TY - JOUR
T1 - Association of intracranial abnormalities with the development of epilepsy and drug-resistant epilepsy in patients with Parry-Romberg syndrome
AU - Gunasekera, Charlene L.
AU - Middlebrooks, Erik H.
AU - Burkholder, David B.
AU - Chen, Baibing
AU - Sirven, Joseph I.
AU - Wong-Kisiel, Lily C.
AU - Freund, Brin E.
AU - Tatum, William O.
AU - De la Garza-Ramos, Cynthia C.
AU - Okromelidze, Lela
AU - Feyissa, Anteneh M.
N1 - Publisher Copyright:
© 2022
PY - 2022/11/15
Y1 - 2022/11/15
N2 - 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 +.
AB - 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 +.
KW - Drug-resistant epilepsy
KW - Hemifacial atrophy
KW - Immune-mediated seizures
KW - Intracranial abnormalities
KW - Parry-Romberg syndrome
KW - Symptomatic epilepsy
KW - White matter disease
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U2 - 10.1016/j.jns.2022.120455
DO - 10.1016/j.jns.2022.120455
M3 - Article
C2 - 36242808
AN - SCOPUS:85139723711
SN - 0022-510X
VL - 442
JO - Journal of the neurological sciences
JF - Journal of the neurological sciences
M1 - 120455
ER -