By: Rahima Olatinwo
What is Jeavons syndrome?
Jeavons syndrome or epilepsy with eyelid myoclonia (EEM) is a rare, generalized epilepsy condition that results in rapid eyelid twitching.
The Genetics of Jeavons syndrome
Jeavons syndrome is idiopathic, so the causes are not known. However, it is believed to be a genetic condition. People diagnosed with Jeavons usually have a family history of epilepsy.
It typically appears in children around 2-14 years old with the average age of onset being 7 years old, and it is more prevalent in girls than in boys.
What to Look For
Children with Jeavons syndrome usually show three main symptoms.
- Eyelid Myoclonia with or without absence seizures
Eyelids will quickly twitch/flutter (myoclonia). Eyes will roll upward in a jerky manner, while the head tilts backward (retropulsion). Children can temporally lose awareness (with absence seizures) or maintain awareness (without absence seizures)
2. Eye closure-induced seizures
Seizures usually begin immediately after children close their eyes. They are very brief but happen many times a day.
3. Photosensitivity
Seizures are triggered by children closing their eyes in the presence of sunlight or bright lights. Seizures usually don’t occur if children close their eyes in a dark space with minimal light.
More about Seizures in Jeavons Syndrome
Eyelid myoclonia is the main marker for Jeavons syndrome. However, children can also experience absence seizures and generalized tonic-clonic seizures either spontaneously or in the presence of bright lights. Children may also experience body jerks in other areas like arms or legs (myoclonic jerks), but this is rare.
Similar Conditions
The symptoms of Jeavons syndrome can resemble those of…
Facial tics: eyelid spasms/twitching; rapid blinking; head jerking
Juvenile myoclonic epilepsy: myoclonic seizures; generalized tonic-clonic seizures photosensitivity
Childhood absence epilepsy: absence seizures; eyes may roll upward and flutter
Treatment and Management
There are no cures for Jeavons syndrome currently, and many children are resistant to treatment. However, for some, it is possible to manage the symptoms with antiepileptic drugs. In addition, some found that switching to the Modified Atkins diet helped with their symptoms.
Summary
Jeavons syndrome or epilepsy with eyelid myoclonia is an idiopathic generalized epilepsy condition that affects children. The symptoms of Jeavons syndrome include eyelid fluttering and seizures that result from the eyes closing in the presence of bright lights. Eyelid myoclonia is the most common seizure type, but absence, generalized tonic-clonic, and myoclonic jerks in the limbs can also be seen. While there is no definite treatment, AEDs can help manage seizures, and some have found diets like the Modified Atkins diet to be helpful.
References
Cincinnati Children’s Hospital Medical Center. “Childhood Absence Epilepsy | Symptoms, Diagnosis & Treatment.” Www.cincinnatichildrens.org, www.cincinnatichildrens.org/health/c/childhood-absence-epilepsy#:~:text=Childhood%20absence%20epilepsy%20(CAE)%20is.
National Organization for Rare Disorders. “Epilepsy with Eyelid Myoclonia – Symptoms, Causes, Treatment | NORD.” Rarediseases.org, 11 July 2023, rarediseases.org/rare-diseases/epilepsy-with-eyelid-myoclonia/. Accessed 2 Jan. 2024.
Paibool, Watuhatai, et al. “Modified Atkins Diet in Children with Epilepsy with Eyelid Myoclonia (Jeavons Syndrome).” Epilepsy & Behavior, vol. 145, 1 Aug. 2023, pp. 109347–109347, https://doi.org/10.1016/j.yebeh.2023.109347. Accessed 2 Jan. 2024.
Smith, Kelsey M., et al. “Jeavons Syndrome: Clinical Features and Response to Treatment.” Pediatric Neurology, vol. 86, Sept. 2018, pp. 46–51, https://doi.org/10.1016/j.pediatrneurol.2018.06.001.
Sonbol, Mona M. “Juvenile Myoclonic Epilepsy Clinical Presentation: History, Physical Examination, Comorbidities.” Emedicine.medscape.com, 15 Feb. 2022, emedicine.medscape.com/article/1185061-clinical?form=fpf.
Zawar, Ifrah, and Elia Pestana Knight. “Epilepsy with Eyelid Myoclonia (Jeavons Syndrome): A Comprehensive Update.” Pediatric Neurology, vol. 121, Dec. 2020, pp. 75–80, https://doi.org/10.1016/j.pediatrneurol.2020.11.018. Accessed 30 Apr. 2021.
