, left-sided weakness, and cognitive impairment. There was no history of feeding, swallowing, or speech difficulties. The birth history was unremarkable. Family history revealed no known consanguinity. General examination revealed no facial dysmorphic features. Neurological examination revealed cognitive deficit with an intelligent quotient (IQ) of 66. Electroencephalogram (EEG)revealed frequent spike discharges with phase reversal in the right temporal leads suggestive of abnormal awake EEG record showing right temporal epileptic focus. Neurological examination revealed decreased muscle power (4/5 in left upper and lower limbs), hyperactive reflexes with increased muscle tone, spastic in nature on left side associated with cognitive deficits. The patient then underwent MRI brain, acquired with a 1.5-T unit (Siemens, Erlangen, Germany). MRI brain revealed enlarged, dysplastic, and vertically oriented right Sylvian fissure, which was continuous with the right central sulcus. There was thickening of gray matter (the cortical thickness in peri-Sylvian region measured upto 7.5 mm) intermixed with thin and shallow sulci and broad gyri involving the right peri-Sylvian region extending dorsally upto peri-Rolandic region suggestive of coarse peri-Sylvian polymicrogyria [Figure 1] and [Figure 2]. There was ipsilateral cerebral and brainstem hemiatrophy associated with right thalamostriatal hypoplasia. The right lateral ventricle was dilated and its body showed an inverted appearance [Figure 2]. Left cerebral parenchyma was normal in signal intensity pattern with normal gray–white matter differentiation. The clinical features of recurrent seizures, contralateral hemiparesis, and cognitive impairment associated with imaging features of enlarged, dysplastic, and vertically oriented right Sylvian fissure with ipsilateral peri-Sylvian polymicrogyria suggested the diagnosis of congenital unilateral peri-Sylvian syndrome. The parents were counseled about the disease and the patient was managed with anticonvulsants.
Figure 1: Sagittal T1 (a and b) sections of MRI brain showing enlarged, dysplastic, and vertically oriented right Sylvian fissure continuous with the right central sulcus. There is thickening of gray matter intermixed with thin and shallow sulci and broad gyri involving right peri-Sylvian and right frontal regions

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Figure 2: Axial T1 sections (a-c) of MRI brain show thickening of gray matter (cortical thickness in peri-Sylvian region measuring upto 7.5 mm) intermixed with thin and shallow sulci and broad gyri involving right peri-sylvian region extending dorsally upto perirolandic region suggestive of coarse peri-Sylvian polymicrogyria. (a and b) There is ipsilateral cerebral hemiatrophy associated with right thalamostriatal hypoplasia. (a-c) Right lateral ventricle was dilated and its body showed an inverted appearance. (b) Left cerebral parenchyma was normal in signal intensity pattern with normal gray–white matter differentiation

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A malformation of cortical development, polymicrogyria, occurs due to interruption of the process of normal cortical development during the late stages of neuronal migration and during the stages of cortical organization. As a result, there is abnormal development of the deeper layers of the cerebral cortex and the formation of multiple small gyri.[1],[2] Polymicrogyria may be unilateral (∼40%) or bilateral (∼60%).[2] The most common location is around the Sylvian fissure, especially its posterior aspect.[2],[3] Unilateral peri-Sylvian dysplasia corresponds to the unilateral counterpart of the bilateral peri-Sylvian syndrome.[4],[5] Unilateral polymicrogyria can be hemispheric or focal.[1] Congenital unilateral peri-Sylvian syndrome manifests with seizures and cognitive impairment along with hemiplegia and pyramidal signs contralateral to the associated unilateral peri-Sylvian polymicrogyria. It presents later and has less severe symptoms as compared to its bilateral counterpart.[2] Treatment depends upon the symptoms and their severity. Treatment with anticonvulsant drugs may help prevent, reduce, or control various types of epilepsy associated with this disorder.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Barkovich AJ, Kuzniecky RI, Jackson GD, Guerrini R, Dobyns WB. A developmental and genetic classification for malformations of cortical development. Neurology 2005;65:1873-87.  Back to cited text no. 1
    
2.
Barkovich AJ. Current concepts of polymicrogyria. Neuroradiology 2010;52:479-87.  Back to cited text no. 2
    
3.
Leventer RJ, Jansen A, Pilz DT, Stoodley N, Marini C, Dubeau F, et al. Clinical and imaging heterogeneity of polymicrogyria: A study of 328 patients. Brain 2010;133:1415-27.  Back to cited text no. 3
    
4.
Kuzniecky R, Andermann F, Guerrini R. Congenital bilateral perisylvian syndrome: Study of 31 patients. The CBPS Multicenter Collaborative Study. Lancet 1993;341:608-12.  Back to cited text no. 4
    
5.
Sebire G, Husson B, Dusser A, Navelet Y, Tardieu M, Landrieu P. Congenital unilateral perisylvian syndrome: Radiological basis and clinical correlations. J Neurol Neurosurg Psychiatry 1996;61:52-6.  Back to cited text no. 5
    


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