The impact of non-motor disorders on the quality of life of patients with cervical muscular dystonia

Cervical dystonia (CD) has a wide range of non-motor (neuropsychiatric, sensory, dyssomnic) disorders. Relationships between dystonia, nonmotor manifestations of the disease and patients quality of life (QoL) require clarification.

Objective: to clarify the impact of dystonia, sensory, affective, behavioral disorders, quality of sleep and wakefulness on the quality of life of patients with CD.

Patients and methods. We examined 61 patients with CD (mean age – 48.03±11.49 years, mean duration of CD – 4.89±4.05 years). We used Toronto Western Hospital Spasmodic Torticollis Rating Scale (TWSTRS), Cervical Dystonia Quality of Life Questionnaire (CDQ-24, with five subscales: «stigma», «emotional wellbeing», «pain», «activities of daily living», «social/family life»), Spielberger–Khanin Inventory (STAI),Beck Depression Inventory (BDI), Barratt Behavioral Impulsivity (BIS-11), Yale–Brown Obsessive Compulsive Scale (Y-BOCS), Montreal Cognitive Assessment Scale (MoCA), Stroop Test (VST), 12 Word Memory Test, Pittsburgh Sleep Quality Assessment Scale (PSQI), Epworth Sleepiness Scale (ESS).

Results and discussion. There was a deterioration in all characteristics of QoL in patients with CD, largely in emotional well-being, stigmatization, pain syndrome (50% change from the maximum CDQ-24 score). A statistically significant moderate correlation was established between the total assessment of QoL and the severity of dystonia (r=0.35; p<0.01), a statistically significant strong correlation between the total assessment of QoL and depression index (r=0.73; p<0.001 ), moderate strength correlation – with indicators of anxiety (r=0.65; p<0.01), obsessivecompulsive disorders (r=0.61; p<0.01), sleep quality (r=0.52; p<0.001), impulsiveness in behavior (r=0.31; p<0.01), weak relationship with the assessment of executive cognitive functions (according to the Stroop test interference index; r=0.24; p<0.01). A statistically significant moderate correlation was found between the assessments of activity in everyday, social/family life and impulsiveness in behavior (r=0.33; p<0.001); between stigma, emotional well-being and an indicator of executive cognitive functions (r=0.3; p<0.05). There were no statistically significant relationships between indicators of affective, cognitive functions, behavioral disorders and the severity of dystonia. There was a moderate correlation between pain score and dystonia severity (r=0.35; p<0.01).

Conclusion. The decrease in QoL in patients with CD is largely due to affective, sensory, and behavioral disorders. Diagnosis and appropriate therapy of the non-motor aspects of the disease are required for adequately improvement of QoL of patients.

1. Defazio G. The epidemiology of primary dystonia: current evidence and perspectives. Eur J Neurol. 2010;17(Suppl. 1):9-14. doi: 10.1111/j.1468-1331.2010.03053.x

2. Chernukha TN. Distonicheskiye giperkinezy: patofiziologicheskaya kharakteristika, klinika, diagnostika, lecheniye: Avtoref. diss. … dokt. med. nauk [Dystonic hyperkinesis: pathophysiological characteristics, clinic, diagnosis, treatment: Abstract of the thesis diss. … doc. med. sci.]. Minsk; 2019 (In Russ.).

3. Novaretti N, Cunha ALN, Bezerra TC, et al. The Prevalence and Correlation of Non-motor Symptoms in Adult Patients with Idiopathic Focal or Segmental Dystonia. Tremor Other Hyperkinet Mov (N Y). 2019 Feb 4;9:596. doi: 10.7916/fhnv-v355

4. Charnukha TN, Likhachev SA. Peculiarities of a psychoemotional status of patients with dystonia. Nevrologiya i neyrokhirurgiya. Vostochnaya Yevropa (Minsk). 2016;6(4):506-15 (In Russ.).

5. Stamelou M, Edwards MJ, Hallett M, Bhatia KP. The non-motor syndrome of primary dystonia: clinical and pathophysiological implications. Brain. 2012 Jun;135(Pt 6):1668-81. doi: 10.1093/brain/awr224. Epub 2011 Sep 20.

6. Van den Dool J, Tijssen MA, Koelman JH, et al. Determinants of disability in cervical dystonia. Parkinsonism Relat Disord. 2016 Nov;32:48-53. doi: 10.1016/j.parkreldis.2016.08.014. Epub 2016 Aug 15.

7. Zalyalova ZA, Khafizova IF, Khayatova ZG. Pathophysiological bases of rehabilitation in cervical dystony. Farmateka. 2017;15:7-15 (In Russ.).

8. Eichenseer SR, Stebbins GT, Comella CL. Beyond a motor disorder: a prospective evaluation of sleep quality in cervical dystonia. Parkinsonism Relat Disord. 2014;20:405-8. doi: 10.1016/j.parkreldis.2014.01.004

9. Paus S, Gross J, Moll-Müller M, et al. Impaired sleep quality and restless legs syndrome in idiopathic focal dystonia: a controlled study. J Neurol. 2011;258:1835-40. doi: 10.1007/s00415-011-6029-6

10. Salouchina NI, Nodel' MR, Tolmacheva VA. Non-motor disorders in patients with muscular dystonia. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova. 2018;118(9):98-105. doi: 10.17116/jnevro201811809198 (In Russ.).

11. Tolmacheva VA, Nodel MR, Saloukhna NI. Non-motor disorders in cervical dystonia. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2018;10(3):135-40. doi: 10.14412/2074-2711-2018-3-135-140 (In Russ.).

12. Saloukhina NI, Nodel' MR. Poster presentation: Neuropsychiatric disorders in patients with spastic torticollis. Proceedings of the XI All-Russian Congress of Neurologists and the IV Congress of the National Stroke Association St. Petersburg, June 15–19, 2019. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova. 2019;119(5):556 (In Russ.).

13. Orlova OR, Timerbayeva SL, Khat'kova SE, et al. The use of the drug. Dysport (botulinum toxin type A) for the treatment of focal dystonia (medical technology). FS No2011/446 dated December 27, 2011 Moscow; 2012. 108 p. (In Russ.).

14. Yang J, Shao N, Song W, et al. Nonmotor symptoms in primary adult-onset cervical dystonia and blepharospasm. Brain Behav. 2016 Dec 18;7(2):e00592. doi: 10.1002/brb3.592

15. Likhachev SA, Chernukha TN, Navosha SA. Pain syndrome in the clinical picture of oromandibular dystonia. Rossiyskiy zhurnal boli. 2014;1(42):60 (In Russ.).

16. Ben-Shlomo Y, Camfield L, Warner T. What are the determinants of quality of life in people with cervical dystonia? J Neurol Neurosurg Psychiatry. 2002 May;72(5):608-14. doi: 10.1136/jnnp.72.5.608

17. Pekmezovic T, Svetel M, Ivanovic N, et al. Quality of life in patients with focal dystonia. Clin Neurol Neurosurg. 2009 Feb;111(2):161-4. doi: 10.1016/j.clineuro.2008.09.023. Epub 2008 Nov 7.

18. Page D, Butler A, Jahanshahi M. Quality of life in focal, segmental, and generalized dystonia. Mov Disord. 2007;22:341-7. doi: 10.1002/mds.21234

19. Druzhinina OA, Zhukova NG, Shperling LP. Quality of life at cervical distonia. Byulleten' sibirskoy meditsiny = Bulletin of Siberian Medicine. 2020;19(1):43-9. doi: 10.20538/1682-0363-2020-1-43-49 (In Russ.).

20. Ndukwe I, O’Riordan S, Walsh CB, Hutchinson M. Trust the Patient Not the Doctor: The Determinants of Quality of Life in Cervical Dystonia. Front Neurol. 2020 Sep 4;11:991. doi: 10.3389/fneur.2020.00991

21. Smit M, Kamphuis ASJ, Bartels AL, et al. Fatigue, Sleep Disturbances, and Their Influence on Quality of Life in Cervical Dystonia Patients. Mov Disord Clin Pract. 2016 Dec 5;4(4):517-23. doi: 10.1002/mdc3.12459. eCollection Jul-Aug 2017.

22. Ceylan D, Erer S, Zarifoglu M, et al. Evaluation of anxiety and depression scales and quality of LIFE in cervical dystonia patients on botulinum toxin therapy and their relatives. Neurol Sci. 2019 Apr;40(4):725-31. doi: 10.1007/s10072-019-3719-9. Epub 2019 Jan 18.

23. Barahona-Correa B, Bugalho P, Guimaraes J, Xavier M. Obsessive-compulsive symptoms in primary focal dystonia: a controlled study. Mov Disord. 2011 Oct;26(12):2274-8. doi: 10.1002/mds.23906. Epub 2011 Aug 9.

24. Monaghan R, Cogley C, Burke T, et al. Non-motor features of cervical dystonia: Cognition, social cognition, psychological distress and quality of life. Clin Park Relat Disord. 2020 Dec 7;4:100084. doi: 10.1016/j.prdoa.2020.100084

25. Berardelli I, Ferrazzano G, Pasquini M, et al. Clinical course of psychiatric disorders in patients with cervical dystonia. Psychiatry Res. 2015 Sep 30;229(1-2):583-5. doi: 10.1016/j.psychres.2015.07.076. Epub 2015 Jul 29.