Up to 96 percent of patients with Batten disease experience some type of sleep problem.

The most common sleep disorders experienced by those with Batten disease are waking in the middle of the night and nightmares. Children with Batten disease may be particularly resistant to returning to sleep, a phenomenon referred to as settling problems.

Why is sleep altered in Batten disease?

How we fall asleep and wake up are regulated by an internal clock. This clock controls our circadian rhythm — the physiological ups and downs of our day which are responsible for making us feel sleepier or more awake.

In Batten disease, there is an excessive accumulation of a substance known as lipofuscin inside cells, which is particularly harmful to the brain cells. The condition gets progressively worse over time and damages many parts of the brain, including the part that houses the internal body clock.

Circadian rhythms are strongly affected by exposure to light and darkness. Vision impairment, a common problem in Batten disease, is thought to predispose a patient to sleep problems. Other symptoms of Batten disease such as dementia, seizures, involuntary muscle contractions known as myoclonus, and mood changes may also affect the quality of sleep.

Signs of poor sleep

People who are not sleeping well may be more irritable, aggressive, depressed, and more prone to mood swings and making mistakes during the day. Hyperactivity, a decrease in attention span, and poor memory may also be present. In children, this may be reflected as disciplinary problems in school.

Managing sleep problems

Practicing certain habits that help improve the quality of sleep is paramount in managing sleep problems. This is called sleep hygiene, and includes limiting daytime naps to 30 minutes, exercising, avoiding caffeine, nicotine, and heavy foods such as spicy and fatty meals that trigger indigestion right before sleep, establishing a relaxing bedtime routine, and maintaining a comfortable sleep environment.

Sleeping in cots or having soft mattresses next to raised beds to cushion falls are important safety precautions, especially for patients who move around a lot during sleep due to myoclonus.

Taking medications to control the other symptoms of Batten disease may help ease sleep problems. Also, medications that are taken solely to improve sleep such as Catapres (clonidine) and Lyrica (pregabalin) may be beneficial. Melatonin can also encourage sleep.

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