Batten disease is a rare inherited neurological disorder which causes seizures, vision loss, and progressive motor and cognitive decline.

Most of the treatments available today for Batten disease are palliative, aimed at improving a patient’s quality of life and slowing disease progression.

Anticonvulsants can be taken daily to reduce the frequency and severity of seizures, but some anticonvulsants can also be used in emergencies to halt prolonged seizures. These emergency or rescue medications are taken in addition to daily anticonvulsant therapies and are not intended to replace them.

About rescue treatments

Rescue medications are fast-acting preparations of anticonvulsant therapies applied to control or end a seizure. Some rescue treatments can be given by parents or caretakers while others must be applied by emergency medical personnel.

Most seizures are brief, but a single seizure lasting longer than five minutes, or a cluster of seizures during which the patient does not regain consciousness (called status epilepticus) can be fatal and constitute a medical emergency. Rescue medications can be crucial in such cases.

If rescue medicines are prescribed, this information should be added to the patient’s emergency seizure management plan, along with instructions from the patient’s physician on when and how they should be used.

Types of rescue medications 

Rescue medicines are usually benzodiazepines, including diazepam, Ativan (lorazepam), and Versed (midazolam).

Rescue therapies cannot be in tablet form because patients usually cannot swallow a pill during a seizure. Therefore, alternative delivery methods are used, usually sublingual (under the tongue) or buccal (between cheek and gum). Some rescue medicines are given by injection; these are usually administered by trained medical personnel only. There is also a gel preparation of diazepam marketed under the brand name Diastat that can be given rectally. This is often used in children. Finally, clinical trials are ongoing for rescue medicines that can be given as a nasal spray.

Vagus nerve stimulation (VNS) can also be a type of rescue treatment for patients with an implanted device. These patients can use a magnet to activate the VNS generator at the time of the seizure. A parent or caregiver can also be trained to activate the VNS in case of emergency.

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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.