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Association of intracranial abnormalities with the development of epilepsy and drug-resistant epilepsy in patients with Parry-Romberg syndrome

Published:October 08, 2022DOI:https://doi.org/10.1016/j.jns.2022.120455

      Highlights

      • Ipsilateral intracranial manifestations are common in patients with Parry-Romberg syndrome associated epilepsy (PRSe+)
      • The severity of ipsilateral brain abnormalities is a risk factor for developing PRSe+ but not drug resistance epilepsy.
      • The timing of immunotherapy did not influence the development of PRSe + or drug resistance epilepsy.
      • Intracranial findings on brain MRI could serve as a biomarker for epilepsy in Parry-Romberg syndrome.

      Abstract

      Background

      Epilepsy represents an essential component of Parry Romberg syndrome (PRS). This study aimed to identify clinical factors that influence the development of epilepsy and drug-resistant epilepsy (DRE) in PRS.

      Methods

      We retrospectively reviewed the medical records of eighty patients with PRS. Data including the age of onset for PRS, history of seizures, use and timing of immunotherapy, antiseizure medication use, and EEG and brain imaging findings were reviewed. For comparison with the patients with epilepsy (PRSe+) group, we selected 18 age and sex-matched controls from the patient without epilepsy (PRSe-) cohort using propensity score matching.

      Results

      Eighteen (22.5%) had epilepsy: 12 were female, and the median age was 14.5 years (range = 6–48 years). Eleven patients developed DRE. The median latency between the onset of cutaneous manifestations and diagnosis and timing and use of immunotherapy was similar between the PRSe + and PRSe- groups. Intracranial abnormalities were commonly seen in the PRSe + group (16 vs. 2, p < 0.01). White matter disease and ipsilateral atrophy were common among the PRSe + group. Timing and use of immunotherapy, epileptiform discharges, and brain imaging abnormalities did not differ between those with DRE and without.

      Conclusions

      The presence and degree of severity of ipsilateral brain abnormalities are risk factors for the development of epilepsy in PRS but not factors in predicting drug resistance. The timing of immunotherapy did not influence the development of PRSe + or DRE. Prospective studies are needed to identify biomarkers for epilepsy and assess the role of immunotherapy on seizure outcomes in PRSe + .

      Keywords

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