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Ultrastructural appearance of the abnormal intraneuronal deposits in different forms of the neuronal ceroid-lipofuscinoses (NCL)

Ultrastructural appearance of the abnormal intraneuronal deposits in different forms of the neuronal ceroid-lipofuscinoses (NCL)
The ultrastructural appearances of the abnormal intraneuronal deposits vary between different forms of the neuronal ceroid-lipofuscinoses. Four basic types can be delineated:
(A) Granular osmiophilic deposits are characteristic of the infantile and other forms of CLN1, ×10,000 magnification.
(B) Curvilinear profiles are typical of classic late infantile neuronal ceroid-lipofuscinosis CLN2, ×20,000 magnification.
(C) Fingerprint bodies are the predominant type of intraneuronal inclusions in the juvenile form CLN3, ×30,000 magnification.
(D) Many inclusions in the variant forms of late infantile neuronal ceroid-lipofuscinosis correspond to the rectilinear complex, ×15,000 magnification.
Reproduced from: Haltia M. The neuronal ceroid-lipofuscinoses. J Neuropathol Exp Neurol 2003; 62(1):1-13. By permission of Oxford University Press. Copyright © 2003.
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