Summary about Disease
Lennox-Gastaut Syndrome (LGS) is a severe form of epilepsy that begins in childhood, typically between the ages of 3 and 5 years, but can occur anytime between 1 and 10 years. It is characterized by multiple types of seizures, cognitive impairment, and a slow or stalled psychomotor development. LGS is often resistant to treatment and requires a multidisciplinary approach to manage.
Symptoms
Multiple seizure types: Tonic seizures (stiffening), atonic seizures (drop attacks), absence seizures (staring spells), myoclonic seizures (brief jerks), and focal seizures are common.
Cognitive impairment: Developmental delays or intellectual disability is almost always present.
Behavioral disturbances: Aggression, hyperactivity, autism-like behaviors can occur.
Slowed psychomotor development: Delays in motor skills and coordination.
EEG abnormalities: Specific EEG patterns, including slow spike-and-wave complexes.
Causes
LGS can result from various underlying causes. In many cases, the exact cause is unknown (idiopathic). Known causes include:
Brain malformations: Cortical dysplasias.
Genetic disorders: Tuberous sclerosis complex, Down syndrome, and other chromosomal abnormalities.
Brain injury: Perinatal hypoxia-ischemia (lack of oxygen at birth).
Infections: Meningitis or encephalitis.
Metabolic disorders: Certain metabolic conditions.
Previous Infantile Spasms (West syndrome): LGS sometimes evolves from West syndrome.
Medicine Used
Many medications are used to manage LGS, often in combination. These medications aim to reduce the frequency and severity of seizures. Common medications include:
Valproic acid
Lamotrigine
Topiramate
Rufinamide
Clobazam
Felbamate
Cannabidiol (CBD) – Epidiolex
Fenfluramine (Fintepla)- Used for Dravet Syndrome & Lennox-Gastaut Syndrome In some cases, ketogenic diet therapy, vagus nerve stimulation (VNS), or surgery (such as corpus callosotomy) may be considered.
Is Communicable
No, Lennox-Gastaut Syndrome is not a communicable disease. It is not contagious and cannot be spread from person to person.
Precautions
Seizure precautions: Ensuring a safe environment to prevent injury during seizures (padding sharp corners, wearing helmets when appropriate).
Medication adherence: Strict adherence to prescribed medication schedules is crucial.
Regular monitoring: Regular check-ups with a neurologist to monitor seizure control and medication side effects.
Emergency plan: Having a seizure action plan in place for caregivers and school personnel.
Communication: Open communication between parents, caregivers, and medical professionals.
Comorbidities: Precautions related to related co-morbidities like behaviorial or cognitive impairments may be required
How long does an outbreak last?
LGS is a chronic condition, not an acute "outbreak." The condition persists throughout the individual's life, although the severity and frequency of seizures can fluctuate.
How is it diagnosed?
Diagnosis of LGS typically involves:
Clinical evaluation: Reviewing the patient's medical history and observing the types of seizures.
Electroencephalogram (EEG): EEG is essential to identify characteristic patterns, such as slow spike-and-wave complexes.
Neuroimaging: MRI of the brain to identify any structural abnormalities.
Developmental assessment: Evaluating cognitive and motor skills.
Genetic testing: If a genetic cause is suspected.
Timeline of Symptoms
Onset: Typically between 3 and 5 years of age, but can occur anytime between 1 and 10 years.
Early stages: May begin with a change in seizure type or frequency in a child who already has epilepsy.
Progression: Over time, multiple seizure types develop, cognitive impairment becomes apparent, and behavioral problems may emerge.
Lifelong condition: LGS is a lifelong condition, but the severity of symptoms can vary over time, and management strategies may need to be adjusted.
Important Considerations
Multidisciplinary care: Management requires a team of specialists, including neurologists, developmental pediatricians, therapists, and educators.
Quality of life: Focus on improving the individual's quality of life and maximizing their potential.
Family support: LGS places a significant burden on families; support groups and resources are essential.
Refractory epilepsy: Many individuals with LGS have seizures that are difficult to control, requiring consideration of various treatment options.
Sudden Unexpected Death in Epilepsy (SUDEP): LGS patients are at an elevated risk for SUDEP; appropriate precautions should be discussed with the neurologist.