Epilepsy in “Sunflower syndrome”: electroclinical features, therapeutic response, and long-term follow-up

Belcastro, Vincenzo; Casellato, Susanna; Striano, Pasquale; Capovilla, Giuseppe; Savasta, Salvatore; Foiadelli, Thomas; Sofia, Vito; Giuliano, Loretta; Riva, Antonella; Elia, Maurizio; Cesaroni, Elisabetta; Bonaventura, Carlo Di; Giallonardo, Teresa A.; Striano, Salvatore; Gambardella, Antonio; Ferlazzo, Edoardo; Verrotti, Alberto

doi:10.1016/j.seizure.2021.09.021

Cited by 7 publications

(10 citation statements)

References 14 publications

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“…“Sunflower syndrome,” a term coined by Ames and Saffer 37 and recently summarized by Belcastro and colleagues, 38 describes a condition characterized by stereotypic seizures. Patients turn their bodies and look toward a light source, usually sunlight and then extend their elbow and lift one hand and wave abducted fingers in front of their eyes.…”

Section: General Clinical Issuesmentioning

confidence: 99%

“…The natural history of sunflower syndrome is not well characterized, but the literature suggests that the frequency of handwaving episodes and light‐seeking behavior decreases as the patients grow older. In general, the epilepsy is refractory to anti‐seizure medicines, 38 but valproate and fenfluramine may be helpful 40,45–47 . A variety of nonpharmacological approaches have also been found to be useful, including stimulus avoidance; wearing hats, sunglasses, or special tinted glasses; staying indoors; focused attention; and using one or both hands for other activities 39,40,48 .…”

Section: General Clinical Issuesmentioning

confidence: 99%

See 1 more Smart Citation

Visually sensitive seizures: An updated review by the Epilepsy Foundation

et al. 2022

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Light flashes, patterns, or color changes can provoke seizures in up to 1 in 4000 persons. Prevalence may be higher because of selection bias. The Epilepsy Foundation reviewed light-induced seizures in 2005. Since then, images on social media, virtual reality, three-dimensional (3D) movies, and the Internet have proliferated. Hundreds of studies have explored the mechanisms and presentations of photosensitive seizures, justifying an updated review. This literature summary derives from a nonsystematic literature review via PubMed using the terms "photosensitive" and "epilepsy." The photoparoxysmal response (PPR) is an electroencephalography (EEG) phenomenon, and photosensitive seizures (PS) are seizures provoked by visual stimulation. Photosensitivity is more common in the young and in specific forms of generalized epilepsy. PS can coexist with spontaneous seizures. PS are hereditable and linked to recently identified genes. Brain imaging usually is normal, but special studies imaging white matter tracts demonstrate abnormal connectivity. Occipital cortex and connected regions are hyperexcitable in subjects with light-provoked seizures. Mechanisms remain unclear. Video games, social media clips, occasional movies, and natural stimuli can provoke PS.

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Section: General Clinical Issuesmentioning

confidence: 99%

Section: General Clinical Issuesmentioning

confidence: 99%

Visually sensitive seizures: An updated review by the Epilepsy Foundation

et al. 2022

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“…FFA has also been tested on a small group of patients suffering from Sunflower syndrome a rare photosensitive epilepsy. Patients with Sunflower Syndrome have the tendency to seek light sources and present highly stereotyped behaviors defined as hand waving episodes (HWE) ( Belcastro et al, 2021 ). In this open-label study, 10 patients with Sunflower syndrome were treated with FFA at an initial dose of 0.2 mg/kg/day, subsequently increased to a maximum of 0.7 mg/kg/day.…”

Section: Introductionmentioning

confidence: 99%

Improving Therapy of Pharmacoresistant Epilepsies: The Role of Fenfluramine

et al. 2022

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Epilepsy is among the most common neurological chronic disorders, with a prevalence of 0.5–1%. Despite the introduction of new antiepileptic drugs during recent years, about one third of the epileptic population remain drug-resistant. Hence, especially in the pediatric population limited by different pharmacokinetics and pharmacodynamics and by ethical and regulatory issues it is needed to identify new therapeutic resources. New molecules initially used with other therapeutic indications, such as fenfluramine, are being considered for the treatment of pharmacoresistant epilepsies, including Dravet Syndrome (DS) and Lennox-Gastaut Syndrome (LGS). Drug-refractory seizures are a hallmark of both these conditions and their treatment remains a major challenge. Fenfluramine is an amphetamine derivative that was previously approved as a weight loss drug and later withdrawn when major cardiac adverse events were reported. However, a new role of fenfluramine has emerged in recent years. Indeed, fenfluramine has proved to be a promising antiepileptic drug with a favorable risk–benefit profile for the treatment of DS, LGS and possibly other drug-resistant epileptic syndromes. The mechanism by which fenfluramine provide an antiepileptic action is not fully understood but it seems to go beyond its pro-serotoninergic activity. This review aims to provide a comprehensive analysis of the literature, including ongoing trials, regarding the efficacy and safety of fenfluramine as adjunctive treatment of pharmacoresistant epilepsies.

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“…There has been considerable speculation on the motivation for self-induction. Theories include wilful avoidance of unpleasant situations, seeking pleasure, relief of tension, addiction and compulsion ( 25 ).…”

Section: Discussionmentioning

confidence: 99%

“…A particular sun-seeking behavior is described in the Sunflower Syndrome (SFS), a photosensitive epilepsy now described as a rare childhood-onset generalized epilepsy characterized by photosensitivity, heliotropism, and drug resistant highly stereotyped seizure ( 25 ). Patients with SFS are commonly females, are extremely photosensitive and only rarely suffer from other form of seizure ( 25 ). Typically, the patient turns his face towards the sun, waves his hand in front of the face, rapidly blinks, or performs other movements that create a similar flicker effect.…”

Section: Discussionmentioning

confidence: 99%

Case report: A relevant misdiagnosis: Photosensitive epilepsy mimicking a blinking tic

Burlo

Barbi²,

Carrozzi³

et al. 2022

Front. Pediatr.

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Blinking in children is most frequently a functional and transient symptom. Nonetheless, sometimes it is the first clinical manifestation of a neurological disorder. The differential diagnosis between voluntary actions, tics and other neurological disorders among which seizures may be challenging and misdiagnosis is common. A 6-year-old girl in good health was admitted for a recent history of bilateral eye blinking. Blinking did not interfere with the girl's activities. The patients reported that blinking seemed to be triggered by sunlight exposure and that girl sometimes seemed to be attracted by the sunlight. Ophthalmological diseases had been already excluded. The girl was addressed to our hospital for neurological consultation, as tic disease was considered the most probable hypothesis. Neurological examination was negative. In the field of differential diagnosis of photosensitive abnormal eyelid movements, the hypothesis of seizures was explored and further investigated with a video-EEG recording with light stimulation. This exam demonstrated a photoparoxysmal response (PPR) to intermittent photic stimulation with appearance on EEG of bilateral spike and polyspike waves associated with eyelid jerks. This girl suffers from generalized epilepsy with photosensitivity. Photosensitivity is a common feature of many epilepsy syndromes, mainly occurring in children and adolescents. To control the seizures, it is essential to avoid the triggering stimulus, by wearing specific glasses. Additional antiseizures treatment is often necessary, at first with valproate and levetiracetam, and ethosuximide, lamotrigine, and benzodiazepines as the second choice. Overlapping phenomenology of seizures and movement disorders is well known in paediatric clinical practice. Moreover, epilepsy and movement disorder may coexist, mainly in children. Seizures with semeiology limited to eye motor manifestations may mimic functional blinking, tics, and other motor events frequently observed in childhood. Differentiating seizures from other non-epileptic paroxysmal movements may be challenging and specialist evaluation is needed for proper treatment and prognostic counselling.

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Epilepsy in “Sunflower syndrome”: electroclinical features, therapeutic response, and long-term follow-up

Cited by 7 publications

References 14 publications

Visually sensitive seizures: An updated review by the Epilepsy Foundation

Visually sensitive seizures: An updated review by the Epilepsy Foundation

Improving Therapy of Pharmacoresistant Epilepsies: The Role of Fenfluramine

Case report: A relevant misdiagnosis: Photosensitive epilepsy mimicking a blinking tic

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