Benzodiazepines, also called “benzos,” are a class of medicines used to treat a variety of medical conditions due to their anti-anxiety, sedative, and anti-seizure properties.

How benzodiazepines work

In a healthy person, nerve cells communicate with one another via electrical signals that are passed on by changing the flow of charged particles in and out of the nerve cells. In Batten disease patients, sudden abnormal bursts of electrical signals cause seizures.

Benzodiazepines work by attaching to specific nerve cell receptors called gamma-aminobutyric acid-A (GABA-A) receptors. This attachment reduces the excitability of nerve cells in the brain and makes them resistant to stimulation, reducing the incidence and severity of seizures and bringing about a calming effect.

Benzodiazepines and Batten disease

Benzodiazepines are used in Batten disease as rescue medications to treat potentially fatal seizure emergencies.

Examples of benzodiazepines include Onfi (clobazam), Klonopin (clonazepam)Tranxene T-Tab (clorazepate), Valium (diazepam), Ativan (lorazepam), and Versed (midazolam). These medicines are injected into the bloodstream or placed under the tongue; Batten disease patients are unable to swallow during a seizure.

A specific form of midazolam called Buccolam (available only in the United Kingdom) is a solution that can be placed into the space between the gum and the cheek. A specific preparation of diazepam called Diastat is available as a gel that can be administered rectally. A midazolam formulation called Nayzilam (USL261), which can be administered nasally, is being investigated as a rescue medicine for the treatment of repetitive seizures.

Are benzodiazepines safe?

Benzodiazepines are safe to use if taken at low doses for short periods of time. They are potentially addictive when taken at high doses or for more than two weeks.

Side effects of benzodiazepines

Benzodiazepines frequently have side effects and sometimes appear to have negative effects on the general course of the disease. They may also lose their effectiveness with time.

The most common side effects include dizziness, unsteadiness, and problems with memory. Other common side effects include confusion, constipation, nausea, sexual dysfunction, unusually slow and shallow breathing, and vision problems (blurred or double vision).

When benzodiazepine medications are stopped abruptly, patients may develop withdrawal symptoms. Common symptoms include anxiety, cramps, convulsions, trouble sleeping, restlessness, muscle tension, irritability, sweating, tremors, and vomiting.

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