Batten disease is the name given to a group of neurodegenerative disorders that usually begin during childhood. Generalized seizures are a type of seizure that can be experienced by patients with any type of Batten disease.
How generalized seizures develop
Nerve cells communicate with each other by firing tiny electrical impulses. Seizures happen when there is a sudden, rapid spike in electrical activity in the brain. The pattern of abnormal electrical activity (where it begins and how it spreads) determines the type of seizure experienced.
Generalized seizures are caused by abnormal electrical activity occurring simultaneously in all parts of the brain. This differs from partial seizures, which usually affect only one part of the brain.
Types and symptoms of generalized seizures
There are six types of generalized seizures: absence seizures, atonic seizures, myoclonic seizures, clonic seizures, tonic seizures, and tonic-clonic seizures.
Absence seizures, also known as petit mal seizures, typically cause patients to adopt a blank stare with or without blinking. This may commonly be mistaken for daydreaming, as these episodes are usually short, lasting less than 15 seconds.
Atonic seizures, or drop attacks, cause the muscles to go limp. Falls are a major concern in these types of seizures.
Myoclonic seizures are the opposite of atonic seizures and are characterized by brief, jerky movements in different muscles of the body. Clonic seizures cause similar muscle jerks and spasms in all parts of the body and may last longer.
In tonic seizures, patients stiffen up and often lose consciousness. Their eyes may roll back, and their neck and back may arch. Patients may make gargling noises as their chest muscles tighten, and their face may take on a bluish color as breathing becomes more difficult.
A tonic-clonic seizure, also known as a grand mal seizure, occurs when patients initially experience muscle stiffening followed by muscle jerking.
Managing generalized seizures
Medications known as anticonvulsants are the mainstay therapies to prevent seizures from occurring. Commonly used anticonvulsant medications in Batten disease are valproic acid and levetiracetam.
Patients who have frequent seizures may be prescribed benzodiazepines such as diazepam or midazolam. These medications, known as rescue medicines, are specifically meant to stop seizures once they have begun and should not be used regularly since they do not prevent seizures from happening.
A seizure management plan can help alert caregivers and bystanders about the patient’s condition, as well as what to do in an emergency situation.
Because generalized seizures are incapacitating and can happen at any time, caregivers should be alert and watch for triggers that may inadvertently set off a seizure.
In the event that a seizure begins, it is important to not restrain the patient in any way. This includes avoiding putting objects in his or her mouth. Padding the surrounding area with blankets and cushions and removing sharp objects can help avoid injuries during a seizure. A person may be drowsy after a seizure so a caregiver should stay with the patient until he or she is fully awake.
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