Skip to main content

Advertisement

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

From: The progression rate of spinocerebellar ataxia type 2 changes with stage of disease

N subjects (M/F) 49 (27/22)
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 19/43b
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
  1. a mean and standard deviation (range)
  2. b Six subjects were excluded due to the presence of comorbidities such as diabetes melittus
  3. c MMSE: Mini Mental State Evatuation