Table 2 MRI and MRA findings in patients with IVS4 versus classical Fabry mutations

MRI findings

 Pulvinar sign, n (%)

8 (32.0)

6 (50.0)

0.4701^a

  95% CI

0.15-0.54

0.21-0.79

 Infarct, n (%)

8 (32.0)

4 (33.3)

0.6759^b

  95% CI

0.15-0.54

0.10-0.65

  Anterior circulation stroke

2 (8.0)

2 (16.7)

  Posterior circulation stroke

2 (8.0)

0

  Both anterior and posterior circulation stroke

4 (16.0)

2 (16.7)

  None of them

17 (68.0)

8 (66.7)

 Hemorrhage (microbleeds), n (%)

0

2 (16.7)

0.0991^a

 Fazekas score, n

25

12

0.7378^a

  0: No or a single punctate WM lesion

15 (60.0)

6 (50.0)

  1: Multiple punctate WM lesions

7 (28.0)

4 (33.3)

  2: Beginning of lesion confluence

2 (8.0)

2 (16.7)

  3: Large confluent lesion

1 (4.0)

0

 Height bifurcation of the BA, n

25

12

0.4276^*

  0: At or below the dorsum sellae

6 (24.0)

2 (16.7)

  1: Within the suprasellar cistern

9 (36.0)

3 (25.0)

  2: At the third ventricle floor

10 (40.0)

6 (50.0)

  3: Indenting and elevating the third ventricle floor

0

1 (8.3)

  Abnormal height bifurcation

10 (40.0)

7 (58.3)

 Laterality of the BA, n

25

12

0.3652^a

  0: Midline throughout

12 (48.0)

4 (33.3)

  1: Medial to lateral margins of clivus or dorsum sellae

12 (48.0)

6 (50.0)

  2: Lateral to lateral margins of clivus or dorsum sellae

1 (4.0)

2 (16.7)

  3: In the cerebellopontine angle cistern

0

0

 Abnormal laterality

1 (4.0)

2 (16.7)

0.2407^a

 95% CI

0.00-0.20

0.02-0.48

MRA findings

 BA diameter, mm

  Mean (SD)

2.7 (0.6)

3.3 (0.7)

  95% CI

2.5-3.0

2.9-3.8

  Median (range)

2.7 (1.4-4.0)

3.2 (2.3-4.7)

0.0293^b

 Vascular stenosis/BA hypoplasia, n ^c (%)

2 (8.3)

0

0.5429^a

  95% CI

0.01-0.27

0.74-1.00