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Patients with Wilson’s disease (WD) need lifelong pathogenetic therapy (PGT) to prevent progress of neurodegenerative processes in particular. However, a pharmacological management of copper balance is very diffi cult task because there are the possibility of worseningdramatically of a patient’s neurological status, the potential side eff ects of drugs, inadequacy of the PGT scheme, and the PGT regime violation or refusal of some patients. The necessity of eff ective methodfor assessment of the adequacy of PGT and monitoring of compliance of patients has led us to create the application Motus TestSet for a Tablet PC with a touch screen. Motus Test Set is aimed for testing of motor actions of the hands, since among theWD neurological manifestations, tremor and hyperkinesis are the most common and sensitive to therapy. Motus Test Set consists of four kinds of tasks (drawing of the cube and tracts in labyrinths, a continuous following a moving object, "piano") which are performed by fi ngers.Hand motor activity was assessed by means of Motus Test Set at the beginning and at the end of hospitalization in 30 WD patients with different PGTs. The usage of the Motus Test Set in most patients (86.67 %) did not cause any problem, as well as did not aff ect their psychosomatic conditions. The computerized testing has defi ned diff erences betweenpatients with various PGT schemes, both on chronometric indices and quality of drawing. The best quality of drawings was demonstrated by patients with Zincteral monotherapy. The patients with the Cuprenyl dose of 1500 to 2250 mg per day and half of the patients with PGTregime violation had the worst drawing. In patients after a lowering dosage/replacement of Cuprenyl, the time of contact with the screen increased, the drawing time of the cube was reduced by almost60 %, the quali ty of graphic images improved signifi cantly. In patients, who returned to PGT, the time of contact with the PC screen in labyrinths tasks signifi cantly increas, the quality of the tracts drawn wasimproved, the algorithm of a cube drawing was restored. Therefore, the Motus Test Set is a useful tool to assess the PGT adequacy / effi cacy and the compliance of WD patients.

Monitoring of adequacy/efficacy of pathogenetic therapy in patients with Wilson’s disease by means of computerized testing of hand motor activity