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Batten disease

Batten disease Definition

Batten disease is an unusual, deadly, autosomal recessive neurodegenerative disorder which starts in early days of a person. The disease is also referred to as Spielmeyer-Vogt-Sjogren-Batten disease it is the most usual type of a group of disorders referred to as neuronal ceroid lipofuscinosis (or NCLs). History The disease is named after the British pediatrician Frederick Batten who initially explained about the Batten disease in 1903. It is the most usual form of a collection of disorders referred to as neuronal ceroid lipofuscinosis. Though Batten disease is commonly observed as the juvenile type of NCL several physicians refer to Batten disease to all types of NCL or ceroid lipofuscinosis.

Batten disease Prognosis

As time passes by, patients or usually children experience mental damage, severe seizures, and progressive sight loss and motor skills. Finally, children with Batten disease turn out to be blind, bedridden, and frantic. Batten disease is usually deadly when patients reach their late teens or twenties.

Batten disease Symptoms and Signs

Premature symptoms of this disorder commonly come into view about ages 4-10, with slow beginning of vision complications, or seizures or fits. Early signs include: Behavior changes Personality changes Recurring speech or echolalia Gradual learning or regression Stumbling Clumsiness Gradual head development in the infantile form Poor movement in lower extremities such as legs and feet Reduced body fat and muscle mass Curving of the spine Hyperventilation and/or breath-holding spells Teeth grinding Constipation As time passes, affected children undergo mental injury, deterioration seizures/fits, and progressive failure of sight, speech and motor skills. Batten Disease is life preventive ailment life anticipation differs basing on the kind or deviation.

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