Understanding Sunflower syndrome: Results of an online questionnaire.

OBJECTIVE: To characterize the clinical phenotype, treatments, and impact on quality of life of Sunflower syndrome. METHODS: A 138-question survey was created focusing on seizure description, disease course, treatment history, medical history, family history, and aspects of quality of life of individuals with Sunflower syndrome. The survey was administered to individuals with Sunflower syndrome who experience hand waving episodes (HWE) and/or their caregivers via Research Electronic Data Capture (REDCap). RESULTS: Sixty-eight responses were included in analysis. Seventy-one% of respondents were female. The mean age of participants was 13.6 years, with 84% of respondents under the age of 18. The average age of onset of HWE was 6.7 years. HWE frequency varied from a few episodes per week to multiple episodes per hour. Sixty-two% of participants experienced other seizure types. Participants had been on an average of 1.9 anti-seizure medications with varying efficacy. Other methods to reduce HWE included wearing a hat or sunglasses, hand holding, using special tinted lenses, and avoiding the sun and bright lights. Sixty-nine% of participants reported anxiety or depression related to their epilepsy, and 65% said their HWE affected their social life. SIGNIFICANCE: Sunflower syndrome is a highly stereotyped, refractory epilepsy which significantly impacts the lives of affected individuals. It remains underrecognized and poorly understood. These results characterize Sunflower syndrome in a large population of affected individuals and provides a basis for future research to better understand the epilepsy and improve clinical care.

Elements of Pregnancy and Parenthood Policies of Importance to Medical Students and Included in a Sample of Medical Schools' Websites and Student Handbooks.

Background: Medical students who are parents or considering parenthood often want information about school policies. An earlier survey of 194 medical students from one U.S. school examined seven "elements that [students thought] should be included in a school policy on pregnancy/maternity leave." For example, students want to know "how much time a student can take off during medical school and still graduate with their class." We performed multivariate and multivariable analyses of the University of South Dakota survey to understand its generalizability and usefulness. Methods: The earlier survey also included 35 demographic variables about individual students. We tested empirically for associations between the demographics and the seven policy items, thereby evaluating generalizability of the survey results to different demographic groups. We then surveyed public websites of a sample of U.S. medical schools to evaluate usefulness of the knowledge of the seven items. For the 33 surveyed schools, we documented if each of the items was present on publicly available webpages and handbooks. Results: The seven items had content validity as a necessary and sufficient set of items. There also were no significant associations of the items with demographic variables. Therefore, there is little chance that differences among medical schools in their average demographic would affect the items needed for their websites and student handbooks. Among the surveyed medical school websites, 1 of 33 had all seven items (upper 95% confidence limit: 14% of schools nationally would be expected to have all seven items shown). Conclusions: These findings show that it is known what information students want to know about in a school policy on pregnancy and parental leave. Adding these items to public websites is a necessary and an easily actionable intervention to help current and future medical students.

Characterizing Sunflower syndrome: a clinical series.

To characterize the clinical phenotype of Sunflower syndrome. Sunflower syndrome is a rare photosensitive epilepsy syndrome characterized by highly stereotyped seizures, photosensitivity, and heliotropism. We retrospectively reviewed the medical records of patients seen in the Massachusetts General Hospital for Children (MGHfC) pediatric epilepsy program with a history of Sunflower syndrome. Twenty-four patients were identified; 18 were female. At the time of initial MGHfC evaluation, patients' ages ranged from 6.4 to 25 years, with a median age of 11.5 years. All patients presented with hand-waving episodes (HWEs), although one patient no longer demonstrates this, but now has eye blinking episodes on exposure to light. Four have associated eye fluttering as a component of their most prevalent light-induced seizures. The average age at onset of HWEs was six years. Seventeen developed other symptoms prior to the onset of HWEs. The most prevalent symptom was an attraction to light and possible absence seizures. Light-induced seizures were generally refractory to broad-spectrum antiepileptic drugs (AEDs). Only three patients had a reduction of HWEs with the use of AEDs. Several non-pharmacological strategies reduced seizure frequency, however, efficacy varied. These non-pharmacological strategies included avoiding stimulus, focusing on other tasks, and occupying or restraining the hand that was involved in hand-waving. The use of tinted glasses reduced seizure frequency in 17 patients, however, no patient achieved seizure freedom. Twenty-two patients had available EEGs, 20 of which showed interictal epileptiform discharges. Additionally, many of the patients experienced a negative impact on their self-concept due to anxiety, depression, or negative interactions with peers. Sunflower syndrome is a generalized, pharmacoresistant epilepsy with childhood onset and remains poorly understood. To improve clinical care and scientific understanding, long-term prospective research exploring the natural history, etiology, and effective treatments for Sunflower syndrome should be conducted. [Published with video sequence].