Peripheral nerve conduction in metachromatic leucodystrophy (sulphatide lipidosis)

Peripheral Nerve Conduction in Metachromatic Leukodystrophy (sulphatide Lipidosis).

Peripheral neuropathy of metachromatic leucodystrophy: observations on segmental demyelination and remyelination and the intracellular distribution of sulphatide.

Peripheral nerve abnormalities in metachromatic leucodystrophy have been noted many times since Jacobi (1947) first reported metachromatic material in the sciatic nerve of her patient. The finding of metachromatic granules in a peripheral nerve biopsy, usually the sural nerve, has been recommended as a method of diagnosing this disease (Thieffry and Lyon, 1959; Hagberg, Sourander, and Thoren, 1...

Infantile Metachromatic Leucodystrophy.

Metachromatic leucodystrophy is an uncommon type of diffuse cerebral sclerosis. This condition is found at various ages, but it is convenient to classify cases into congenital, infantile, juvenile, and adult varieties. The infantile form, which presents a fairly constant clinical and pathological pattern, was first described by Greenfield (1933) who recorded two cases, and four further examples...

causing late infantile metachromatic leucodystrophy

Received 3 November 1993 Revised version accepted for publication 28 March 1994 Abstract Metachromatic leucodystrophy is an autosomal recessive degenerative disease of the nervous system caused by the deficiency of the lysosomal enzyme arylsulphatase A (ARSA). We report here on the high incidence of late infantile MLD among Muslim Arabs originating from Jerusalem, most probably because of a fou...

The Diagnosis of Metachromatic Leucodystrophy during Life.

Metachromatic leucodystrophy is deserving an increasing interest from neurologists and pediatricians, mainly because it is the only disease in the whole group of diffuse cerebral sclerosis amenable to an intra vitam diagnosis by means of bloodless procedures. The merit for this belongs to Austin 1 , who introduced a simple test to demonstrate metachromatic bodies in the urine sediment. Although...