Sunflower Syndrome: A lifetime of misdiagnosis in a young adult
Abstract number :
2.176
Submission category :
4. Clinical Epilepsy / 4B. Clinical Diagnosis
Year :
2017
Submission ID :
349503
Source :
www.aesnet.org
Presentation date :
12/3/2017 3:07:12 PM
Published date :
Nov 20, 2017, 11:02 AM
Authors :
Ramya Raghupathi, Cleveland Clinic; Dileep Nair, Cleveland Clinic; and Vineet Punia, Cleveland Clinic
Rationale: Self-induction by visual stimuli is a rare form of photosensitive epilepsy. Flickering light is a common stimulus used for self-induction. We present a case of a young adult with Sunflower syndrome, a rarely reported variant of photosensitive epilepsy with self-induction 1 Methods: V is a 20 year old ambidextrous woman who developed stereotypical hand waving episodes over closed eyes at five years of age, commonly noted in naturally lit environment. She could respond during these episodes but had poor recall of events with prolonged spells. They were initially diagnosed as behavioral tics and years later, following an episode of witnessed convulsion, prompted by her habitual hand waving, as Occipital epilepsy or Jeovan’s syndrome. Topamax and Keppra were tried briefly and she was eventually placed on Valproate and Lamotrigine combination, with no further convulsions. She stopped medications during pregnancy and reestablished care for her persistent habitual hand waving behavior, now interfering with child care. No self-stimulatory or gratifying feelings were reported. She described herself as “a bug to light”. Her prior brain MRI was reported to have no significant finding. Results: VEEG evaluation showed generalized spike-wave/polyspikes discharges with photo-paroxysmal response with intermittent photic stimulation. No epileptiform discharges or eyelid myoclonia was noted on eye closure. Several typical rhythmic hand waving spells over her eyes, were recorded (Figure1). The onset of this behavior was followed by an interruption of the background EEG activity, which evolved into a paroxysmal, sharply contoured spike activity (Figure 2). These lasted less than 10 seconds, which was a learned behavior over the years to avoid prolonged social awkwardness.She was diagnosed with generalized epilepsy with photosensitivity (pattern-induced)2. Cognitive behavior therapy and Z1 blue lenses were recommended in addition to Lamotrigine. Conclusions: The term sunflower syndrome was proposed by Ames and Saffer1 describing a 11-year-old girl who induced seizures by turning her head to bright lights and waving her hand over her eyes. There have been rare case reports since the initial descripton2,4. Episodes vary from childhood to adolescence and have a female preponderance and are commonly seen in patients with learning disabilities2.They have also been reported to provide a pleasant sensation or release feelings of stress4 and adolescents in particular are noted to evoke such feelings. Absences and myoclonus are commonly reported2..Our patient’s self-induction of absence and generalized seizures and EEG paroxysms by stereotyped hand waving in light is consistent with Sunflower Syndrome. To the best of our knowledge, this is the first case of an intellectually intact young adult to be diagnosed with Sunflower syndrome. The rarity of this condition may confound timely diagnosis. The highlight of our case is the challenges brought by the delayed diagnosis in a young adult mother whose spells now hindered child care. We hope this case helps increase the awareness about this rare condition, which is easily amenable to behavioral therapy and anti-epileptic drugs.References:1. Ames FR, Saffer D. The sunflower syndrome. A new look at “self-induced” photosensitive epilepsy. J Neurol Sci 1983; 59: 1-11.2. Panayiotopoulos CP. Self-induced pattern-sensitive epilepsy. Arch Neurol 1979; 36: 48-50.3. Psychiatric aspects of self-induced epileptic seizures Beng-Yeong Australian and New Zealand Journal of Psychiatry 2002; 36:534–5434. Belcastro V, Striano P; Self-induced seizures in sunflower epilepsy: a video-EEG report. Epileptic Disord 2014; 16 (1): 93-5 Funding: None
Clinical Epilepsy