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Batten Disease

S.K. Mohanasundari , S.K. Mohanasundari

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International Journal of Pediatric Nursing 3(1):p 57-62, January - April 2017. | DOI: DOI: http://dx.doi.org/10.21088/ijpen.2454.9126.3117.9

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Abstract

 Batten disease is extremely rare juvenile inherited disorder but it is the most common of neuronal ceroid lipofuscinoses (NCLs). It is fatal disorder that affects the nervous system. After 4 to 6 years of normal development, children with this condition develop progressive vision loss, intellectual and motor disability, behavior and personality changes, speech difficulties, and seizures. Most people with Batten disease die in their teens or early twenties. it can be diagnosed with help of Fluorescent deposits, Visual Evoked Potentials and Electroretinograms, Blood tests, Urine tests, Skin or tissue sampling, Electroencephalogram (EEG), Brain scans, Measurement of enzyme activity and DNA analysis. There is cure for Juvenile Batten disease. Therefore specialist symptom management and therapy is essential to assist in maintaining a good quality of life for children and their families. Holistic support for parents, siblings and wider family members is extremely important throughout their journey.

Keywords: Juvenile Batten Disease; Inherited Disorder; Neuronal Ceroid Lipofuscinoses (NCLs).

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DOI: DOI: http://dx.doi.org/10.21088/ijpen.2454.9126.3117.9

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