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New Enzymatic Findings in Infantile Neuroaxonal Dystrophy

Overview
Journal Acta Neuropathol
Specialty Neurology
Date 1983 Jan 1
PMID 6410678
Citations 3
Authors
M Elleder
A Jirasek
Affiliations
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Abstract

Two siblings with infantile neuroaxonal dystrophy (INAD) exhibited highly increased activity of nonspecific esterase (resistant to physostigmine and sensitive to organophosphates) topically correlating with the morphological manifestations of the disease (spheroids), confined in those two particular cases to the central nervous system (CNS). This is demonstrated for the first time. NADH tetrazolium reductase was another enzyme noted with increased activity and similar topography. The lesions were devoid of any marked activity of typical mitochondrial (succinate and alpha-glycerolphosphate) dehydrogenases and lysosomal (acid phosphatase and beta-glucuronidase) enzymes. The diagnostic significance of monitoring non-specific esterase and NADH tetrazolium reductase activities in the diagnosis of INAD is discussed in particular.

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References
1.
KAMOSHITA S, NEUSTEIN H, LANDING B . Infantile neuroaxonal dystrophy with neonatal onset. Neuropathologic and electron microscopic observations. J Neuropathol Exp Neurol. 1968; 27(2):300-23. DOI: 10.1097/00005072-196804000-00008. View
2.
Jellinger K, Jirasek A . Neuroaxonal dystrophy in man: character and natural history. Acta Neuropathol. 1971; 5:Suppl 5:3-16. DOI: 10.1007/978-3-642-47449-1_2. View
3.
Yagishita S, Kimura S . Infantile neuroaxonal dystrophy (Seitelberger's disease). A light and ultrastructural study. Acta Neuropathol. 1975; 31(3):191-200. DOI: 10.1007/BF00684558. View
4.
Cowen D, OLMSTEAD E . Infantile neuroaxonal dystrophy. J Neuropathol Exp Neurol. 1963; 22:175-236. DOI: 10.1097/00005072-196304000-00001. View
5.
HUTTENLOCHER P, Gilles F . Infantile neuroaxonal dystrophy. Clinical, pathologic, and histochemical findings in a family with 3 affected siblings. Neurology. 1967; 17(12):1174-84. DOI: 10.1212/wnl.17.12.1174. View