| 单词 | Batten Diseases |
| 释义 | Batten Diseases《英文msh词典》Batten Diseases [入口词] Batten Diseases [主题词] Neuronal Ceroid-Lipofuscinosis [英文释义] An inherited degenerative disease characterized by neuronal cytoplasmic inclusions which stain positively for ceroid and lipofuscin. Affected individuals develop retinal degeneration,seizures,myoclonus,ataxia,rigidity,and progressive dementia. Clinically there are four subtypes,divided by age of onset of symptoms: infantile (Santavuori-Haltia type),late infantile (Jansky-Bielschowsky type),juvenile (Spielmeyer-Vogt type),and adult (Kuf's disease). The late infantile and juvenile forms may both also be referred to as Batten Disease and Batten-Mayou Disease. (Adams et al.,Principles of Neurology,6th ed,p957) |
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