Leucodystrophy, metachromatic

A significant proportion of patients with an LSD have neurological involvement, which can be manifested as developmental delay and behavioral changes (Table 2). The presence of specific findings may suggest the diagnosis (e.g., leucodystrophy in patients with metachromatic leucodystrophy or globoid cell leucodystrophy cherry... 

Quader MA, Healy TE. Muscle fibrillation following thiopentone and pancuronium bromide. An association with metachromatic leucodystrophy. Anaesthesia 1977 32(7) 644-6. 

Anomalous accumulation of sulfatides was observed in the tissues (for example, nervous tissues, liver, and kidney) of patients having metachromatic leucodystrophy.113,114 Besides the normal ceramide monohexoside sulfate, a new glycosphingolipid sulfuric ester having a... 

The monocarboxylic acid connected in amide conjugation to the amino group of the sphingosine residue proved to consist mainly of the Ci8-fatty acid. The gangliosides of Niemann-Pick disease and metachromatic leucodystrophy differ from those of normal, infant and adult brains, in that C24-acids were not detected. The development of new ultramicro methods led to the exact (differential) determination of the constituents of these gangliosides215 of disease. 

It has been shown that the lipid composition of the brain of mentally diseased adults is often similar to that of normal infants much more sulphatide is found in cases of metachromatic leucodystrophy [69, 72, 177] large amounts of one ganglioside appear in Tay-Sachs disease [96, 177] and a greatly increased sphingomyelin content of the brain is found with the Niemann-Pick disease [164, 177]. The cerebroside sulphate esters of the brain of normal persons and of those suffering from metachromatic leucodystrophy have been isolated by TLC and shown to be identical [69]. 

Scholz s metachromatic leucodystrophy, which is characterized by abnormal depositions of sulfatides in the central nervous system (Jatzkewitz 1958, 1960, 1964). 

Hagberg, B., P. Sourander, L. Svennerholm, and H. Voss Late infantile metachromatic leucodystrophy of the genetic type. Acta paediat. (Uppsala) 49, 135 (1960). 

Clinical and laboratory diagnosis of metachromatic leucodystrophy. Cerebr. Palsy... 

Austin, J., D. McAfee, D. Armstrong, M. O Rourke, L. Shearer, and B. Bachhawat Abnormal sulfatase activities in two human diseases (metachromatic leucodystrophy and gargoylism). Biochem. J. 93, 15c (1964). 

Rouser, G., C. Galli, and G. Kritchevsky Lipid class composition of normal human brain and variations in metachromatic leucodystrophy, Tay-Sachs, Niemann-Pick, chronic Gaucher s and Alzheimer s disease. J. Amer. Oil Chemists Soc. 42, 404 (1965). 

Table 2 a. Composition of cerebral cortex in metachromatic leucodystrophy (Analysed material from frontal lobe. All figures are expressed as per cent of dry weight)... 

Table 3. Ganglioside patterns of grey and white matter in metachromatic leucodystrophy...

Fullerton, P. M. Peripheral nerve conduction in metachromatic leucodystrophy (sulphatide lipidosis). J. Neurol. Neurosurg. Psychiat. 27, 106 (1964). 

Hagberg, B. Clinical symptoms, signs and tests in metachromatic leucodystrophy. In Brain lipids and lipoproteins, and the leucodystrophies, p. 134. Eds J. Folch-Pi and H. J. Bauer. Amsterdam-London-New York Elsevier Publishing Co. 1963. 

Metachromatic leucodystrophy generalized lipidosis. Determination of sulfatides in... 

Helfant, M. H., M. Borjesson, and B. Hellstrom The value of urinary sediment. Examination as a screening method in suspected cases of metachromatic leucodystrophy. Acta paediat. (Uppsala) 51, 49 (1962). 

Jefferson, M. Late infantile metachromatic leucodystrophy.Proc. roy.Soc.Med.51,160 (1958)... 

Jervis, G. A. Infantile metachromatic leucodystrophy (Greenfield s disease). J. Neuropath exp. Neurol. 19, 325 (1960). 

Mossakowski, M., G. Mathieson, and N. Cumings On the relationship of metachromatic leucodystrophy and amaurotic idiocy. Brain 84, 585 (1961). 

---