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Peripheral Nerve Changes in the Diagnosis of Metachromatic Leucodystrophy
Author(s) -
HAGBERG BENGT,
SOURANDER PATRICK,
THORÉN LARS
Publication year - 1962
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1962.tb08659.x
Subject(s) - pathognomonic , metachromatic leukodystrophy , pathology , metachromasia , medicine , perineurium , biopsy , peripheral nerve , sural nerve , anatomy , disease , staining
Summary A histopathological study was made on peripheral nerves and spinal roots from biopsies and autopsies of 8 cases with metachromatic leucodystrophy (sulphatide lipidosis), 4 cases of globoid cell leucodystrophy (Krabbe's disease), 9 cases with other progressive neurological disorders of infancy and childhood and 4 controls. The sural nerve was found to give satisfactory biopsy specimens and a suitable biopsy technique is described. At histological examination, brown metachromatic deposits were revealed by the cresyl violet‐acetic acid method of v. Hirsch & Peiffer in all cases with metachromatic leucodystrophy, but were never observed in any of the other cases examined. The granular metachromatic material was localized mainly to Schwann cells and histiocytes which were accumulated in clusters around capillaries of the endo‐ and perineurium. For diagnostic purposes the most reliable and so far pathognomonic change in peripheral nerve biopsy specimens was considered to be the demonstration of brown metachromatic granular deposits in perivascular phagocytes. It is concluded that histological examination of peripheral nerves can serve as a useful supplementary tool in the diagnosis of this genetically transmitted degenerative disorder during life.