Adrenoleykodistrofiya
Contents:
Description:
Adrenoleykodistrofiya (melanokozhny leukodystrophy, Addison-Shildera's disease) - the degenerative disease of white matter of a brain connected with insufficiency of adrenal glands.
Adrenoleykodistrofiya's symptoms:
The signs of insufficiency of adrenal glands which are combined with intellectual, behavioural insufficiency (lability of mood, euphoria, a depression), a dysmnesia, increase in tendon jerks with existence of clonuses, gait disturbance, gradual development of spasticity (quadriplegia) are most expressed. Also the visual disturbance, pseudobulbar syndromes, an atrophy of optic nerves are observed. Focal attacks of spasms, paresis of outside muscles of eyes, a gomonimny hemianopsia, decrease in pupillary tests, paresis of a vertical look can be noted. The maintenance of cortisol in a blood plasma - is normal. Clinically And. it is similar to Shilder's disease. From the last it is distinguished by the perverted reaction to introduction of AKTG and detection of fatty acids with a long chain in fibroblasts.
Adrenoleykodistrofiya's reasons:
The mode of inheritance is recessive, linked to X-chromosome. This disease caused by euzymatic defect of exchange of fatty acids, perhaps belongs to the hereditary diseases connected with disbolism.
Adrenoleykodistrofiya's treatment:
Replacement therapy by hormones. Dietotherapy: enough proteins, fats, carbohydrates and vitamins, especially C and B1 (dogrose broth, blackcurrant, yeast); table salt in the increased quantity (20 g/days); salts of potassium reduce up to 1,5–2 g/days.
