Epilepsy syndromes are disorders characterized by seizures and other distinct concurrent symptoms. The National Institute of Neurological Disorders and Stroke reports that researchers have defined hundreds of epilepsy syndromes. Each syndrome involves specific seizure types, a characteristic age of onset and a predictable pattern of disease progression and response to treatment.
Juvenile Absence Epilepsy
Juvenile absence epilepsy is characterized by the onset of sporadic absence seizures in early to late adolescence. An absence seizure is an abrupt, brief lapse in consciousness, during which the sufferer is temporarily disengaged. Physical activity and interaction cease for a few seconds during an absence seizure. Activity resumes immediately after passage of the seizure. The person affected characteristically has no awareness of the seizure event.
The International League Against Epilepsy documentation on juvenile absence epilepsy notes that approximately 75 percent of patients experience occasional, generalized tonic-clonic seizures, with loss of consciousness and characteristic muscle involvement. Among those with juvenile absence epilepsy, generalized tonic-clonic seizures most commonly occur upon awakening.
Males and females are equally affected by juvenile absence epilepsy, which typically occurs in otherwise healthy children and persists into adulthood. In an article published by Epilepsy.com/Professionals, Dr. C. Panayiotopoulos notes that approximately 70 percent to 80 percent of patients achieve seizure control with medication.
Lennox-Gastaut Syndrome
Lennox-Gastaut syndrome is a childhood-onset form of epilepsy characterized by frequent seizures of varying types. This epilepsy syndrome generally occurs in children with underlying mental retardation, which typically progresses with ongoing seizure activity. Tonic seizures are characteristic of Lennox-Gastaut syndrome, involving brief periods of unconsciousness with bodywide muscle stiffening. Other seizure types that commonly occur with Lennox-Gastaut syndrome include generalized tonic-clonic seizures, myoclonic seizures, atypical absence seizures and "drop attacks," during which the child suddenly drops to the floor with involuntary muscle activity or a complete lack of muscle tone.
Children with Lennox-Gastaut syndrome typically have behavioral problems in addition to a seizure disorder and mental retardation. Hyperactivity, aggression, emotional instability, hypersexuality, psychosis and autistic behavior patterns may occur. The International League Against Epilepsy documentation on Lennox-Gastaut syndrome notes that the disorder most commonly manifests in children age 2 to 8 years, with boys disproportionately affected. Control of seizure activity with Lennox-Gastaut proves difficult and incomplete in most cases.
Jeavons Syndrome
Jeavons syndrome, also known as eyelid myoclonia with absence, is characterized by eyelid jerking coupled with brief absence seizures and light sensitivity. Eye closure in a bright environment triggers seizure activity. Flashing light also typically provokes eyelid myoclonia with absence. Multiple seizure episodes occur daily with this syndrome.
In an article published by Epilepsy.com/Professionals, Dr. C. Panayiotopoulos reports that the usual age of onset of Jeavons syndrome is age 2 to 14 years, with girls affected more frequently than boys. The syndrome persists into adulthood and proves highly resistant to treatment with anti-seizure medications.
References
- International League Against Epilepsy: Juvenile Absence Epilepsy
- Epilepsy.com/Professionals: Juvenile Absence Epilepsy
- International League Against Epilepsy: Lennox-Gastaut Syndrome
- Epilepsy.com/Professionals: Lennox-Gastaut Syndrome
- Epilepsy.com/Professionals: Jeavons Syndrome (Eyelid Myoclonia with Absences)
