Sulfatides in Metachromatic Leucodystrophy

C22 and C24 fatty acids kidney sulfatides in ML differ from those of normals by a lower content of mononnsaturated fatty acids, a higher proportion of hydroxy acids and a particularly high proportion of C22 fatty acids, mainly in the mono-hexose-sulfatide (Martensson et al. 1966). 

Since the carbohydrate moiety of the monohexose-sulfatide is galactose, as in normals, it appears certain that the stored sulfatide in ML is identical with the normal sulfatide. 

The clinical picture of ML including the neurological findings is not specific. Motor involvement, rigidity and spasticity as well as mental retardation and seizures are observed in a variety of brain diseases. Sometimes, the sequence of symptoms (Hagberg 1963) may aid in establishing the diagnosis. 

Biopsies from peripheral nerves (sciatic nerve) or from brain tissue may establish the diagnosis when a large amount of metachromatic material is found. The occurrence of small amounts is non-specific and may be seen in other demyelinating diseases (Einarson and Neel 1938 Jervis 1960). 

Diagnostic difficulties arise in subacute sclerosing encephalitis, although as a rule its course is more rapid and electroencephalographic changes are typical. Other virus encephalitides show unequivocally faster progression than ML. 

---