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May 24, 2022; 98 (21) Resident & Fellow Section

Teaching NeuroImage: Drug Refractory Epilepsy With Developmental Dysarthria Due to Bilateral Perisylvian Polymicrogyria

Manda Goveen, Ayush Agarwal, Ajay Garg, View ORCID ProfileAchal Kumar Srivastava
First published April 4, 2022, DOI: https://doi.org/10.1212/WNL.0000000000200665
Manda Goveen
From the Departments of Neurology (M.G., A.A., A.K.S.) and Neuroradiology (A.G.), All India Institute of Medical Sciences, New Delhi.
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Ayush Agarwal
From the Departments of Neurology (M.G., A.A., A.K.S.) and Neuroradiology (A.G.), All India Institute of Medical Sciences, New Delhi.
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Ajay Garg
From the Departments of Neurology (M.G., A.A., A.K.S.) and Neuroradiology (A.G.), All India Institute of Medical Sciences, New Delhi.
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Achal Kumar Srivastava
From the Departments of Neurology (M.G., A.A., A.K.S.) and Neuroradiology (A.G.), All India Institute of Medical Sciences, New Delhi.
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Teaching NeuroImage: Drug Refractory Epilepsy With Developmental Dysarthria Due to Bilateral Perisylvian Polymicrogyria
Manda Goveen, Ayush Agarwal, Ajay Garg, Achal Kumar Srivastava
Neurology May 2022, 98 (21) 903-904; DOI: 10.1212/WNL.0000000000200665

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A 15-year-old boy presented with 5 years of refractory focal seizures with 4–6 episodes per day. The seizure semiology was described as numbness of the left half of the body followed by tonic posturing and head version to left with loss of awareness lasting for 30 seconds. Additional symptoms included cognitive impairment with delayed and dysarthric speech. Neuropsychological assessment revealed impaired intellectual and cognitive ability. MRI of the brain demonstrated bilateral perisylvian polymicrogyria (Figure, A–C). BPP is a cortical development malformation1 characterized by widened Sylvian fissures with an increased number of abnormally small gyri and shallow sulci. Patients present with dysarthria and refractory seizures.2

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Figure 1 Axial (A), Coronal (B), and Sagittal (C) T1-Weighted Images Show Undulations and Irregularity of the Cortical Surface Along Bilateral Sylvian Fissures and Superior Temporal Sulci

Apparent thickening of polymicrogyric cortex and stippling and irregularity at the gray-white matter junction (arrowheads) are also seen compared with frontal polar gray matter (arrows).

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The authors report no targeted funding

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The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

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Footnotes

  • Go to Neurology.org/N for full disclosures.

  • Submitted and externally peer reviewed. The handling editor was Roy Strowd III, MD, Med, MS.

  • Teaching slides links.lww.com/WNL/B913

  • Received November 4, 2021.
  • Accepted in final form March 16, 2022.
  • © 2022 American Academy of Neurology

References

  1. 1.↵
    1. Friede RL
    . Developmental Neuropathology. 2nd ed. Springer; 1989.
  2. 2.↵
    1. Barkovich AJ,
    2. Hevner R,
    3. Guerrini R
    . Syndromes of bilateral symmetrical polymicrogyria. AJNR Am J Neuroradiol. 1999;20(10):1814-1821.
    OpenUrlAbstract/FREE Full Text

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