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Abstract
Background and Objectives Cingulate epilepsy (CE) is a rare type of focal epilepsy that is challenging to diagnose because of the polymorphic semiology of the seizures, mimicking other types of epilepsy, and the limited utility of scalp EEG.
Methods We selected consecutive patients with drug-resistant CE who were seizure-free after surgery, with seizure onset zone (SOZ) confirmed in the cingulate cortex (CC) by histology or stereo EEG. We analyzed subjective and objective ictal manifestations using video recordings and correlated semiology with anatomical CC subregion (anterior, anterior middle, posterior middle, and posterior) localization of SOZ.
Results We analyzed 122 seizures in 57 patients. Seizures were globally characterized by complex behaviors, typically natural seeming and often accompanied by emotional components. All objective ictal variables considered (pronation of the body or arising from a lying/sitting position, tonic/dystonic posturing, hand movements, asymmetry, vocalizations, fluidity and repetitiveness of motor manifestations, awareness, and emotional and autonomic components) were differently distributed among CC subregions (p < 0.05). Along the rostro-caudal axis, fluidity and repetitiveness of movement, vocalizations, body pronation, and emotional components decreased anterior–posteriorly, whereas tonic/dystonic postures, signs of lateralization, and awareness increased. Vestibular and asymmetric somatosensory, somatosensory, and epigastric and enteroceptive/autonomic symptoms were distributed differently among CC subregions (p < 0.05). Along the rostro-caudal axis, vestibular, somatosensory, and somatosensory asymmetric symptoms increased anterior–posteriorly.
Discussion CE is characterized by a spectrum of semiologic manifestations with a topographic distribution. CE semiology could indicate which cingulate sector is primarily involved.
Glossary
- ACC=
- anterior cingulate cortex;
- aMCC=
- anterior midcingulate cortex;
- CC=
- cingulate cortex;
- CE=
- cingulate epilepsy;
- FCD=
- focal cortical dysplasia;
- MCC=
- midcingulate cortex;
- PCC=
- posterior cingulate cortex;
- pMCC=
- posterior midcingulate cortex;
- SEEG=
- stereo EEG;
- SOZ=
- seizure onset zone
Footnotes
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.
- Received July 13, 2021.
- Accepted in final form January 18, 2022.
- © 2022 American Academy of Neurology
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