Epidural spinal cord stimulation in hereditary spastic paraplegia: preliminary results
Журнал: Вопросы нейрохирургии имени Н.Н. Бурденко. 2026;90(2):62-69.
DOI: 10.17116/neiro20269002162
Read: 1903 раза
Abstract
Hereditary spastic paraplegia is a diverse group of inherited neurodegenerative diseases characterized by spasticity and weakness in the lower extremities. Treatment is comprehensive and includes pharmacotherapy and physical therapy. If these approaches are ineffective, neurosurgical methods such as rhizotomy or intrathecal baclofen therapy are applied. Epidural spinal cord stimulation is still poorly studied.
OBJECTIVE
To evaluate safety and efficacy of epidural spinal cord stimulation in patients with hereditary spastic paraplegia.
MATERIAL AND METHODS
The study (2015—2024) included patients with hereditary spastic paraplegia refractory to conservative treatment. After test stimulation, permanent spinal stimulation systems (classic generator-based and wireless external) were implanted in 18 patients. The modified Ashworth scale, the Spastic Paraplegia Rating Scale (SPRS), the Timed Up and Go Test (TUG), the Ten-Meters Walk Test (10MWT), and the SF-36 questionnaire were used to assess neurological status preoperatively and six months after surgery. The study was approved by local ethics committee.
RESULTS
Implantation of permanent stimulation system (electrodes at the level of Th10-Th12) was performed in 18 patients (13 men, 5 women). Of these, 7 patients received wireless systems with external energy source, while 11 ones received classic generator systems. Six months after the intervention, we observed improvements in the following parameters compared to pre-implantation state: MAS score from 3 to 1.5—1 (18 patients); TUG test by 11.2—19.7% (17 patients); 10MWT test by 12.6—20.5% (17 patients); SPRS score by 27.9% (17 patients); SF-36 score 30—66% (16 patients).
CONCLUSION
Epidural spinal cord stimulation may be considered for hereditary spastic paraplegia, but further research is needed to clarify its role in symptomatic treatment of spastic paraplegia.
Keywords
- epidural spinal cord stimulation
- spasticity
- hereditary spastic paraplegia
Дата поступления: 27.01.2026
Дата принятия в печать: 30.01.2026
Дата публикации: 03.04.2026
- Parodi L, Coarelli G, Stevanin G, Brice A, Durr A. Hereditary ataxias and paraparesias: clinical and genetic update. Current Opinion in Neurology. 2018;31:462-471. https://doi.org/10.1097/WCO.0000000000000585
- Shribman S, Reid E, Crosby AH, Houlden H, Warner TT. Hereditary spastic paraplegia: from diagnosis to emerging therapeutic approaches. The Lancet Neurology. 2019;18(12):1136-1146. https://doi.org/10.1016/S1474-4422(19)30235-2
- Awuah WA, Tan JK, Shkodina AD, Ferreira T, Adebusoye FT, Mazzoleni A, Wellington J, David L, Chilcott E, Huang H, Abdul-Rahman T, Shet V, Atallah O, Kalmanovich J, Jiffry R, Madhu DE, Sikora K, Kmyta O, Delva MY. Hereditary spastic paraplegia: Novel insights into the pathogenesis and management. SAGE Open Medicine. 2023;12:20503121231221941. https://doi.org/10.1177/20503121231221941
- Ruano L, Melo C, Silva MC, Coutinho P. The global epidemiology of hereditary ataxia and spastic paraplegia: a systematic review of prevalence studies. Neuroepidemiology. 2014;42(3):174-183. https://doi.org/10.1159/000358801
- Vander Stichele G, Durr A, Yoon G, Schüle R, Blackstone C, Esposito G, Buffel C, Oliveira I, Freitag C, van Rooijen S, Hoffmann S, Thielemans L, Cowling BS. An integrated modelling methodology for estimating global incidence and prevalence of hereditary spastic paraplegia subtypes SPG4, SPG7, SPG11, and SPG15. BMC Neurology. 2022;22(1):115. https://doi.org/10.1186/s12883-022-02595-4
- Ortega Suero G, Abenza Abildúa MJ, Serrano Munuera C, Rouco Axpe I, Arpa Gutiérrez FJ, Adarmes Gómez AD, Rodríguez de Rivera FJ, Quintans Castro B, Posada Rodríguez I, Vadillo Bermejo A, Domingo Santos Á, Blanco Vicente E, Infante Ceberio I, Pardo Fernández J, Costa Arpín E, Painous Martí C, Muñoz García JE, Mir Rivera P, Montón Álvarez F, Bataller Alberola L, Gascón Bayarri J, Casasnovas Pons C, Vélez Santamaría V, López de Munain A, Fernández-Eulate G, Gazulla Abío J, Sanz Gallego I, Rojas Bartolomé L, Ayo Martín Ó, Segura Martín T, González Mingot C, Baraldés Rovira M, Sivera Mascaró R, Cubo Delgado E, Echavarría Íñiguez A, Vázquez Sánchez F, Bártulos Iglesias M, Casadevall Codina MT, Martínez Fernández EM, Labandeira Guerra C, Alemany Perna B, Carvajal Hernández A, Fernández Moreno C, Palacín Larroy M, Caballol Pons N, Ávila Rivera A, Navacerrada Barrero FJ, Lobato Rodríguez R, Sobrido Gómez MJ. Epidemiology of ataxia and hereditary spastic paraplegia in Spain: A cross-sectional study. Neurologia. 2023;38(6):379-386. https://doi.org/10.1016/j.nrleng.2023.04.003
- Coutinho P, Ruano L, Loureiro JL, Cruz VT, Barros J, Tuna A, Barbot C, Guimarães J, Alonso I, Silveira I, Sequeiros J, Marques Neves J, Serrano P, Silva MC. Hereditary ataxia and spastic paraplegia in Portugal: a population-based prevalence study. JAMA Neurology. 2013;70(6):746-755. https://doi.org/10.1001/jamaneurol.2013.1707
- Magzhanov RV, Saifullina EV, Idrisova RF. Epidemiology of hereditary spastic paraplegia in Bashkortostan Republic. Medical Genetics. 2013;(7):12-16. (In Russ.).
- Harding AE. Classification of the hereditary ataxias and paraplegias. Lancet. 1983;1(8334):1151-1155. https://doi.org/10.1016/s0140-6736(83)92879-9
- Darios F, Coarelli G, Durr A. Genetics in hereditary spastic paraplegias: Essential but not enough. Current Opinion in Neurobiology. 2022;72:8-14. https://doi.org/10.1016/j.conb.2021.07.005
- Schüle R, Wiethoff S, Martus P, Karle KN, Otto S, Klebe S, Klimpe S, Gallenmüller C, Kurzwelly D, Henkel D, Rimmele F, Stolze H, Kohl Z, Kassubek J, Klockgether T, Vielhaber S, Kamm C, Klopstock T, Bauer P, Züchner S, Liepelt-Scarfone I, Schöls L. Hereditary spastic paraplegia: Clinicogenetic lessons from 608 patients. Annals of Neurology. 2016;79(4):646-658. https://doi.org/10.1002/ana.24611
- Bellofatto M, De Michele G, Iovino A, Filla A, Santorelli FM. Management of Hereditary Spastic Paraplegia: A Systematic Review of the Literature. Frontiers in Neurology. 2019;10:3. https://doi.org/10.3389/fneur.2019.00003
- Di Ludovico A, Ciarelli F, La Bella S, Scorrano G, Chiarelli F, Farello G. The therapeutic effects of physical treatment for patients with hereditary spastic paraplegia: a narrative review. Frontiers in Neurology. 2023;14:1292527. https://doi.org/10.3389/fneur.2023.1292527
- Park TS, Joh S, Walter DM, Meyer NL. Selective Dorsal Rhizotomy for Treatment of Hereditary Spastic Paraplegia-Associated Spasticity in 37 Patients. Cureus. 2021;13(9):e17690. https://doi.org/10.7759/cureus.17690
- Margetis K, Korfias S, Boutos N, Stylianos Gatzonis, Marios Themistocleous, Anna Siatouni, Zoi Dalivigka, Theofanis Flaskas, George Stranjalis, Efstathios Boviatsis, Damianos Sakas. Intrathecal baclofen therapy for the symptomatic treatment of hereditary spastic paraplegia. Clinical Neurology and Neurosurgery. 2014;123:142-145. https://doi.org/10.1016/j.clineuro.2014.05.024
- Heetla HW, Halbertsma JP, Dekker R, Staal MJ, van Laar T. Improved gait performance in a patient with hereditary spastic paraplegia after a continuous intrathecal baclofen test infusion and subsequent pump implantation: a case report. The Archives of Physical Medicine and Rehabilitation. 2015;96(6):1166-1169. https://doi.org/10.1016/j.apmr.2015.01.012
- Sharma J, Bonfield C, Steinbok P. Selective dorsal rhizotomy for hereditary spastic paraparesis in children. Child’s Nervous System. 2016;32(8):1489-1494. https://doi.org/10.1007/s00381-016-3122-2
- Nagel SJ, Wilson S, Johnson MD, Machado A, Frizon L, Chardon MK, Reddy CG, Gillies GT, Howard MA 3rd. Spinal Cord Stimulation for Spasticity: Historical Approaches, Current Status, and Future Directions. Neuromodulation. 2017;20(4):307-321. https://doi.org/10.1111/ner.12591
- Suponeva NA, Yusupova DG, Ilina KA, Melchenko DA, Butkovskaya AA, Zhirova ES, Taratukhina AS, Zimin AA, Zaitsev AB, Klochkov AS, Lyukmanov RKh, Kotov-Smolensky AM, Khizhnikova AE, Gatina GA, Kutlubaev MA, Piradov MA. Validation of the Modified Ashworth Scale in Russia. Annals of Clinical and Experimental Neurology. 2020;14(1):89-96. (In Russ.). https://doi.org/10.25692/ACEN.2020.1.10
- Schüle R, Holland-Letz T, Klimpe S, Kassubek J, Klopstock T, Mall V, Otto S, Winner B, Schöls L. The Spastic Paraplegia Rating Scale (SPRS): a reliable and valid measure of disease severity. Neurology. 2006;67(3):430-434. https://doi.org/10.1212/01.wnl.0000228242.53336.90
- Ware JE Jr, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care. 1992;30(6):473-483.
- Dekopov AV, Shabalov VA, Tomsky AA, Hit MV, Salova EM. Chronic Spinal Cord Stimulation in the Treatment of Cerebral and Spinal Spasticity. Stereotactic and Functional Neurosurgery. 2015;93(2):133-139. https://doi.org/10.1159/000368905
- van de Pol LA, Burgert N, van Schie PEM, Slot KM, Gouw AA, Buizer AI. Surgical treatment options for spasticity in children and adolescents with hereditary spastic paraplegia. Childs Nerv Syst. 2024;40:855-861. https://doi.org/10.1007/s00381-023-06159-w
- Smolyankina EI, Zinenko DYu. Selective dorsal rhizotomy in children with hereditary spastic paraplegia. Burdenko’s Journal of Neurosurgery. 2025;89(1):46-51. (In Russ., In Engl.). https://doi.org/10.17116/neiro20258901146
- Pucks-Faes E, Dobesberger J, Hitzenberger G, Matzak H, Mayr A, Fava E, Genelin E, Saltuari L. Intrathecal Baclofen in Hereditary Spastic Paraparesis. Frontiers in Neurology. 2019;10:901. https://doi.org/10.3389/fneur.2019.00901
- Gushcha AO, Kashcheev AA, Karabanov AV, Illarioshkin SN Chronic spinal cord stimulation in Strumpell disease: the first clinical observation. Neurological journal. 2015;6:24-29. (In Russ.). https://dx.doi.org/10.18821/1560-9545-2015-20-6-22-27
- Pinto de Souza C, Coelho DB, Campos DDSF, Dos Santos Ghilardi MG, de Oliveira Vicente EC, González-Salazar C, Junior MCF, Barsottini OGP, Pedroso JL, Fonoff ET. Spinal cord stimulation improves motor function and gait in spastic paraplegia type 4 (SPG4): Clinical and neurophysiological evaluation. Parkinsonism & Related Disorders. 2021;83:1-5. https://doi.org/10.1016/j.parkreldis.2020.12.008
- Tufo T, Ciavarro M, Di Giuda D, Piccininni C, Piano C, Daniele A. Spinal cord stimulation may improve gait and cognition in hereditary spastic paraplegia with mental retardation: a case report. Neurological Sciences. 2023;44(3):961-966. https://doi.org/10.1007/s10072-022-06487-w
Recommended articles:
Experience of using Relatox in the complex rehabilitation of patients with lower limb spasticity after focal brain damage of various etiologies.S.S. Korsakov Journal of Neurology and Psychiatry. 2026;126(3):126-135
Prognostic factors of GMFCS class improvement after selective dorsal rhizotomy in children with cerebral palsy.Burdenko's Journal of Neurosurgery. 2026;90(2):54-61
Improvement of spinal sensorimotor adaptation in children with cerebral palsy with various locomotor patterns using botulinum therapy and compression orthoses.S.S. Korsakov Journal of Neurology and Psychiatry. 2025;125(10-2):127-135
Sequential use of botulinum toxin A and bovhyaluronidase azoximer in the correction of post-stroke spasticity.S.S. Korsakov Journal of Neurology and Psychiatry. 2025;125(2):80-85
Selective dorsal rhizotomy in children with hereditary spastic paraplegia.Burdenko's Journal of Neurosurgery. 2025;89(1):46-51