Comparing Clinical and Radiographic Characteristics of Parry-romberg Syndrome with and Without Epilepsy: A Multicenter Cohort Study
Abstract number :
2.409
Submission category :
18. Case Studies (case reports and small series less than 5 subjects will not be accepted)
Year :
2022
Submission ID :
2204691
Source :
www.aesnet.org
Presentation date :
12/4/2022 12:00:00 PM
Published date :
Nov 22, 2022, 05:26 AM
Authors :
Leona Gunasekera, MD – Mayo Clinic Florida; Baibing Chen, MD – Resident, Neurology, Mayo Clinic Florida; Erik Middlebrooks, MD – Professor of Radiology, Radiology, Mayo Clinic College of Medicine and Science; William Tatum, DO – Professor of Neurology, Neurology, Mayo Clinic College of Medicine and Science; Joseph Sirven, MD – Professor of Neurology, Neurology, Mayo Clinic College of Medicine and Science; Anthony Ritaccio, MD – Professor of Neurology, Neurology, Mayo Clinic College of Medicine and Science; Brin Freund, MD – Fellow, Neurology, Mayo Clinic Florida; Anteneh Feyissa, MD – Associate Professor of Neurology, Neurology, Mayo Clinic College of Medicine and Science
Rationale: Parry Romberg syndrome (PRS) is an acquired condition of unilateral atrophy of the connective tissues of the face, at times occurring in association with ipsilateral intracranial abnormalities and concomitant neurologic manifestations including epilepsy. We aim to identify clinical variables that: 1) predict the development of epilepsy in PRS and 2) the development of drug-resistant epilepsy (DRE) among patients with PRS associated epilepsy (PRSe+).
Methods: We performed a retrospective study of 80 patients with PRS (sixteen PRSe+) evaluated in the Mayo Clinic Health System between 2000 and 2020. Age and sex frequency-matched 16 patients with PRS without epilepsy (PRSe-) were randomly selected from the cohort. Data including age at which hemifacial atrophy first became evident, the presence or absence of epilepsy, use and timing of immunotherapy, and brain imaging findings, including white matter disease, meningeal enhancement, cortical thickening, and atrophy, were obtained from the medical records. For the PRSe+ group, clinical data, including the age of onset, seizure semiology, electroencephalogram (EEG) findings, epilepsy classification, timing and type of immunotherapy, and antiseizure medications and surgery, were also reviewed.
Results: Among those with PRSe+, 11 were female; the median age at diagnosis of PRS was 15 years (range of 6-48 years). The mean latency between onset of cutaneous manifestations and onset of seizure activity in the PRSe+ group was 2.5 years. The mean latency between onset of cutaneous manifestations and diagnosis with PRS in the PRSe+ and PRSe- group were 5.7 years and 4.9 years, respectively (p=0.68). Intracranial abnormalities were seen in fifteen patients with PRSe+ (94%), while only 1 PRSe- patient had intracranial abnormalities (6.25%) (p < 0.01
Case Studies (case reports and small series less than 5 subjects will not be accepted)