Seizures occur in all types of Batten disease, with most patients experiencing more than one particular kind of seizure.
These can include what are known as Jacksonian seizures.
What are Jacksonian seizures?
Seizures are caused by the sudden and uncontrolled firing of electric signals by nerve cells in the brain that soon spreads to other areas.
In partial seizures, also called focal seizures, the uncontrolled signaling is limited to one area in the brain. In generalized seizures, in contrast, such abnormal electric activity occurs simultaneously across the brain.
A Jacksonian seizure is typically what is called a simple partial seizure, meaning that a person having this type of seizure does not lose consciousness or awareness, and excessive neuron firing only occurs in one part of the brain, so that abnormal movement or sensation is limited to one part or one side of the body.
It usually starts with twitching or tingling in a finger, toe, or a corner of the mouth, and then “marches” to affect larger parts of the body, such as the entire hand or foot. For this reason, a Jacksonian seizure is also known as a Jacksonian march. These types of seizures may also include other symptoms, such as licking lips, turning the head, moving the eyes, and muscle cramps.
However, in Batten patients and possibly others, Jacksonian seizure is also the name given to a partial seizure that starts in a small part of the brain but quickly spreads to other areas and become a generalized seizure, involving the entire body. The initial partial seizure may last for only a short time before a tonic-clonic or generalized seizure is evident.
Jacksonian seizure management
Because an individual is fully aware during a Jacksonian seizure, first aid is usually not required.
Caregivers and family members should keep a seizure management plan readily available that describes exactly what to do when a seizure occurs.
Anticonvulsants that are taken daily can reduce the frequency and severity of Jacksonian seizures, as they can other types of seizures. Valproic acid, Keppra (levetiracetam), Lamictal (lamotrigine), and Klonopin (clonazepam) are commonly used to treat Batten disease patients.
Rescue medicines, such as rectal diazepam or intranasal midazolam, can be used to help stop prolonged seizures.
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