People with Batten disease often experience symptoms such as muscle weakness, abnormally stiff and rigid muscles (spasticity), involuntary movements (tremors or jerky muscle movements known as myoclonus), and poor coordination (ataxia).

These symptoms usually begin during early childhood, except in rare cases of adult Batten disease. How and when these symptoms change or worsen are not predictable, although they tend to get worse with time. It also is common for children to lose previously learned skills such as walking and crawling.

Physiotherapy and occupational therapy may help ease these symptoms and improve patients’ quality of life. These interventions should be started as soon as possible to maintain existing function and delay complications.

Physiotherapy

In Batten disease, there is a loss of gross motor skills, which are movements that allow a child to crawl, walk, sit, and run. Because of a loss of balance and poor coordination, patients may appear to be clumsier and have changes in the way they walk or in their posture.

Physiotherapists are able to help overcome problems with gross motor skills by implementing exercises that help strengthen muscles and improve balance in patients with Batten disease.

Physiotherapists also may recommend and use adaptive equipment such as splints and braces to help correct posture and make it easier for the patient to move around.

Joint contractures, where there is a permanent stiffening of an underused joint, may develop as the result of muscle spasticity. This can be prevented with stretching, heat/cold therapy, and ultrasound and electrical stimulation, all of which can be performed by a physiotherapist. These same methods also may be able to relieve pain caused by stiff muscles.

Physical therapy should be guided by the patient’s own limits, especially in children where too much stress may trigger seizures, another symptom common in people with Batten disease.

Occupational therapy

Patients with Batten disease also may have difficulties performing fine motor skills, which are actions involving smaller muscle groups such as those in the fingers. The ability to perform activities of daily living such as writing, dressing, bathing, and feeding may be affected.

Occupational therapists are able to help patients improve their fine motor skills and coordination, and maintain their dexterity through training exercises. These exercises work on strengthening hands, wrists, and fingers so that patients are able to develop better hand grip and control.

Occupational therapists also may be able to suggest adaptive equipment that enables patients to live and function independently. Some examples include utensils with bigger handles or weighted utensils to make scooping up food easier and using velcro and zippers with clothing and shoes rather than laces and buttons to help overcome poor coordination.

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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.