Objective: To describe OCT findings in spinocerebellar ataxia (SCA) type 10, correlate it with expansion size and disease severity and compare with those of SCA3

Background: SCA3  is the worldwide most prevalente SCA and presents with a CAG expansion at 14q24.3-q32 while SCA10 is rare and shows an ATTCT expansion at 22q13-qter.

The anatomopathological and clinical findings in SCA10 seems to be less agressive than those in SCA3. For an in vivo, non invasive, approach of the correlation between central nervous system and clinical evolution we can use the optic coherence tomography (OCT) to measure retinal nerve fiber layer (RNFL) and ganglion cell layer (GCL) thickness.

Methods: We analised 19 individuals with SCA (10 with SCA3 and nine with SCA10) recruited from the ataxia ambulatory, neurology service o f Hospital de Clínicas of Paraná – Brazil. They were submited to OCT and clinical evaluation using SARA score. Demographic data, time from disease onset and age of onset were collected. Expansion size was retrieved from medical records.

Results: We foud no correlation between size of expansion, SARA and RNFL or GCL  thickness in SCA10. RNFL seemed to be thicker in SCA10, although not statistically significant (p>0,05). There was no diference in GCL thickness between the groups. SARA, median age and time from disease onset did not differ between two groups. In this set, SCA10 individuals had an earlier disease onset .

In SCA3 there was a negative correlation between SARA and RNFL thickness in nasal area.

Conclusions: 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 agressive than others SCA. The findings in SCA3 are in accordance with the literature.

References: Teive, H. A. G. & Ashizawa, T. Primary and secondary ataxias. Curr. Opin. Neurol. 28, 413–22 (2015).

Teive, H. A. G. et al. Spinocerebellar ataxia type 10 – A review. Park. Relat. Disord. 17, 655–661 (2011).

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