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May 11, 2021; 96 (19) Resident & Fellow Section

Teaching NeuroImages: Ohtahara Syndrome due to Unilateral Perisylvian Polymicrogyria

View ORCID ProfileSabarish Sekar, Pavankumar Rudrabhatla, Vinayagamani Selvadasan, Bejoy Thomas, Chandrasekharan Kesavadas
First published January 5, 2021, DOI: https://doi.org/10.1212/WNL.0000000000011497
Sabarish Sekar
From the Department of Imaging Sciences and Interventional Radiology (S.S., B.T., C.K.), Department of Neurology (P.R.), and Department of Imaging Sciences and Interventional Radiology (V.S.), Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
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  • ORCID record for Sabarish Sekar
Pavankumar Rudrabhatla
From the Department of Imaging Sciences and Interventional Radiology (S.S., B.T., C.K.), Department of Neurology (P.R.), and Department of Imaging Sciences and Interventional Radiology (V.S.), Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
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Vinayagamani Selvadasan
From the Department of Imaging Sciences and Interventional Radiology (S.S., B.T., C.K.), Department of Neurology (P.R.), and Department of Imaging Sciences and Interventional Radiology (V.S.), Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
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Bejoy Thomas
From the Department of Imaging Sciences and Interventional Radiology (S.S., B.T., C.K.), Department of Neurology (P.R.), and Department of Imaging Sciences and Interventional Radiology (V.S.), Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
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Chandrasekharan Kesavadas
From the Department of Imaging Sciences and Interventional Radiology (S.S., B.T., C.K.), Department of Neurology (P.R.), and Department of Imaging Sciences and Interventional Radiology (V.S.), Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
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Teaching NeuroImages: Ohtahara Syndrome due to Unilateral Perisylvian Polymicrogyria
Sabarish Sekar, Pavankumar Rudrabhatla, Vinayagamani Selvadasan, Bejoy Thomas, Chandrasekharan Kesavadas
Neurology May 2021, 96 (19) e2456-e2457; DOI: 10.1212/WNL.0000000000011497

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A 10-month-old boy, born of nonconsanguineous parents, without any perinatal insult, presented with tonic spasms from the 13th day of life. He started having tonic seizures from 4 months of age, with global developmental delay. On examination, there were no neurocutaneous markers, facial dysmorphism, or focal neurologic deficits. EEG (figure) showed generalized and multifocal epileptiform discharges along with burst-suppression pattern. MRI of brain revealed left perisylvian polymicrogyria (figure).

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Figure MRI and EEG

Coronal T2 (A) and coronal T1 (B, C) images show left perisylvian polymicrogyria (A), abnormally thick and bumpy coarse appearance of cortex (B), and widening of sylvian fissure (C). (D) Arterial spin labeling (postlabeling delay 2,500 ms) image shows left perisylvian hypoperfusion corresponding to polymicrogyria (arrowhead). (E) EEG shows typical burst-suppression pattern (time base: 20 mm/s, gain: 10 μV/mm, high-frequency filter: 70 Hz, low-frequency filter: 1 Hz).

Ohtahara syndrome is an electroclinical syndrome, characterized by infantile-onset epileptic encephalopathy and typical burst-suppression pattern on EEG, which remains unchanged during sleep and wakefulness.1 It is most commonly associated with structural malformations including neuronal migration disorder or dysgenesis, mutations in genes including ARX, CDKL5, SLC25A22, STXBP1, and KCNQ2, and various metabolic disorders.1,2 Presence of structural malformations, like left-sided perisylvian polymicrogyria in our case, precludes extensive genetic and metabolic analysis and differentiates it from early myoclonic encephalopathy.

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No targeted funding reported.

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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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  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Teaching slides http://links.lww.com/WNL/B303

  • © 2021 American Academy of Neurology

References

  1. 1.↵
    1. Beal JC,
    2. Cherian K,
    3. Moshe SL
    . Early-onset epileptic encephalopathies: Ohtahara syndrome and early myoclonic encephalopathy. Pediatr Neurol 2012;47:317–323.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Pavone P,
    2. Spalice A,
    3. Polizzi A,
    4. Parisi P,
    5. Ruggieri M
    . Ohtahara syndrome with emphasis on recent genetic discovery. Brain Dev 2012;34:459–468.
    OpenUrlCrossRefPubMed

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