Patterns and modulations of Pendular nystagmus in a family with hereditary spastic paraplegia

Eun Hye Oh, Jae Hyeok Lee, Jin Hong Shin, Hyang Sook Kim, Ji Soo Kim, Hyo Jung Kim, Seo Young Choi, Kwang Dong Choi, David S. Zee, Jae Hwan Choi

Research output: Contribution to journalArticle

Abstract

Hereditary spastic paraplegia (HSP) is characterized by progressive spasticity and weakness of the lower extremities. Additional findings include ataxia, extrapyramidal signs, and dementia. Pendular nystagmus (PN) has been reported in some subtypes of HSP caused by PLP1 (SPG2) or paraplegin (SPG7) mutation. To describe the patterns and modulation of PN in HSP, we performed eye movement recording using video-oculography in a Korean family with HSP and PN. The PN was convergent-divergent in the oblique plane with a frequency of 6 to 7 Hz and maximum amplitude at about 1.5°. The nystagmus diminished briefly after blinks and horizontal saccades, and decreased during eccentric gaze and convergence. Horizontal saccades shifted the phase of the oscillations. During lateral gazes, the PN increased in the abducting eye, but decreased in the adducting eye. Vibratory stimuli decreased the nystagmus mostly in the left but not in the right eye. No pathogenic mutation was found in the genetic loci known for causing spastic paraplegia by whole-exome sequencing. The unusual features and modulation of PN in our patients with HSP emphasize the role of disconjugate and disjunctive capabilities of the brain in ocular motor control, and exemplify what can go wrong.

Original languageEnglish (US)
Pages (from-to)169-173
Number of pages5
JournalJournal of the Neurological Sciences
Volume383
DOIs
StatePublished - Dec 15 2017

Keywords

  • Gaze
  • Hereditary spastic paraplegia
  • Pendular nystagmus
  • Vibration

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Oh, E. H., Lee, J. H., Shin, J. H., Kim, H. S., Kim, J. S., Kim, H. J., Choi, S. Y., Choi, K. D., Zee, D. S., & Choi, J. H. (2017). Patterns and modulations of Pendular nystagmus in a family with hereditary spastic paraplegia. Journal of the Neurological Sciences, 383, 169-173. https://doi.org/10.1016/j.jns.2017.11.011