UKRAINS'KYI VISNYK PSYKHONEVROLOHII

The Scientific and Practical Journal of Medicine
ISSN 2079-0325(p)
DOI 10.36927/2079-0325

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

Type of Article

In the Section

Index UDK:

Abstract

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 worsening dramatically 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 method for assessment of the adequacy of PGT and monitoring of compliance of patients has led us to create the application Motus Test Set for a Tablet PC with a touch screen. Motus Test Set is aimed for testing of motor actions of the hands, since among the WD 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 between patients 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 PGT regime 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 almost 60 %, 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 was improved, 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.

Pages

References

  1. Pfeiffer R. F. Wilson’s disease // In book: Movement Disorders. Neurologic Principles and Practice (ed.: Watts R. L., Koller W. C.). N. Y. : McGraw-Hill Medical Publishers, 2004. P. 779—797.
  2. Pfeiffer R. F. Wilson’s Disease // Semin. Neurol. 2007. Vol. 27, No. 2. P. 123—132. DOI: https://doi.org/10.1055/s-2007-971173.
  3. Roberts E. A., Schilsky M. L. A practice guideline on Wilson disease // Hepatology. 2003. Vol. 37, No. 6. P. 1475—1492. DOI: https://doi.org/10.1053/jhep.2003.50252.
  4. Brewer G. J. Wilson’s Disease: A Clinician’s Guide to Recognition, Diagnosis, and Management. Boston : Kluwer Academic Publishers, 2001. 190 р.
  5. Wilson’s disease: clinical, genetic and pharmacological findings / L. Leggio, G. Addolorato, L. Abenavoli, G. Gasbarrini // Int. J. Immunopathol. Pharmacol. 2005. Vol. 18, No. 1. P. 7—14. DOI: https://doi.org/10.1177/039463200501800102.
  6. Hedera P. Update on the clinical management of Wilson’s disease // Appl. Clin. Genet. 2017. Vol. 10. P. 9—19. DOI: https://doi.org/10.2147/TACG.S79121.
  7. Hoogenraad T. Paradigm shift in  the treatment of  Wilson’s disease: zinc therapy now treatment of  choice  // Brain Dev. 2006. Vol.  28. P. 141—146. DOI: https://doi.org/10.1016/j.braindev.2005.08.008.
  8. Treatment of Wilson disease with ammonium tetrathiomolybdate / G. J. Brewer, F. Askari, M. T. Lorincz [et al.] // Arch. Neurol. 2006. Vol. 63. P. 521—527. DOI: https://doi.org/10.1001/archneur.63.4.521.
  9. Prognostic factors in patients presenting with severe neurological forms of Wilson’s disease / L. K. Prashanth, A. B. Taly, S. Sinha [et  ]  // QJM: An  International Journal of  Medicine. 2005. Vol. 98, No. 8. P. 557—563. DOI: https://doi.org/10.1093/qjmed/hci095.
  10. Sternlieb I., Scheinberg I. Penicillamine therapy for hepatolenticular degeneration // J.  Am. Med. Assoc. 1964. Vol. 189. P. 748—754. DOI: https://doi.org/10.1001/jama.1964.03070100042008.
  11. Worsening of neurologic syndrome in patients with Wilson’s disease with initial penicillamine therapy / G. Brewer, C. Terry, A. Aisen, G. Hill // Arch. Neurol. 1987. Vol. 44. P. 490—493. DOI: https://doi.org/10.1001/archneur.1987.00520170020016.
  12. Treatment of Wilson’s disease with tetrathiomolybdate: V. Control of free copper by tetrathiomolybdate and a comparison with trientine / G.   Brewer, F.  Askari, R.  B.  Dick [et  al.]  // Transl. Res. 2009. Vol.  154, No.  2. P. 70—77. DOI: https://doi.org/10.1016/j.trsl.2009.05.002.
  13. Touch operated keyboard. European Patent Office. Publication number: EP0185671B1. Published: 02.07.86 (Bull. 86/27). URL: https://patents.google.com/patent/EP0185671B1.
  14. Computerized Neuropsychological Assessment Devices: Joint Position Paper of the American Academy of Clinical Neuropsychology and the National Academy of Neuropsychology / R.  Bauer, G.  L.  Iverson, A.  N. Cernich [et  al.]  // Arch. Clinic. Neuropsychology. 2012. Vol.  27. P. 362—373. DOI: https://doi.org/10.1093/arclin/acs027.
  15. Automated Neuropsychological Assessment Metrics (v4) Traumatic Brain Injury Battery: military normative data / A. Vincent, T. Roebuck-Spencer, K. Gilliland, R. Schlegel  // Military Medicine. 2012. Vol.  177, No.  3. P. 256—269. DOI: https://doi.org/10.7205/milmed-d-11-00289.
  16. NIH toolbox for assessment of neurological and behavioral function / R. C. Gershon, M. V. Wagster, H. C. Hendrie [et al.] // Neurology. 2013. Vol. 80, No. 11. Suppl. 3. S2-6. DOI: https://doi.org/10.1212/WNL.0b013e3182872e5f.
  17. The computerized self test (CST): an interactive, internet accessible cognitive screening test for dementia / J. H. Dougherty, R. Cannon, C. R. Nicholas [et  al.]  // J.  Alzheimers Dis. 2010. Vol. 20, No. 1. P. 185—195. DOI: https://doi.org/10.3233/JAD-2010-1354.
  18. Computer assessment of mild cognitive impairment / J. Saxton, L. Morrow, A. Eschman [et al.] // Postgrad. Med. 2009. Vol. 121. P. 177—185. DOI: https://doi.org/10.3810/pgm.2009.03.1990.
  19. A. s. 78399 of Ukraine. Computer program "Cross-platform language ecosystem of software components Prototypus Operationis Testificationis pro Morbi Anime et Nervi" ("Ecosystem of software components POTesMANU") / D. O. Kutikov (Ukraine). No. 79539; declared 11.04.2018; reg. 19.04.2018; published on July 27, 2018, Bulletin. Copyright and Related Rights No. 49. С. 184.