Neuronal ceroid lipofuscinosis is the general name for a family of at least eight genetically separate neurodegenerative lysosomal storage diseases that result from excessive accumulation of lipopigments in the bodys tissues these lipopigments are made up of fats and proteins. Neuronal ceroid lipofuscinosis ncl refers to a group of conditions that affect the nervous system signs and symptoms vary widely between the forms but generally include a combination of dementia vision loss and epilepsyalthough the ncls were historically classified according to their age of onset and clinical symptoms the most recent classification system is primarily based on their . Neuronal ceroid lipofuscinoses ncl refers to a group of rare disorders of the nerve cells ncl is passed down through families inherited these are the three main types of ncl adult kufs or parry disease juvenile batten disease late infantile jansky bielschowsky disease. The neuronal ceroid lipofuscinoses ncls form a special group within the inherited lysosomal storage disorders they are also collectively called batten disease according to the british neurologist and neuropathologist frederick batten 1865 1918 an early pioneer of the field who contributed to the identification of two forms of ncl see section 2. Batten disease is the name for a group of inherited nervous system disorders that most often begin in childhood and interfere with a cells ability to recycle a cellular residue called lipofuscin batten is commonly being used to describe the many forms of the disease called neuronal ceroid lipofuscinosis
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