Rare Disorders of Childhood in the News - Batten Disease - featured October 20, 2010
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Batten disease is a rare, but fatal, neurodegenerative disorder that begins in childhood and ends up in the death of a child before they obtain the age of twelve. Its occurrence is one in 300,000 babies and no cure for the disease has been discovered.
Neuronal Ceroid Lipofuscinoses (NCL) is the general name of the family of eight neurodegenerative disorders in which neurons progressively lose structure and function and ultimately die. The name NCL is given to this family of neuron disorders due to the accumulation of lipopigments in the body tissues. Other NCL diseases include Parkinson’s, a disorder of the central nervous system, causing loss of speech and other functions, Alzheimer’s, loss of memory, and Huntington’s, which affects muscle coordination causing dementia.
Batten Disease was discovered by a British pediatrician Frederick Batten in 1903 and was named after him. It is an inherited disorder of the nervous system that begins in childhood and affects both male and female children. It is inherited as an autosomal recessive pattern, meaning, in order for a child to inherit this disease; he must receive defective genes from both the parents.
The initial symptoms of this disease include lost of vision and seizure, it can be observed very early through an eye examination and eye specialist can refer the patient to a neurologist. The early signs also include personality and behavior changes, slow learning and clumsiness and stumbling. Over time a loss of muscle control is observed as a pattern and dementia occurs.
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Learn More at Batten Disease Support and Research Association
Batten disease is a rare, but fatal, neurodegenerative disorder that begins in childhood and ends up in the death of a child before they obtain the age of twelve. Its occurrence is one in 300,000 babies and no cure for the disease has been discovered.
Neuronal Ceroid Lipofuscinoses (NCL) is the general name of the family of eight neurodegenerative disorders in which neurons progressively lose structure and function and ultimately die. The name NCL is given to this family of neuron disorders due to the accumulation of lipopigments in the body tissues. Other NCL diseases include Parkinson’s, a disorder of the central nervous system, causing loss of speech and other functions, Alzheimer’s, loss of memory, and Huntington’s, which affects muscle coordination causing dementia.
Batten Disease was discovered by a British pediatrician Frederick Batten in 1903 and was named after him. It is an inherited disorder of the nervous system that begins in childhood and affects both male and female children. It is inherited as an autosomal recessive pattern, meaning, in order for a child to inherit this disease; he must receive defective genes from both the parents.
The initial symptoms of this disease include lost of vision and seizure, it can be observed very early through an eye examination and eye specialist can refer the patient to a neurologist. The early signs also include personality and behavior changes, slow learning and clumsiness and stumbling. Over time a loss of muscle control is observed as a pattern and dementia occurs.
Read the Rest of this Article on StarCityNews.com
Learn More at Batten Disease Support and Research Association
Tags: Rare Diseases and Disorders News of the Week Batten Disease Newsletter 22 October 2010
