Urbach-Wiethe disease in O.R.L. practice (A clinical and histochemical study of the laryngeal lesions)

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Abstract
The positive reaction for succinic dehydrogenase and alpha esterase, shown by the characteristic deposits, could mean that they were partly formed of carbohydrate and lipid substances. The absence of superficial nerve fibers in all sections from patients examined, in comparison with those of the controls which showed nerve fibers cut in various directions was noteworthy. There is much controversy about neural affection in UWD. This might be due to the fact that different investigators examined different stages of the disease. The nerve lesions may not occur early and probably increase progressively to complete atrophy of the superficial nerve fibers, in advanced cases. This could also explain the choking attacks. The mucous glands were scanty and surrounded by the deposit of UWD. They showed decreased activity of the enzymes studied. This glandular affection might be the cause of the diminished lubrication which exaggerates the speech problem. Urbach-Wiethe disease [UWD] is a rare disorder, inherited as an autosomal recessive. The clinical data in 6 patients with well-established UWD were studied histopathologically and histochemically.

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