Rituximab improves not only back stiffness but also "stiff eyes" in stiff person syndrome: Implications for immune-mediated treatment

J Neurol Sci. 2020 Jan 15:408:116506. doi: 10.1016/j.jns.2019.116506. Epub 2019 Nov 2.

Abstract

Objective: Stiff person syndrome (SPS) is usually characterized by truncal muscle rigidity and episodic painful spasms, but it sometimes appears with ocular symptoms called "stiff eyes". We recorded saccade movements in an SPS patient manifesting with "stiff eyes" conditions with slow saccade velocity and evaluated the effect of immunotherapy including rituximab on saccade parameters.

Methods: We repeatedly conducted saccade eye recordings using video-based eye tracking system on a 42-year-old male SPS patient with slow saccade. The velocity and onset latency of visual guided saccades (VGS) were measured at each recording. Because VGS velocity is affected by saccade amplitude, estimated peak velocity (Vmax) was also calculated by taking the relationship between the velocity and the amplitude of saccade into account.

Results: The mean VGS velocity improved significantly after two courses of rituximab administration compared with its lowest value. The estimated Vmax decreased as the clinical manifestations worsened, but it increased after rituximab administration. Other neurological symptoms in this patient such as muscle rigidity and gait instability also improved after the treatment.

Conclusion: Slow saccade in a "stiff eyes" patient improved after rituximab administration. Our study also indicated that the saccade eye recording is useful for evaluating the clinical condition of SPS when it is complicated with ocular symptoms.

Keywords: Burst neurons; Glutamic acid decarboxylase (GAD); Latch circuit; Omnipause neuron; Slow saccade; Stiff person plus; Video-based eye tracking system.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Back Muscles / drug effects*
  • Back Muscles / physiology
  • Eye-Tracking Technology*
  • Humans
  • Immunologic Factors / pharmacology
  • Immunologic Factors / therapeutic use*
  • Male
  • Rituximab / pharmacology
  • Rituximab / therapeutic use*
  • Saccades / drug effects*
  • Saccades / physiology
  • Stiff-Person Syndrome / drug therapy*
  • Stiff-Person Syndrome / physiopathology

Substances

  • Immunologic Factors
  • Rituximab