When seizures occur too often or too close together, seizure emergencies or potential emergency situations may arise. People with LGS can have a high number of seizures. Brief single seizures can occur in clusters or groups of many over a number of minutes, hours, or days, then go hours or days without any. Sometimes it’s hard to tell if a change in behavior is related to seizure activity or to underlying neurological or behavioral changes. It may also be hard to tell when one seizure ends and another begins.
All people with LGS, families and caregivers should be aware of seizure patterns and triggers and what a person’s usual seizures look like. This will help you know when a situation occurs that is different than usual, when to give rescue seizure medications, and when emergency medical help may be needed.
What are acute repetitive seizures or clusters?
Seizures of any type may occur in groups or clusters over a number of hours or days. A person usually recovers between seizures and the clusters will end on their own. People with LGS may have clusters of brief seizures over a number of hours (for example atypical absence, myoclonic, tonic, atonic, or partial seizures). Others may have seizures in clusters over a few days in a row. If these clusters persist, a person may start having tonic clonic seizures too or seizures that don’t stop on their own.
Sometimes even if a person is not having seizures that a person can see, the EEG may be showing seizure activity (often we call this ‘subclinical’ or ‘quiet’ seizures). While seizures may not be easy to identify, the person may be unusually sleepy, inattentive, confused, agitated or just not themselves.
If a rescue therapy can be given at home during the cluster, ideally the visible seizures will stop. A rescue therapy may be a medication like rectal diazepam gel (Diastat), lorazepam (Ativan), diazepam (Valium), or midazolam. A magnet to activate the VNS Therapy is also considered a rescue therapy. Ideally, a rescue therapy will stop the seizure clusters. If the treatment doesn’t work and seizures get closer together or longer than usual, the person may need treatment in an emergency room.
People can be at risk for repeated clusters or status epilepticus if:
- They have LGS or uncontrolled epilepsy.
- Seizure clusters last longer than normal.
- Seizures occur closer together.
- Person doesn’t recover as well between seizures or clusters.
- Rescue medicines given to stop the clusters don’t work
Status epilepticus occurs when a person has repeated seizures over a period of time or seizures last too long. Most patients with LGS will have one or several episodes of status epilepticus. There are two main types of status epilepticus:
Convulsive status epilepticus occurs when:
- One tonic clonic seizure lasts longer than 5 minutes,
- Person doesn’t return to their baseline before another seizure occurs,
- Repeated seizures ocur over a 30 minute period.
Nonconvulsive status epilepticus:
- Occurs when repeated seizures of the same type or a mix of seizures occur together, without clear recovery between events. Seizures may include atypical absence, partial seizures, or other seizures without full loss of consciousness.
- May consist of brief seizures over minutes, hours or days that may look like periods of confusion, cognitive changes or behavioral problems. It may be hard to identify individual seizures during this period without an EEG.
- These seizures often take days to weeks for the child to recover from and likely contribute to the progressive intellectual decline.
- Is seen in half of patients with LGS and is associated with frequent seizure activity on EEG that contributes to development delay and cognitive problems.
Why are seizure rescue plans needed?
Every patient with Lennox-Gastaut Syndrome should have a plan developed for what to do if they miss a dose of medication and for treatment of seizure emergencies. These plans can help everyone learn what seizure emergencies are and how to treat them if or when they occur. There are several treatments that can be used at home or in the community to prevent or stop an emergency situation.
- The most common seizure rescue medication is a form of benzodiazepine. This could include lorazepam, diazepam, rectal diazepam (Diastat AcuDial) or midazolam.
- If a patient has an implanted vagus nerve stimulator, the magnet would also be considered a rescue treatment.
- Seizure Response Plans (also called rescue plans or seizure action plans) should be tailored to the patient, with clear guidelines as to when to administer the medication or magnet, when to repeat the dose (if necessary) and when to go to the emergency department for further care.
- Having a plan in place will allow the best possible treatment of a seizure emergency and allow the family and the patient to have the best possible quality of life. Stopping frequent emergencies allows the patient to function better and may decrease further medical problems or complications.
Summary: Every person with LGS should have an emergency treatment plan that is tailored to their seizures. They must have access to emergent medical care and ideally a rescue therapy that can be given at home or in the community. Plans for responding to seizures and emergencies should be reviewed periodically with the family and communicated to their primary care physician and community caregivers.