Diazepam is an anticonvulsant used in emergency situations to treat prolonged seizures. The medicine is marketed as a tablet under the brand name Valium to treat anxiety disorders, alcohol withdrawal symptoms, and muscle spasms. Rectal diazepam, like Diastat, can be used to treat prolonged seizures caused by Batten disease.

How diazepam works

Batten disease is the name given to a group of conditions known as neuronal ceroid lipofuscinoses (NCLs). NCLs are caused by mutations in genes that contain the information necessary to produce proteins involved in processing and clearing cellular waste. These mutations cause cellular wastes called lipofuscins to accumulate in cells, resulting in neurodegeneration and causing serious seizures.

Diazepam is a type of benzodiazepine, a family of chemicals that bind to a specific receptor in the nervous system. While the exact mechanism of action for diazepam is not known, it is thought to work by enhancing the effect of an inhibitory neurotransmitter or cell signaling molecule called gamma-aminobutyric acid (GABA). GABA works to reduce the excitability of nerve cells, in effect “tuning down” the nerve signals.

Diazepam acts on the receptor that normally binds GABA, binding to a region called the benzodiazepine allosteric modulatory site. In this way, diazepam makes the GABA receptor more responsive and sensitive to the amount of GABA that is present. GABA binds to its receptor as usual, and tunes down the nerve signaling far more than usual, which reduces seizure severity, hopefully to the point that the seizure ends.

How is rectal diazepam used?

Most seizures are brief, lasting only a few minutes at most. Seizures lasting longer than five minutes, or clusters of seizures in which the patient does not regain consciousness in between, called status epilepticus, is considered a medical emergency, which can result in brain damage or bodily injury.

For prolonged seizures, doctors may prescribe either intravenous or rectal diazepam. In young children, doctors may prefer to use a rectal administration. To administer rectal diazepam, a needleless syringe is filled with a solution or gel of diazepam and inserted carefully into the rectum, which allows the rapid absorption of the medication.

Side effects of diazepam

Diazepam may cause side effects including drowsiness, dizziness, headaches, stomach pain, or upset stomach.


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.