Selective dorsal rhizotomy for spasticity of genetic etiology

Childs Nerv Syst. 2020 Jul;36(7):1357-1365. doi: 10.1007/s00381-020-04601-x. Epub 2020 Apr 16.


Objective Selective dorsal rhizotomy (SDR) is most commonly applied in the context of the treatment of the spastic diplegic variant of cerebral palsy (CP). Its role in the treatment of spasticity associated with other conditions is not well-established. We sought to review outcomes following SDR for the treatment of functionally limiting spasticity in the setting of a genetic etiology. Methods A systematic literature review was performed using the databases Ovid Medline, Embase, Cochrane Library, and PubMed based on the PRISMA guidelines. Articles were included if they described the application of SDR for spasticity of genetic etiology. Reported outcomes pertaining to spasticity and gross motor function following SDR were summarized. Results Five articles reporting on 16 patients (10 males, 6 females) met the inclusion criteria, of which four reported on SDR for hereditary spastic paraplegia (HSP) and four on syndromic patients or other inherited diseases, with an overall follow-up ranging from 11 to 252 months. These individuals were found to have several genetic mutations including ALS2, SPG4, and SPG3A. The mean age at the time of surgery was 14.9 years (median 10 years, range 3-37 years). Conclusions Although all patients experienced a reduction in spasticity, the long-term gross motor functional outcomes objectively assessed at last follow-up were heterogeneous. There may be a role for SDR in the context of static genetic disorders causing spasticity. Further evidence is required prior to the widespread adoption of SDR for such disorders as, based on the collective observations of this review, spasticity is consistently reduced but the long-term effect on gross motor function remains unclear.

Keywords: Genetic spasticity; Hereditary spastic paraplegia; Selective dorsal rhizotomy.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Causality
  • Cerebral Palsy* / genetics
  • Cerebral Palsy* / surgery
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Male
  • Muscle Spasticity / genetics
  • Muscle Spasticity / surgery
  • Rhizotomy
  • Spastic Paraplegia, Hereditary* / genetics
  • Treatment Outcome
  • Young Adult