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Diagnostic significance of myeloperoxidase assay in neuronal ceroid-lipofuscinoses (Batten-Vogt syndrome)

Departments of Neurology and Nruropathology, Universities of Göttingen and Hamburg, West Germany.

In 13 patients with morphologically established juvenile neuronal ceroid lipofuscinosis and 13 controls, the activity of leukocyte peroxidase (myeloperoxidase) was determined under various conditions: Measurement of water-soluble enzyme, the buffer-soluble enzyme, and the 1euk.ocyte homogenate; application of phosphate buffer (pH 7.0) and borate buffer (pH 7.6); employment of 9 mM, 28 mM, and 55 mM p-phenylenediamine as hydrogen donor; and measurement of the specific enzyme extinction at 10,60, and 180 seconds. A significant difference between mean values for patients and controls could not be established. In both groups, single individuals exhibited definitely increased or reduced enzyme concentrations, leaving us, however, without an adequate explanation for these observations. Our studies indicate that determination of myeloperoxidase has no value in establishing the diagnosis of neuronal ceroid-lipofuscinoses.

Reprint requests should be addressed to Dr. Goebel, Department of Neuropathology, University of Göttingen, Robert-Koch-Str. 40, D-3400 Göttingen, West Germany.

This study was supported by Grant SFH 33 from the Deutsche Forschungsgemeinschaft.

Accepted for publication June 2. 1977.

This article has been cited by other articles:

				H. H. Goebel Topical Review: The Neuronal Ceroid-Lipofuscinoses J Child Neurol, November 1, 1995; 10(6): 424 - 437. [Abstract] [PDF]