Sensory Organization of Postural Balance in a Child with Joubert Syndrome: A Case Report

Document Type : Case Report

Authors

Department of Occupational Therapy, School of Rehabilitation Sciences, Iran University of Medical Sciences, Tehran, Iran

Abstract

Purpose:
Joubert syndrome with hypotonia and ataxia due to the defect in cerebellar vermis causes severe balance and coordination problems and this may prevent therapists to achieve functional goals. Therefore, one of the important purposes in rehabilitation is improving postural balance in these children. In this study, the effect of multi-sensory intervention on a child with Jubert's syndrome was investigated.
Case report:
Subject was a 6-year-old girl with Joubert syndrome. The program for strengthening the sensory organization of balance using tilt-board was run in 16 30-40-minutes sessions. The outcome was measured by Pediatric Balance Scale (PBS) and Clinical Test of Sensory Interaction for Balance (CTSIB) at first, fourth, eighth, twelfth, and sixteenth sessions. Based on the assessments, the balance time of standing changed from 5.6 seconds to more than 5 minutes, 3.5 to about 12 seconds, 0 to 12 seconds, and 0 to about 5 seconds on the firm surface/opened-eyes, firm surface/closed-eye, foam surface/opened-eye, and foam surface/closed-eyes, respectively. The PBS score increased from 50% to 84% of the total score that shows 34% improvement in dynamic balance.
Conclusion:
Sensory organization intervention for balance can play an effective role to improve postural balance in children with neurological deficits. Maintaining posture on the unstable surface/closed-eyes that there is no visual input and perturbed somatosensory input is the most difficult condition to process sensory information for postural balance. To enhance balance in children with Joubert syndrome, because of visual deficits, it is necessary to intervene in the visual system with greater accuracy and finer grading to facilitate the ability of the nervous system to process visual inputs.

Keywords

References

  1. Brancati F, Dallapiccola B, Valente EM. Joubert Syndrome and related disorders. Orphanet J Rare Dis 2010; 5(20): 1-10.
  2. Yachnis AT, Rorke LB. Neuropathology of Joubert syndrome. J child neurol. 1999; 14(10): 655-9.
  3. Bulgheroni S, D'arrigo S, Signorini S, Briguglio M, et al. Cognitive, adaptive, and behavioral features in Joubert syndrome. Am J Med Genet A 2016; 170(12): 3115-3124.
  4. Fong SS, Lee VY, Pang MY. Sensory organization of balance control in children with developmental coordination disorder. Res Dev Disabil 2011; 32(6): 2376-2382.
  5. Daneshmandi H, Barati A, Ahmadi R. The effect of core stabilization training program on the balance of mentally retarded educable students. J Rehabil. 2013; 14(3): 16-24. [Persian]
  6. Sharif-Moradi K, Farah-Pour N. Comparison of the balance performance of the children with spastic cerebral palsy before and after exercise therapy program. J Rehab. 2006; 7(1): 22-28. [Persian]
  7. Massion J, Woollacott MH. Posture and equilibrium. In: Bronstein AD, Brandt T, Woollacott MH, editors. Clinical Disorders of Balance, Posture and Gait. second ed. USA: Oxford University Press Inc.; 2004: 1-19.
  8. Cherng R-J, Su F-C, Chen J-JJ, Kuan T-S. Performance of Static Standing Balance in Children With Spastic Diplegic Cerebral Palsy Under Altered Sensory Environments1. Am J Phys Med Rehab 1999; 78(4): 336-343.
  9. Jirikowic TL, McCoy SW, Lubetzky-Vilnai A, Price R, et al. Sensory control of balance: a comparison of children with fetal alcohol spectrum disorders to children with typical development. J Popul Ther Clin Pharmacol 2013; 20(3): e212-e228.
  10. Deconinck FJ, De Clercq D, Van Coster R, Oostra A, et al. Sensory contributions to balance in boys with developmental coordination disorder. Adapt Phys Activ Q 2008; 25(1): 17-35.
  11. Gupta S, Rao Bk, Kumaran S. Effect of strength and balance training in children with Down’s syndrome: a randomized controlled trial. Clin Rehabil. 2011; 25(5): 425-432.
  12. Ledebt A, Becher J, Kapper J, Rozendaal RM, et al. Balance training with visual feedback in children with hemiplegic cerebral palsy: effect on stance and gait. Motor Control 2005; 9(4): 459-468.
  13. Shumway-Cook A, Hutchinson S, Kartin D, Price R, Woollacott M. Effect of balance training on recovery of stability in children with cerebral palsy. Dev Med Child Neurol 2003; 45(9): 591-602.
  14. Fong SS, Guo X, Cheng YT, Liu KP, et al. A novel balance training program for children with developmental coordination disorder: a randomized controlled trial. Medicine 2016; 95(16): 1-11.
  15. Lee H-Y, Cherng R-J, Lin C-H. Development of a virtual reality environment for somatosensory and perceptual stimulation in the balance assessment of children. Comp Biol Med 2004; 34(8): 719-733.
  16. McCoy SW, Jirikowic T, Price R, Ciol MA, et al. Virtual sensorimotor balance training for children with fetal alcohol spectrum disorders: feasibility study. Phys 2015; 95(11): 1569-1581.
  17. Silkwood-Sherer DJ, Killian CB, Long TM, Martin KS. Hippotherapy—an intervention to habilitate balance deficits in children with movement disorders: a clinical trial. Phys 2012; 92(5): 707-717.
  18. Fong SS, Guo X, Liu KP, Ki W, et al. Task-specific balance training improves the sensory organisation of balance control in children with developmental coordination disorder: a randomised controlled trial. Sci rep 2016; 6: 201-208.
  19. Fong SS, Tsang WW, Ng GY. Taekwondo training improves sensory organization and balance control in children with developmental coordination disorder: randomized controlled trial. Res Dev Disabil 2012; 33(1): 85-95.
  20. Ghoochani BZ, Hosseini S, Derakhshanrad S, Sherafat S, et al. [Vestibular Stimulation: Intervention Protocols, Efficacy and Therapeutic Application in Children Rehabilitation (a Narrative Study)]. Journal of Paramedical Sciences and Rehabilitation 2017; 6(4): 87-100. [Persian]
  21. Franjoine MR, Gunther JS, Taylor MJ. Pediatric balance scale: a modified version of the berg balance scale for the school-age child with mild to moderate motor impairment. Pediatr Phys Ther 2003; 15(2): 114-128.
  22. Richardson PK, Atwater SW, Crowe TK, Deitz, JC . Performance of preschoolers on the pediatric clinical test of sensory interaction for balance. Am J Occup Ther 1992; 46(9): 793-800.
  23. Crowe TK, Deitz JC, Richardson PK, Atwater SW. Interrater reliability of the pediatric clinical test of sensory interaction for balance. Phys Occup Ther Pedi 1991; 10(4): 1-27.
  24. Lotfi Y, Javanbakht M, Sayaf M, Bakhshi E. Modified clinical test of sensory interaction on balance test use for assessing effectiveness of Epley maneuver in benign paroxysmal positional vertigo patients rehabilitation. Auditory and Vestibular Research. 2017; 27(1): 12-18.
  25. Horn LB, Rice T, Stoskus JL, Lambert KH, et al. Measurement characteristics and clinical utility of the clinical test of sensory interaction on balance (CTSIB) and modified CTSIB in individuals with vestibular dysfunction. Archives of physical medicine and rehabilitation. 2015; 96(9): 1747-1748.
  26. Rolley SS, Jacobs SE. Sensory Integration. In: Crepeau EB, Cohen ES, Schell ABA, eds. Willard and Spackman’s Occupational Therapy. 11th ed. Philadelphia, Lippincott Williams & Wilkins; 2009: 792-817.
  27. Cano-De-La-Cuerda R, Molero-Sánchez A, Carratalá-Tejada M, Alguacil-Diego IM, Molina-Rueda F, Miangolarra-Page JC, Torricelli D. Theories and control models and motor learning: clinical applications in neurorehabilitation. Neurología (English Edition) 2015; 30(1): 32-41.
  28. Santoso M, Phillips D. A Study on Visual Perturbations Effect on Balance in a VR Environment.  Cases on Immersive Virtual Reality Techniques: IGI Global (chaptrr 4). In: Yang KCC, Cases on immersive virtual reality techniques. The University of Texas at El Paso, USA; 2019: 67-88.
  29. Streepey JW, Angulo-Kinzler RM. The role of task difficulty in the control of dynamic balance in children and adults. Hum Mov Sci 2002; 21(4): 423-438.
  30. Swan L, Otani H, Loubert PV. Reducing postural sway by manipulating the difficulty levels of a cognitive task and a balance task. Gait Posture 2007; 26(3): 470-474.
  31. Szczęsna A, Pawlyta M, Błaszczyszyn M, Strzelczyk A. Virtual reality application to study the visual influences on human balance. In: International Conference on Man–Machine Interactions 2017 Oct 3 (pp. 102-112). Springer, Cham.
  32. Schmuckler MA, Tang A. Multisensory factors in postural control: Varieties of visual and haptic effects. Gait & posture. 2019 Jun 1;71:87-91.
  33. Christie A. A meaningful occupation: The just right challenge. Aust Occup Ther J 1999; 46(2): 52-68.
  34. Okimoto AM, Bundy A, Hanzlik J. Playfulness in children with and without disability: Measurement and intervention. Am J Occup Ther 2000; 54(1): 73-82.
  35. Ríos-Rincón AM, Adams K, Magill-Evans J, Cook A. Playfulness in children with limited motor abilities when using a robot. Physical & occupational therapy in pediatrics 2016; 36(3): 232-246.
  36. Westcott SL, Burtner P. Postural control in children: implications for pediatric practice. Phys occupa ther pedi 2004; 24(1-2): 5-55.
  37. Cherng RJ, Chen JJ, Su FC. Vestibular system in performance of standing balance of children and young adults under altered sensory conditions. Percept mot skills 2001; 92(3_suppl): 1167-1179.

Journal of Paramedical Sciences & Rehabilitation
Volume 9, Issue 2
August 2020
Pages 109-121

Files

Share

How to cite

Statistics