Results of a questionnaire in 100 ALS patients and 100 control cases.

A questionnaire with 54 relevant questions was studied in 100 ALS patients and 100 age and sex matched control cases. The findings were discussed in relation to 7 questionnaires in the literature. No differences were found between ALS patients and controls. Especially the increase of frequency of gastrointestinal operations, previous poliomyelitis, toxic metal exposure frequency, antecedent fractures and excess milk drinking as described in the literature were not confirmed.

Brain gangliosides in the presenile dementia of Pick.

Histochemical analysis of frontal and temporal lobes from four patients with Pick presenile dementia indicated intracellular and extracellular deposits of gangliosides. Thin layer chromatography of gangliosides disclosed the presence of an unknown ganglioside, a decrease of N-acetylgalactosamine-GDla and an increase of GTla and/or GD2 in white matter of Pick brain. Chromatography of gray matter and quantitation of the sialic acid content yielded results similar to controls. It is suggested that degradation and removal of gangliosides is incomplete in Pick disease.

Experimental amyotrophic lateral sclerosis in the guinea-pig.

In guinea-pigs a diet which was deficient in ascorbic acid provoked neurogenic atrophy of the muscles, degeneration of the anterior horn motor cells and demyelination of the pyramidal tract. Muscles showed a decrease of cytochrome and cytochrome c oxidase. Subcutaneous haemorrhages were also found, particularly around the knee joints. The haemorrhages and the histopathology of the muscles and spinal cord were prevented by a high dosage of ascorbic acid. In animals with an ascorbic acid deficiency, histopathology of muscles and spinal cord were also prevented by L-cysteine, L-methionine and L-hydroxyproline. Subcutaneous haemorrhages were not prevented by these supplements.

A histochemical study in Huntington's disease and control cases.

1. Extracellular deposits of cerebrosides and free fatty acids were found in the formaldehyde fixed frozen sections of the frontal lobe in 8 cases of Huntington's disease, in one case of the infantile form of Gaucher's disease, 2 cases of Krabbe's globoid cell leucodystrophy, 2 cases of metachromatic leucodystrophy, one case with multiple sclerosis, 2 cases with cerebral contusion and one case with bacterial meningitis. 2. The cerebroside deposits were present in the white matter as well as in the grey matter. 3. The significance of these findings in relation to their etiology is discussed.

Increased T3 deiodination in dystrophic and neonatally denervated mouse muscle.

T3, free iodide levels and morphological features were compared in normal, 129 ReJ dy/dy dystrophic and neonatally denervated skeletal muscles of mice, killed 6 hr after a single intraperitoneal injection of iodine-labelled hormone. Both the dystrophic andthe denervated muscles were found to contain equal amounts of T3 but more iodide than the normal specimens. Increased iodide levels are considered to reflect augmented T3-catabolism by muscle dehalogenase and may indicate low free hormone levels in the soluble enzyme fraction of the cell, despite its normal overall T3 content. The close light-microscopic resemblance of 129 ReJ dy/dy dystrophy and neonatal denervation is confirmed. The possibility is discussed that one or several of the muscular dystrophies are forms of target organ hypothyroidism.

Sarcoplasmic masses. Enzyme histochemistry and autoradiography.

A comparative enzyme-histochemical study was made of the sarcoplasmic masses in a muscle biopsy specimen from a patient with myotonic dystrophy and those in the jaw-muscle of the Rana temporaria tadpole. The enzyme-histochemical patterns of the two methods proved to be identical. An autoradiographic study was made of the sarcoplasmic masses in the R temporaria tadpole with DL-thyroxine-2-14C and levomethionine 35S. The radioactivity labeled compounds were found to be stored or incorporated mainly in the myofibrillae, and virtually not at all in the sarcoplasmic masses.