Table 2 Video-oculographic findings in our AOA1, AOA2, AT patients and control group.

From: Comparing ataxias with oculomotor apraxia: a multimodal study of AOA1, AOA2 and AT focusing on video-oculography and alpha-fetoprotein

n=

AOA1

AOA2

AT

Controls

p

12

11

17

17

Age at VO

37 [15.4–58.7]

37.6 [21.6–51])

28.6 [22–54.8]

33 [25–38]

0.7744

SARA

23.5 [12–35]

22 [17.5–30]

20.3 [7.5–33]

NR

0.4418

SARA/DD

1 [0.6–2.4]

0.9 [0.6–1.9]

0.9 [0.2–1.8]

NR

0.4514

SDFS

5 [3–6]

6 [3–6]

4 [1–7]

NR

0.4639

SDFS/DD

0.21 [0.1–0.6]

0.24 [0.1–0.4]

0.20 [0.02–0.4]

NR

0.3023

Fixation

SWJ

4 (33.3)

7 (63.6)**

5 (31.3)

0

** < 0.01

DBN

3 (25)

7 (63.6)**

6 (42.9)*

0

* < 0.05 ** < 0.01

GEN

7 (58.3)**

9 (81.8)****

8 (50)**

0

** < 0.01 **** < 0.0001

Abnormal fixation

8 (58.3)a **

11 (100) a ****

13 (75)****

0

  a < 0.05 ** < 0.01 **** < 0.0001

Saccades

Horizontal

- Latency

 Mean latency

202.3 [139.5–492]

191.5 [136–354]

198 [160.5–449]

173.5 [138.5–245]

0.1773

 SD of latency

50.25 [27.5–136.5]

42.0 [28.5–89.5]

48.5 [16.5–150.5]

33.0 [14.5–103]

0.0643

 

All ataxia (AOA1, AOA2, AT): 48.3 [16.5–150.5]b

33.0 [14.5–103]

b < 0.05

 Increased latency

4 (40)

3 (27.3)

5 (33.3)

3 (18.8)

0.4097

-Gain/Amplitude

Centrifugal saccades

 Hypometric

9 (81.8)****

8 (72.7)****

14 (82.4)****

0

**** < 0.0001

 Hypermetric

2 (18.2)****

1 (9.1)****

2 (11.8)****

0

**** < 0.0001

Centripetal saccades

Hypometric

5 (45.5)****

3 (27.3)****

8 (47.1)****

0

**** < 0.0001

Hypermetric

4 (36.4)****

8 (72.7)****

6 (35.3)****

0

**** < 0.0001

-Horizontal velocity

decreased

4 (33.3)

2 (18.2)

1 (6.3)

0

 < 0.05

Cerebellar symptoms

8 (72.7)***

11 (100)****

13 (81.3)****

0

*** < 0.001 **** < 0.0001

Vertical

- Vertical velocity decreased

3 (25)

6 (54.6)**

3 (20)

0**

** < 0.01

-Upward latency

Mean latency

241 [154–326]

221 [133–335]

239 [153–302]*

176 [152–221]*

* < 0.05

SD of latency

All ataxia (AOA1, AOA2, AT): 58 [16–194]

41 [23–81]

0.0864

- Downward Latency

Mean latency

193 [156–263]

185 [131–287]

196 [161–309]

180.5 [147–249]

0.3603

SD of latency

All ataxia (AOA1, AOA2, AT): 47 [12–142]

36 [15–125]

0.1347

Smooth pursuit saccadic or abolished

10 (91)

11 (100)

12 (75)

ND

0.4443

Antisaccades

% of errors

23.5 [0–77.5]

51.5 [6–80.5]***

45.5 [6–86.5]***

10.5 [0–32]***

*** < 0.001

number of patients > 15% errors

5 (62.5)

10 (91)**

12 (92.3)**

5 (33.3)**

** < 0.01

  1. Categorical variables are expressed as numbers and percentages (n(%)); continuous variables as median [range].
  2. aSignificant differences after Bonferroni-Holm post-test between the AOA1 and AOA2 groups.
  3. *, **, *** and **** Significant differences after Bonferroni-Holm post-test relative to the control group.
  4. bSignificant differences after unpaired two-tailed Mann Whitney t test between the groups “all ataxia” and “controls”.
  5. The SARA and SDFS rates of decline (points per year; SARA/DD and SDFS/DD), defined as respectively SARA and SDFS scores (maximum - minimum)/disease duration, are indexes of disease progression.
  6. Abnormal Fixation is defined as at least one of: SWJ, DBN or GEN.
  7. Cerebellar symptoms are defined as presenting at least one of: DBN or GEN or Hypermetric horizontal saccades.
  8. Abbreviations: AOA1: ataxia with oculomotor apraxia type 1, AOA2: ataxia with oculomotor apraxia type 2, AT: Ataxia Telangiectasia; DBN: down beat nystagmus; DD: Disease Duration; GEN: gaze-evoked nystagmus; ND: Not done; NR: Not Relevant; SARA: Scale for the Assessment and Rating of Ataxia; SD: Standard Deviation; SDFS: Spinocerebellar degeneration functional score, SWJ: Square Waves Jerks, VO: video-oculography.
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