Objective: Evaluate cognition and emotion regulation of patients with early onset ataxia (EOA).

Background: Early onset ataxia (EOA) is a movement disorder caused by a dysfunction of the cerebellum or its networks, developing before the age of 25. Recent research has emphasized the cerebellum’s role in cognition and emotion regulation. Deficits concerning executive functioning, language, working memory, and spatial perception have been reported, but systematic evaluation and an investigation of the relation with emotion regulation is lacking.

Methods: In 24 EOA patients (19 male, MAge=19 yrs, SD=8.38, MIQ=78.75, SD=17.95) we performed a neuropsychological assessment covering intelligence (subtests from the WISC or WAIS), memory (RAVLT, Doors, Digit span), attention and processing speed (LDST, TMT), executive functions (BADS-C zoo map), social cognition (FEEST-36, ToM Nepsy-II) and language (Fluency). Patients’ proxies filled in the CBCL or ABCL to assess anxiety and withdrawn behavior. Test data were compared to an age- and gender matched healthy control group (n=24, 17male, MAge=19 yrs, SD=7.88, MIQ=102.75, SD=11.71).

Results: After controlling for verbal IQ, verbal learning (p=.032) was significantly lower in the EOA patients. Other tests did not differ between the two groups. Anxiety was negatively correlated with emotion recognition (p=.02). Withdrawn behavior was negatively correlated with ToM (p<.041) and emotion recognition (p=.033).

Conclusions: Apart from deficits in verbal learning, our patients showed intact cognitive functions. Deficits in verbal learning have not been reported earlier in EOA. This finding can have implications for treatment as studies on other patient groups have shown that deficits in verbal learning can hinder treatment compliance. In addition, we found an association of social cognition and emotion regulation in EOA patients. Future research needs to find out more about consequences of this relation.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/cognition-and-emotion-regulation-in-early-onset-ataxia/