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NEUROIMAGES
Year : 2016  |  Volume : 64  |  Issue : 5  |  Page : 1086-1087

Bilateral perisylvian polymicrogyria: An interesting presentation of malformation of cortical development in an adult


1 Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
2 Department of Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Date of Web Publication12-Sep-2016

Correspondence Address:
Deepak Menon
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.190263

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How to cite this article:
Menon D, Swaika S, Menon R, Thomas B, Radhakrishnan A. Bilateral perisylvian polymicrogyria: An interesting presentation of malformation of cortical development in an adult. Neurol India 2016;64:1086-7

How to cite this URL:
Menon D, Swaika S, Menon R, Thomas B, Radhakrishnan A. Bilateral perisylvian polymicrogyria: An interesting presentation of malformation of cortical development in an adult. Neurol India [serial online] 2016 [cited 2019 Oct 17];64:1086-7. Available from: http://www.neurologyindia.com/text.asp?2016/64/5/1086/190263




A 42-year-old manual laborer having had learning disabilities with delayed language milestones, presented with refractory seizures since 17 years of age. The seizure started with a sensory aura over the left upper limb followed by its extensor posturing suggesting ictal onset over the right primary sensory area followed by ictal propagation to supplementary motor area. On examination, he had an intelligence quotient of 80, pragmatic language impairment, and subtle bipyramidal signs in the form of bifacial weakness, upper and lower limb Grade I spasticity with exaggerated deep tendon reflexes with normal sensory modalities.

His magnetic resonance imaging showed distinctive features of bilateral perisylvian polymicrogyria with irregular cortical surface, apparently increased thickness of the cortex with an uneven but well appreciable gray-white junction [Figure 1]. His interictal electroencephalogram showed bilateral centroparietal as well as posterior head region spikes and polyspikes, some of which had tangential dipole as well as secondary bilateral synchrony, while his ictal recording showed onset over the midline and right centroparietal regions as low voltage fast propagation to the frontal regions [Figure 2].
Figure 1: T1-weighted axial (a), T2-weighted axial (b), T2-weighted high-resolution coronal (c), T1-weighted turbo inversion recovery coronal (d) images showing bilateral symmetrical uneven cortical thickening with jagged surface in the perisylvian regions (arrows)

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Figure 2: (a) Bipolar montage showing a slightly earlier ictal onset over the right centroparietal region observed as low voltage fast activity with subsequent faster evolution over the left centroparietal regions. (b) Average montage showing bilateral centroparietal spikes and polyspike wave discharges during sleep

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Polymicrogyria constitutes cortical malformations secondary to abnormal postmigrational development characterized by multiple small cortical folds with histology revealing disordered cortical lamination.[1] Often centered around the perisylvian region, they are clinically characterized by perisylvian syndrome – oromotor apraxia, facial diplegia, masticatory weakness, mental retardation and seizures.[2] Contiguous cortical regions are often involved including the posterior extension to parietal lobe, which may explain the onset of seizures as a sensory aura in this patient.

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  References Top

1.
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. 1
    
2.
Barkovich AJ, Hevner R, Guerrini R. Syndromes of bilateral symmetrical polymicrogyria. AJNR Am J Neuroradiol 1999;20:1814-21.  Back to cited text no. 2
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