Table 1.

Summary of studies on cognitive deficits in hereditary ataxias, highlighting the main neuropsychological findings

Study	Hereditary ataxia	Patients (n)	Cognitive deficits
Radvany et al., 1993	SCA3	35	Visual memory, visuospatial processing, colour discrimination
Maruff et al., 1996	SCA3	6	Visual attention
Zawacki et al., 2002	SCA3	6	Executive function
Bürk et al., 2003	SCA1, 2 and 3	36	Verbal memory and executive deficits
Kawai et al., 2004	SCA3	16	Verbal and visual memory, verbal fluency, visuospatial and constructional
Klinke et al., 2010	SCA1, 2, 3 and 6	36	Frontal-attention and executive dysfunction
Tedesco et al., 2011	SCA1, SCA2, FRDA, AT and DVE	24	Language, visuospatial abilities, executive function, sequencing abilities and visuospatial memory
Braga-Neto et al., 2012	SCA3	38	Executive and visuospatial functions
Verhoeven et al., 2012	ARSACS	2	Executive and motivation impairment
Nieto et al., 2012	FRDA	36	Executive, visuoconstrutive and visuoperceptive dysfunction and poor action naming
Roeske et al., 2013	SCA3	11	Verbal learning, verbal and nonverbal memory
Lopes et al., 2013	SCA3	32	Episodic and working memory
Fancellu et al., 2013	SCA1 and 2	42	Executive functions and visuospatial and visuoperceptive function

SCA = spinocerebellar ataxia; ARSACS = autosomal recessive spastic ataxia of Charlevoix-Saguenay; FRDA = Friedreich’s ataxia; AOA2 = ataxia with oculomotor apraxia type 2; AT = ataxia-telangiectasia; DVE = deficiency of vitamin E.