Seizures occur in all types of Batten disease. When a seizure lasts for more than five minutes or a patient experiences numerous seizures without recovering between them, the patient is said to be experiencing status epilepticus. It usually occurs without warning, and the patient usually does not remember the event.

Types of status epilepticus

Any type of seizure can become status epilepticus, when the seizure lasts longer than usual or is repetitive. The most common types of seizures in Batten disease are tonic-clonic seizures, myoclonic seizures, absence seizures, and partial seizures.

During generalized tonic-clonic seizures, the body first becomes stiff. This is followed by trembling and jerking.

Myoclonic seizures are sudden brief jerks.

An absence seizure lasts for a few seconds, and the individual is unresponsive to external stimuli.

Partial seizures are seizures that are limited to one area of the brain. They may be simple (simple partial seizures), with jerking of the extremities and turning eyes and head to one side, or complex (focal impaired-awareness seizures), and involve random movements such as picking at things, chewing, and removing clothes.

Management of status epilepticus 

Status epilepticus can be life-threatening and is considered a medical emergency. It is essential to have a seizure response plan in place. This should include exactly which steps should be taken when a seizure occurs and information for the caregiver about the medications the patient is taking.

Long-lasting seizures are less likely to stop on their own without medication. First aid can help keep seizures under control. This includes keeping calm and staying with the person until the seizure is over. The individual experiencing the seizure should not be restrained or inhibited in his or her movements. Removing nearby objects can help prevent injuries.

The time when the seizure starts should be recorded. If the seizure lasts longer than two minutes, it is likely to be status epilepticus, and an ambulance should be called. Rescue medications can be used to shorten or stop intense seizures. Fast-acting anticonvulsants, such as intranasal midazolam or rectal diazepam are commonly used.

Anticonvulsants can also help reduce the frequency and severity of seizures. Commonly used anticonvulsants in Batten disease include valproic acid, levetiracetam, clonazepam, and lamotrigine.

The kind of anticonvulsant selected depends on the seizure type. Because Batten disease patients may experience different types of seizures, it can take a long time to find the right combination of anticonvulsant therapy needed.

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