Table 1 - Demographic, molecular and neurologic features of study population at baseline

Age at first examination (years)

46.35 ± 12.26

(24 to 71)^a

Age at onset of gait ataxia (years)

33.23 ± 12.37

(12 to 59)^a

Number of CAG repeats at normal ATXN2

22.26 ± 0.80

(22 to 27)^a

Number of CAG repeats at expanded ATXN2

40.35 ± 3.21

(34 to 49)^a

Disease duration at study entry (years)

12.94 ± 6.66

(2 to 27)^a

NESSCA at baseline

14.37 ± 4.32

(3 to 27)^a

SARA at baseline

18.42 ± 8.17

(5 to 33)^a

Main neurological findings at baseline:

Gait ataxia

49/49

Sensory losses (at least two altered proofs on lower limbs - pin prick/light touch, hot/cold (discrimination) and vibration sensations

19/43^b

Pyramidal syndrome (at least two of the following: generalized hyperreflexia, Babinski sign, spastic tonus)

5/49

Dysarthria

48/49

Fasciculations and amyotrophy

7/49

Dystonia (dystonic movements that impair in some degree voluntary movements)

8/49

Parkinsonism (at least two of the following: rigidity, bradychinesia, rest tremor)

17/49

With cognitive decline (MMSE ^c < = 24 or 18, if schooling was >5 or <= 5 years)

12/49

19.16 ± 6.9

(4 to 24)^a

Without cognitive decline

37/49

25.9 ± 2.9

(19 to 30)^a