The Cerebellum

, Volume 16, Issue 4, pp 797–801 | Cite as

A Comparative Optical Coherence Tomography Study of Spinocerebellar Ataxia Types 3 and 10

  • Fernando Spina Tensini
  • Mario T. Sato
  • Naoye Shiokawa
  • Tetsuo Ashizawa
  • Hélio A. G. Teive
Original Paper

Abstract

SCA3 presents with a CAG expansion at 14q24.3-q32 while SCA10 shows an ATTCT expansion at 22q13-qter. SCA10 seems to be less aggressive than SCA3. For an in vivo, noninvasive approach of the correlation between central nervous system and clinical evolution, we can use optic coherence tomography (OCT) to measure retinal nerve fiber (RNFL) and ganglion cell layer (GCL) thickness. To describe OCT findings in SCA10, correlate it with expansion size and disease severity and compare with those of SCA3. We analyzed ten individuals with SCA3 and nine with SCA10 recruited from the neurology service of Hospital de Clínicas of Paraná—Brazil. They were submitted to OCT and clinical evaluation using SARA score. Expansion size, demographic data, time from disease onset, and age of onset were collected. We found no correlation between size of expansion, SARA, and RNFL or GCL thickness in SCA10. RNFL seemed to be thicker in SCA10 (p > 0.05). GCL thickness, SARA, median age, and time from disease onset did not differ between groups. SCA10 individuals had an earlier disease onset. In SCA3, there was a negative correlation between SARA and RNFL thickness in nasal area. To the best of our knowledge, this is the first paper assessing retinal changes by OCT in individuals with SCA10. The lack of correlation between disease progression, age, and time since onset supports the anatomopathological findings which suggest SCA10 is less aggressive than other SCAs. The findings in SCA3 are in accordance with the literature.

Keywords

Ataxia OCT Spinocerebellar ataxia SCA10 SCA3 Optic coherence tomography 

Notes

Compliance with Ethical Standards

The patients signed a voluntary informed consent form, and the protocol was approved by the HC-UFPR Committee for Ethics in Human Research.

Conflict of Interest

Fernando Spina Tensini has received no relevant financial or nonfinancial supports including, but not limited to, grants or funding, employment, affiliations, patents, inventions, honoraria, consultancies, royalties, stock options/ownership, or expert testimony, in the past 3 years to disclose.

Mario Teruo Sato has received no relevant financial or nonfinancial supports including, but not limited to, grants or funding, employment, affiliations, patents, inventions, honoraria, consultancies, royalties, stock options/ownership, or expert testimony, in the past 3 years to disclose.

Naoye Shiokawa has received no relevant financial or nonfinancial supports including, but not limited to, grants or funding, employment, affiliations, patents, inventions, honoraria, consultancies, royalties, stock options/ownership, or expert testimony, in the past 3 years to disclose.

Tetsuo Ashizawa receives research/grant support as a principal investigator of studies for Ionis Pharmaceuticals, Inc. (1515598769-CS2); the Myotonic Dystrophy Foundation; and the National Institutes of Health (NS083564).

Helio Afonso Ghizoni Teive has received no relevant financial or nonfinancial supports including, but not limited to, grants or funding, employment, affiliations, patents, inventions, honoraria, consultancies, royalties, stock options/ownership, or expert testimony, in the past 3 years to disclose.

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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de ClínicasFederal University of ParanáCuritibaBrazil
  2. 2.Neuro-Ophthalmology and Ocular Electrophysiology Sector, Vision Center, Hospital de ClínicasFederal University of ParanáCuritibaBrazil
  3. 3.Retina and Vitreous ClinicCuritibaBrazil
  4. 4.Neuroscience Research ProgramHouston Methodist Research InstituteHoustonUSA

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