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February 21, 2023; 100 (8) Resident & Fellow Section

Teaching NeuroImage: Brain Calcification in a Young Woman With Seizures

Explore the Rare Differentials

Bindu Menon, Gayatri Manam, Pramesh Reddy, Harsha Mandalapu, A. Sudarsana
First published December 16, 2022, DOI: https://doi.org/10.1212/WNL.0000000000201573
Bindu Menon
From the Department of Neurology (B.M., A.S.); Department of Radiology (G.M., P.R.); and Department of Gastroenterology (H.M.), Apollo Speciality Hospitals, Nellore, India.
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Gayatri Manam
From the Department of Neurology (B.M., A.S.); Department of Radiology (G.M., P.R.); and Department of Gastroenterology (H.M.), Apollo Speciality Hospitals, Nellore, India.
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Pramesh Reddy
From the Department of Neurology (B.M., A.S.); Department of Radiology (G.M., P.R.); and Department of Gastroenterology (H.M.), Apollo Speciality Hospitals, Nellore, India.
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Harsha Mandalapu
From the Department of Neurology (B.M., A.S.); Department of Radiology (G.M., P.R.); and Department of Gastroenterology (H.M.), Apollo Speciality Hospitals, Nellore, India.
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A. Sudarsana
From the Department of Neurology (B.M., A.S.); Department of Radiology (G.M., P.R.); and Department of Gastroenterology (H.M.), Apollo Speciality Hospitals, Nellore, India.
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Citation
Teaching NeuroImage: Brain Calcification in a Young Woman With Seizures
Explore the Rare Differentials
Bindu Menon, Gayatri Manam, Pramesh Reddy, Harsha Mandalapu, A. Sudarsana
Neurology Feb 2023, 100 (8) 397-398; DOI: 10.1212/WNL.0000000000201573

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An 18-year-old woman presented with refractory seizures that started at the age of 10 years. There was no history of medication use for other illnesses, radiation exposure, or neurocutaneous stigma. Her CT scan and MRI (GRE) (Figure, A and B) showed bilateral cortical and subcortical calcification in parieto-occipital regions. Based on her imaging findings, celiac disease, epilepsy, and cerebral calcification (CEC) syndrome1,2 was considered. This was confirmed by the presence of high antigliadin IgA (7.16U/mL), IgG (36.12 U/mL), and IgG tissue transglutaminase (645 U/mL) levels. Interictal EEG showed generalized discharges. Differentials were Sturge-Weber syndrome (SWS), congenital folate malabsorption, treatment with methotrexate and antifolate, and radiotherapy. SWS was excluded because of the absence of facial nevus, lobar atrophy, and subcortical calcification on MRI. Our patient had CEC syndrome with silent celiac disease. A gluten-free diet and antiseizure medications were recommended. She was seizure free on follow-up.

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Figure (A) CT Scan and (B) MRI (GRE) Show Bilateral Cortical and Subcortical Calcification in Parieto-occipital Regions

Author Contributions

B. Menon: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data. G. Manam: analysis or interpretation of data. P. Reddy: major role in the acquisition of data; analysis or interpretation of data. H. Mandalapu: analysis or interpretation of data. A Sudarsana: analysis or interpretation of data.

Study Funding

The authors report no targeted funding.

Disclosure

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

Footnotes

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

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

  • Teaching slides links.lww.com/WNL/C548.

  • Received May 24, 2022.
  • Accepted in final form October 3, 2022.
  • © 2022 American Academy of Neurology

References

  1. 1.↵
    1. Bouquet F,
    2. Bouquet F,
    3. Greco L, et al
    . Coeliac disease, epilepsy, and cerebral calcifications. Lancet. 1992;340(8817):439-443.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Sunnikutty AP,
    2. Harding J,
    3. Nelson JC
    . CEC syndrome–a rare manifestation of coeliac disease. Ulster Med J. 2008;77(3):205-206.
    OpenUrlPubMed

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