Batten disease is a rare, inherited neurodegenerative condition. Seizures occur in all types of this disease.

Brain cells communicate with each other by sending electric signals. Seizures occur when the electrical firing of neurons gets out of control.

Types of seizures

Seizures in Batten disease can be divided into three groups: generalized seizures, partial seizures, and status epilepticus.

Generalized seizures

There are six different forms of generalized seizures: absence or petit mal seizures, myoclonic seizures, atonic or drop seizures, tonic seizures, clonic seizures, and tonic-clonic seizures.

Absence or petit mal seizures last only for 30 seconds, during which the individual is not responsive and appears “spaced-out.”

Myoclonic seizures are defined by sudden, brief jerks that might be limited to the face and extremities, or involve the whole body.

When atonic or drop seizures occur, the individual suddenly loses their muscle tone and might fall.

Tonic seizures are characterized by stiffening of the body, enlarged pupils, eyes that roll up or to the side, shallow breathing, and a slow heart rate.

In clonic seizures, the entire body trembles and jerks. Once the seizure is over, the body relaxes and goes limp.

Tonic-clonic seizures start with a tonic seizure, followed by clonic seizures.

Partial seizures

Partial seizures are caused by electric signals being out of control in only a part of the brain. Partial seizure are further classified as simple partial seizures, complex partial seizures, and Jacksonian seizures.

A simple partial seizure is limited to one side of the body and may include jerking of the extremities, tingling, turning eyes and head to one side, and a fearful look on the face.

Complex partial seizures start with blank staring, followed by random movements such as picking at things, removing clothes, and chewing. The individual might appear afraid and start running.

Jacksonian seizures start as a partial seizure and then become generalized.

Status epilepticus

Repetitive seizures with only short intervals in between seizures, or continuous seizures, are called status epilepticus. This kind of seizure requires immediate medical attention.

Seizure management

Anticonvulsants are taken daily to reduce the frequency and intensity of seizures.

When a seizure occurs, some first aid steps can help keep the situation under control. These include staying calm and staying with the individual until the seizure is over. The patient should be in a comfortable position, but not restrained or inhibited in his or her movements. It can also help to move nearby objects away to prevent injury and to make sure that no onlookers are around.

If the seizures are very intense, rescue medicines can help to stop or shorten their duration.

It is essential to have an emergency seizure management plan in place, which describes exactly what to do when a seizure occurs and when to call for help.

What not to do

Nothing should ever be placed in the mouth of the patient during a seizure, and especially no one’s fingers. Putting an object in a person’s mouth during a seizure is dangerous because the person can break or swallow an object or its pieces, or break their teeth against it.

Movement should not be restrained as this might lead to injury, confusion, and make the person aggressive.

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Batten Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.