What Is the Sunflower Syndrome?

The most often related seizure types in a child with sunflower syndrome (SFS) are absence, tonic-clonic, and myoclonic seizures.

Myoclonic seizures

• Recognized by jerks that last only a few seconds, similar to a sudden shock. Seizures can last anywhere from one to three minutes.

Tonic-clonic seizures: Preceded by

• Muscle stiffness
• Loss of consciousness
• Air is forced through the vocal cords and produces sounds of crying or moaning
• A quick, rhythmic jerking of the feet and hands, bending and relaxing the elbows, hips, and knees
• After a few minutes, the jerking will stop, and consciousness will slowly return

Eyelid myoclonia followed by absent seizures

• Eyelid myoclonia is the most common type of seizure in SFS. This usually lasts one to three seconds (rarely up to six seconds). These seizures are characterized by brief, repetitive jerks of the eyelids.
• The eyeballs roll upward at the same time, revealing the white part of the eyes, and the head jerks backward. 
• Some children may experience absence seizures in addition to eyelid myoclonia, resulting in a mild impaired awareness.

Other symptoms

• SFS symptoms include an initial attraction to bright light, followed by seizure activity that includes moments of hand-waving and disturbed consciousness.
• The attraction to light frequently occurs days to months before the commencement of hand-waving episodes.

Seizures of eyelid myoclonia may occur only sometimes in the first few weeks of a child's epilepsy. However, the seizures gradually become more frequent, to the point that they may occur 10, 20, or even 30 (or more) times each day. Seizures are more likely to occur when the child closes their eyes.

• Sunflower syndrome (SFS) is rare pediatric epilepsy characterized by photosensitivity and heliotropism (tendency to move toward the light).
• SFS was previously classified as self-induced photosensitive epilepsy, which occurs when hand-waving causes a seizure.
• According to electroencephalography investigations, the misfiring of neurons in the brain, or epileptiform activity, begins at the same time as the hand-waving action.
• This shows that hand-waving is a symptom of the seizure rather than the cause.

The etiology of sunflower syndrome (SFS) is currently unknown. However, the condition is quite stereotypical and usually appears in the first 10 years of life. This increases the possibility of a hereditary component. The underlying genetics associated with the illness are now being investigated to uncover and understand the possible causes

Potential cause of SFS

• In such syndromes, mutations or deletions of associated genes have been documented, including RORB, KIAA2022, GLUT1, and NAA10 genes. As a result, genetic testing can validate the problematic genes.
• Children with SFS are also photosensitive. This means that the seizures are caused by flickering light. Seizures can be triggered by simply exiting a dark room and entering a sunlit area.
• Risk factors for SFS
• Girls are more likely to be affected than boys; 75 percent of diagnosed patients are girls.
• SFS usually starts in the first decade of life. Physicians most commonly diagnose SFS in children aged between 3 and 10 years.
• SFS is provoked particularly by precipitating factors such as sleep deprivation, alcohol, and some medications during pregnancy.
• At least 33 percent of patients with SFS have a family history of epilepsy, which includes absence epilepsy and juvenile myoclonic epilepsy. There has only been one recorded instance of SFS with a possible family history.

SFS is a type of photosensitive epilepsy. Seizures can occur even if the person does not have a seizure problem at the initial stages, and it's critical to understand what triggers photosensitive epilepsy.

Triggers of photosensitive epilepsy such as SFS

• Light from TV, computer, smartphone, or movie theater screens
• Light that flickers, such as strobes, disco lights, or malfunctioning fluorescent bulbs
• Light that is bright or reflected, such as sunlight reflected from a mirror, window, or snowbank
• Colors or patterns with high contrast, such as stripes and dots
• Light streaming through partly concealed windows
• Moving things, such as escalators, ceiling fans, propellers, and helicopter blades

The etiology of SFS is unknown; however, electroencephalography and imaging investigations have suggested that there may be an issue with the brain's cingulate circuit. The cingulate cortex is a portion of the brain located in the medial region of the cerebral cortex. It is a component of the limbic system and is involved in emotion processing and behavior control. Moreover, it helps modulate autonomic motor function.

There is presently no clinical or laboratory standard for diagnosing sunflower syndrome (SFS). However, doctors may recommend observation under video surveillance and electroencephalogram (EEG) testing.

Video surveillance

• The diagnosis is obtained by carefully examining what occurs during the seizures, particularly the age at which the seizures begin. The doctor may look for triggers that may give them a clue about SFS.
• However, this may be challenging because seizures only last a few seconds. Seizures are sometimes mistaken as a mannerism or a facial tic, especially in young children.

EEG

• Individuals with SFS exhibit abnormal EEGs with symptoms compatible with generalized epilepsy.
• The EEG should also show photosensitivity in this type of epilepsy. Ictal epileptiform activity may develop less than one second after hand-waving begins.
• The EEG may reveal generalized spike-wave discharges seen mostly during self-induced seizures or during photic/light stimulation.

More studies are required to better understand the natural history and probable genetic etiology of SFS. This would increase scientific knowledge and, ultimately, lead to better treatment options for this complicated epilepsy disease.

Sunflower syndrome (SFS) frequently necessitates polytherapy or the administration of more than one drug along with other lifestyle modifications. There is no one-size-fits-all treatment for such a rare syndrome. 

Treatment options include

• Current medications that seem to be working on a few patients
  • Valproic acid (recognized as the most effective treatment of photosensitive epilepsies)
  • Levetiracetam
  • Ethosuximide
  • Benzodiazepines
  • Lamotrigine
  • Trimethadione
  • Fenfluramine (reduces or suppresses hand-waving episodes without evidence of significant side effects; reduction in hand-waving episodes may lead to a decrease in seizure episodes)
  • Antianxiety medications can be used in combination with antiseizure medications if SFS is associated with anxiety
  • Sometimes, a combination of these medicines may be more effective than a single medicine
• Behavioral interventions
• Wearing sunglasses
  • Special or tinted glasses
  • Welder's goggles (most effective)
  • Zeiss Z1 glasses or specific blue lens glasses (may reduce seizures; eliminate the photo-paroxysmal response on the electroencephalogram tracing)
• Others
  • Wearing hats
  • Hand holding
  • Proper encouragement of voluntary inhibition of self-provocation
  • A dietary therapy with a low glycemic index
  • Staying indoors to avoid the sunlight 
  • Focused attention (while playing sports or driving)

There are no particular medications licensed for the therapy. Broad-spectrum anticonvulsant drugs have not been demonstrated to be completely effective in preventing seizures in people with SFS. As a result, there is still a substantial unmet demand for authorized therapy for SFS in children and adults. There may be ongoing research investigations related to this epilepsy condition.

The effects of sunflower syndrome (SFS) have not been fully explored. As the treatment of SFS is in its early stages and causes are yet to be determined, a child with SFS may develop potential complications, including

• Low self-esteem
• Brief disruptions in consciousness
• Injuries and trauma
• Bullying due to the unusual motor movements associated with their seizures
• School performance issues
• Anxiety and depression
• About half of the people with prominent photic induction have learning disabilities or problems with attention
• Misdiagnosis that may lead to drug abuses

Children with SFS may also face additional stress from physicians, family members, and friends who assume the hand-waving episodes are self-inflicted and under conscious control.

SFS is an uncommon disorder that is frequently misdiagnosed. Misdiagnosis of SFS may be reduced if clinicians are aware of its clinical and electroencephalogram features. There is a scarcity of data on the long-term prognosis of SFS, and definitive information on the optimal treatment techniques is inadequate. Consult a neurologist immediately to reduce the negative effects of SFS in the future. If a patient or their family member experiences seizure symptoms that are consistent with SFS, they must contact their primary care physician.