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Table 3 Neuroimaging (MRI or CT scan) data before and after the initiation of folinic acid therapy and clinical outcome

From: Follow-up of folinic acid supplementation for patients with cerebral folate deficiency and Kearns-Sayre syndrome

Cases

 

Time between images (years)

Atrophy

Abnormal high signal on T2-weighted images

Clinical outcome NPMDS* Sections I-III Pre/Post

Age at neuroimaging before therapy

Cerebral

Cerebellar

Cerebrum subcortical

Brain stem

Basal ganglia

Thalamus

Cerebellum

 

Pre/Post

Pre/Post

Pre/Post

Pre/Post

Pre/Post

Pre/Post

Pre/Post

1

8

0.7

  

+ / ++

+/+

+/+

 

+/+

31/40

2

11

2.7

  

+/ ++

+/+

 

+/+

 

7/14

3

17

3

−/+

 

+

+/+

−/+

+

−/+

26/54**

4*

14

3

−/+

 

−/+

 

+/+

 

−/+

29/67**

5

   

+

+

+

+

+

+

Unknown

6

13

2

   

+/+

+/+

 

−/+

17/22

7

  

+

   

+

+

+

Unknown

8

8

2

  

+/ -

    

20/24

  1. *CT scan, + (lesion), ++ (progression), − (not lesion or improvement). ** Score assessed in adult patients using NMDAS.