Motor Training in Degenerative Spinocerebellar Disease: Ataxia-Specific Improvements by Intensive Physiotherapy and Exergames

Table 1

Overview of high-intensity training studies in degenerative ataxia.

	Physiotherapy combined with occupational therapy [36]	Coordinative Physiotherapy [34, 35]	Exergames training [43]
Number of patients	42	16	10
Type of disease	SCA6 (20), ADCA (6), and IDCA (16)	SCA6 (2), SCA2 (1), ADCA (1), IDCA (6), FRDA (3), SANDO (2),and SN (1)	FRDA (4), arCA (3), AOA2 (1), and ADCA (2)
Age ± SD (range)	62.5±8.0 (range: 40–82)	61.4±11.2 (range: 44–79)	15.4±3.5 (range: 11–20)
Gender	22 males, 20 females	8 males, 8 females	5 males, 5 females
Duration of disease	9.8±6.2 (7 months–30 years)	12.9±7.8 (3–25 years)
Baseline SARA	11.3±3.8 (5–21.5)	15.8±4.3 (11–24)	10.9±2.3 (7–13.5)
Control	Crossover for short-term effect	Intraindividual controls for short-term effect	Intraindividual controls
Evidence class	Class Ib	Class III evidence	Class III evidence
Intervention	2 hours × 5 days + 1 hour × 2 days per week for 4 weeks	1 hour, 3 days per week for 4 weeks	1 hr × 4 per week for 2 weeks at lab; variable frequency at subjects’ own motivation for 6 weeks at home
After training	No	Home-training protocols	No
Outcome measures	SARA, FIM, gait speed, cadence, FAC, and falls	SARA, gait speed, balance, BBS, GAS, and movement analysis	SARA, balance, ABC scale, DGI scale, GAS, and movement analysis
Assessment point	Baseline, post 0, 4, 12, and 24 weeks	4 weeks pre, baseline, and post 0, 8 weeks	2 weeks pre, baseline, and post 0
Main results	SARA and gait improved 12 wks but not 24 wks	SARA and gait improved 8 wks after rehabilitation only in patients with cerebellar ataxia not afferent ataxia	SARA and gait improved directly post rehabilitation; improvement correlated with individual’s training intensity at home

SCA: spinocerebellar ataxia; FRDA: Friedreich’s ataxia; IDCA: idiopathic cerebellar ataxia; ADCA: autosomal dominant cerebellar ataxia of unknown type; SANDO: sensory ataxic neuropathy with dysarthria and ophthalmoparesis caused by mutations in the polymerase gamma gene; SN: sensory neuropathy with cerebellar degeneration; arCA: autosomal recessive cerebellar ataxia of unknown type; AOA2: ataxia with oculomotor apraxia type 2; SARA: scale for the assessment and rating of ataxia; ABC: activity-specific balance confidence scale; BBS: Berg balance score; GAS: goal attainment scaling [42]; DGI: dynamic Gait index; FIM: functional independence measure [38]; and FAC: functional ambulation categories. Evidence was graded according to the Oxford Center for Evidence Based Medicine (CEBM) classification. This table presents details of the first three clinical studies of motor rehabilitation in larger cohorts in degenerative spinocerebellar disease [34–36, 43].