Inspired by patients
Abstract
Sunflower syndrome (SS) is a rare, photosensitive epilepsy characterized by an attraction to light and highly stereotyped seizures with associated hand-waving (HW). It is controversial whether HW is part of the seizure or a provoking factor; therefore, we aimed to characterize the ictal electroencephalogram (EEG) in patients with SS. Video-EEG (vEEG) and charts of five patients with SS with HW during vEEG from Massachusetts General Hospital's Pediatric Epilepsy Program were reviewed and analyzed. In four out of five patients, the ictal EEG showed high amplitude (500-700 μV) 3-4 Hz generalized spike/polyspike-and-slow wave discharges, lasting 1.63-24.41 s. One hundred and twelve of 126 HW episodes, correlating to epileptiform activity (vEEG), had a lag time of less than 1.00 s (88.89%) between onset of HW and appearance of epileptiform activity. This suggests that HW does not induce seizure activity. Awareness of the ictal EEG features of this syndrome is important, as patients are frequently described as "self-inducing" their seizures.
Belcastro V, Striano P. Self-induction seizures in sunflower epilepsy: a video-EEG report. Epileptic Disord. 2014 Mar;16(1):93-5. doi: 10.1684/epd.2014.0630. PMID: 24556582.
Abstract
Seizures triggered by visual stimuli are the most common type of reflex seizure. Self-induced seizures produced by stimulation of natural light are rare and self-induction is a mode of seizure precipitation employed by either intellectually disabled or healthy photosensitive individuals. Absences and myoclonic jerks are the most common seizure types in self-induction. We report on a girl with normal intelligence who self-induced seizures by waving her outspread fingers in front of a bright light. This situation is called sunflower epilepsy.
Nava E, Mori AC, Striano P, Ramantani G. Atypical presentation of sunflower epilepsy featuring an EEG pattern of continuous spike waves during slow-wave sleep. Epileptic Disord. 2021 Dec 1;23(6):927-932. doi: 10.1684/epd.2021.1353. PMID: 34704936.
Abstract
Sunflower epilepsy is a rare photosensitive and commonly pharmacoresistant reflex epilepsy characterized by stereotyped seizures involving turning of the head towards light, similar to a sunflower turning towards the sun, and waving of the hands in front of the eyes, sometimes followed by absence seizures, myoclonic jerks, and tonic-clonic seizures. In the original description, seizures in sunflower epilepsy have been perceived as self-induced, but contemporary case series suggest that hand waving corresponds to an ictal phenomenon and not to a precipitating factor. We describe a nine-year-old girl featuring absence seizures with eye rolling or fluttering associated with hand waving movements. The chronological sequence of events based on a video-EEG-documented episode of our patient adds to the controversy surrounding the hypothesis of "self-induced" epileptic seizures in sunflower epilepsy. Shortly after epilepsy diagnosis, our patient presented with an EEG pattern of continuous spike waves in slow-wave sleep, an EEG feature that has not been described before and may relate to the cognitive deficit observed in some patients with sunflower epilepsy. Continuous spike waves in slow-wave sleep resolved, and lasting seizure freedom was achieved by a combination of ethosuximide and lamotrigine, which may be a possible alternative to valproic acid, particularly in girls and women of childbearing age. However, an attempt to taper anti-seizure drugs two years later led to seizure recurrence. We suggest performing sleep EEG recordings for sunflower epilepsy, particularly in patients with developmental stagnation or regression, to timely diagnose and treat continuous spike waves in slow-wave sleep syndrome.
Mazzone S, Landolina L, Utili A, Belcastro V, Striano P, Cordelli DM, Russo A. Neuropsychological analysis in sunflower syndrome suggests the involvement of the ventral attention network on right visual dorsal stream. Epileptic Disord. 2025 Apr;27(2):280-286. doi: 10.1002/epd2.20330. Epub 2025 Feb 7. PMID: 39918374.
Abstract
Sunflower syndrome, a rare photosensitive epilepsy characterized by handwaving episodes and fixation on light sources, remains poorly understood, especially regarding its neuropsychological profile. This study provides a detailed cognitive evaluation of two patients, revealing a likely disruption in the visual dorsal stream, with particular involvement of the ventral attention network (VAN). Despite normal overall intellectual functioning, both patients exhibited significant deficits in Processing Speed Index and inhibitory control, indicating selective executive dysfunction. Coupled with EEG anomalies predominantly in the right frontal lobe, these findings suggest right hemisphere involvement, potentially along the VAN pathway. This study highlights the need for further research, particularly functional neuroimaging, to better understand the neurocognitive aspects of sunflower syndrome and its impact on patients' quality of life.
See: https://childnervoussystem.blogspot.com/2026/03/sunflower-syndrome-redux.html
https://childnervoussystem.blogspot.com/2026/03/sunflower-syndrome-ocular-treatment.html
https://childnervoussystem.blogspot.com/2021/10/epilepsy-in-sunflower-syndrome.html
https://childnervoussystem.blogspot.com/2021/03/more-sunflowers.html
https://childnervoussystem.blogspot.com/2021/01/sunflower-syndrome.html
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