Hepato-lenticular degeneration

Hepato-lenticular degeneration (synonym Wilson disease-Konovalova) is a progressive disease characterized by combined liver and the brain. The disease is associated with hereditary disorders of the metabolism of proteins and copper. Pathomorphological find cirrhosis and gross changes of vessels and cells of the basal ganglia and the cerebral cortex. The copper content in the brain and liver are enlarged in the blood is reduced; increased excretion of copper and amino acids. Often found family cases.
Abdominal form the gepatolentikuliarnaya degeneration. Ill usually in childhood or young age. Develop dyspepsia, jaundice. The disease is type in destroy dystrophy of the liver, ending death even before the development of symptoms of the nervous system.
Early form of hepato-lenticular degeneration usually begins in 8-12 years. Characterized by a heavy defeat liver, hyperkinesia, rapidly progressive stiffness and increase muscle tone, leading to immobility of patients. Broken speech, there are violent laughter and crying. For malignant; disease duration is 2-3 years.
Akinetic-rigid form begins at the age of 15-25 years. Dramatically increased muscle tone, krupnorazmernye, shaking hyperkinesia, aggravated by anxiety and voluntary movements. The liver is affected to a lesser extent and course of the disease less malignant than in an early form.
Shaking form begins at the age of 20-30 years. Slow progressive development krupnomasshtabnogo shake, gradually extending to all extremities, head, torso. In the late stages of the disease increases muscle tone. The liver is affected moderately, the disease long, possible remission.
Ekstrapiramidna-cortical shape begins as one of the above forms, but soon join the rapidly developing paresis, paralysis. Often, seizures, there are focal symptoms of brain lesions.
When hepato-lenticular degeneration can be a mental disorder in the form of lower memory, euphoria, dementia. Typical symptoms include so-called ring Kayser - Fleischer - deposition greenish-brown pigment on the periphery of the iris of the eye. The prognosis is unfavorable.


Fig. Pigment ring on cornea when hepato-lenticular degeneration.

Treatment is directed at regulating the exchange of copper and proteins. Applies a diet that includes dairy and vegetable products. In the hospital under the supervision of a physician appointed drugs, promotes the removal of excess amounts of copper: unithiol and diablocam acid courses within 1 month by the scheme in doses appropriate to their age. Along with that it is expedient the use of amino acid - methionine, leucine, etc. at doses of 0.5 g three times a day. To reduce muscle tone appoint tropatsin, mellitin in doses of 0.01-0.02 g two to three times a day. Are also used antihistamine drugs (Dimedrol, suprastin, pipolfen). Is vitamin - b complex vitamins b, PP, C. Treatment is carried out courses for a long time.