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Table 2 Clinical characteristics of patients with SCA1, SCA2 and unknown genetic etiology

From: Autosomal dominant hereditary ataxia in Sri Lanka

Genetic Diagnosis

N

Mean age symptom onset (years)

Disability level*

SARA**

INAS***

Clinically relevant depression *****

 

(0–40)

(0–16)

 

SCA1

21

34.8 ± 10

4 ± 2

18.8 ± 9.7

3.6 ± 2.4

68.4%

SCA2

1

34.0

6

26

6

100%

Unknown genetic etiology

12

32.7 ± 9.8

3 ± 2.7

13.7 ± 7.1

3.8 ± 2.1

75%

  1. N = patient number included in the study.
  2. *Disability level was quantified using the SPATAX score. (0 = No functional handicap, 1 = No functional handicap but signs at examination, 2 = mild unable to run, walking unlimited, 3 = moderate unable to run, limited walking without aid, 4 = severe walking with one stick, 5 = walking with two sticks, 6 = unable to walk requiring wheelchair, 7 = confined to bed).
  3. **SARA = Scale of Assessment and Rating of Ataxia.
  4. ***INAS = Inventory of Non ataxia Symptoms.
  5. ****Clinically relevant depression as percentage of total subtype patient population - assessed using the Patient Health Questionnaire 9 (PHQ9).
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BMC Neurology

ISSN: 1471-2377

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