Brain Abnormalities and Epilepsy in Patients with Parry-Romberg Syndrome.

BACKGROUND AND PURPOSE: Parry-Romberg syndrome is a rare disorder characterized by progressive hemifacial atrophy. Concomitant brain abnormalities have been reported, frequently resulting in epilepsy, but the frequency and spectrum of brain involvement are not well-established. This study aimed to characterize brain abnormalities in Parry-Romberg syndrome and their association with epilepsy. MATERIALS AND METHODS: This is a single-center, retrospective review of patients with a clinical diagnosis of Parry-Romberg syndrome and brain MR imaging. The degree of unilateral hemispheric atrophy, white matter disease, microhemorrhage, and leptomeningeal enhancement was graded as none, mild, moderate, or severe. Other abnormalities were qualitatively reported. Findings were considered potentially Parry-Romberg syndrome-related when occurring asymmetrically on the side affected by Parry-Romberg syndrome. RESULTS: Of 80 patients, 48 (60%) had brain abnormalities identified on MR imaging, with 26 (32%) having abnormalities localized to the side of the hemifacial atrophy. Sixteen (20%) had epilepsy. MR imaging brain abnormalities were more common in the epilepsy group (100% versus 48%, P < .001) and were more frequently present ipsilateral to the hemifacial atrophy in patients with epilepsy (81% versus 20%, P < .001). Asymmetric white matter disease was the predominant finding in patients with (88%) and without (23%) epilepsy. White matter disease and hemispheric atrophy had a higher frequency and severity in patients with epilepsy (P < .001). Microhemorrhage was also more frequent in the epilepsy group (P = .015). CONCLUSIONS: Ipsilateral MR imaging brain abnormalities are common in patients with Parry-Romberg syndrome, with a higher frequency and greater severity in those with epilepsy. The most common findings in both groups are white matter disease and hemispheric atrophy, both presenting with greater severity in patients with epilepsy.

Age-specific anti-Müllerian hormone and electrocardiographic silent coronary artery disease.

OBJECTIVE: To explore the relationship between age-specific anti-Müllerian hormone level, as a predictor of ovarian reserve status, and electrocardiographic silent coronary artery disease in a population-based, prospective cohort, the Tehran Lipid and Glucose Study. METHODS: For the present study, 1015 reproductive-aged participants in the Tehran Lipid and Glucose Study met our eligibility criteria. According to the Whitehall criteria, silent coronary artery disease was defined as an electrocardiogram showing possible or probable coronary heart disease using Minnesota codes. By excluding those with a history of coronary heart disease and silent coronary artery disease at the initiation of the study (n = 49), there were 108 events of silent coronary artery disease at electrocardiograms among 752 women followed for 9.5 ± 0.9 years (missing data: n = 214); the association between this outcome with age-specific anti-Müllerian hormone levels was explored after adjustment for confounding variables using logistic regression analysis. Cardiovascular disease risk scores were assessed for all participants using the guidelines of the American College of Cardiology and the American Heart Association. RESULTS: There were 108 events of silent coronary artery disease over the 10-year follow-up. Logistic regression analysis, considering age-specific anti-Müllerian hormone and atherosclerotic cardiovascular disease risk score as independent variables, revealed an odds ratio of 1.146 (95% confidence interval 1.008-1.303) for cardiovascular disease risk score (p = 0.038) and odds ratio of 1.002 (95% confidence interval 0.996-1.009) for age-specific anti-Müllerian hormone (p = 0.526). CONCLUSION: No association has been found between age-specific anti-Müllerian hormone levels and events of silent coronary artery disease in a 10-year follow-up of reproductive-aged women.