《英文msh詞典》Neuronal Ceroid Lipofuscinoses
[入口詞] Neuronal Ceroid Lipofuscinoses
[主題詞] Neuronal Ceroid-Lipofuscinosis
[英文釋義] 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)