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Table 2 Magnetic resonance imaging (MRI) abnormalities and progression in individual patients with Parry-Romberg (PRS) and en coup de sabre (ECDS)

From: Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre

 

Demographics

Additional clinical features

MRI findings

ID

Skin

Sex

Age at diagnosis/ first scan (years)

Neurological

Psychiatric

Other

White matter signal

White matter volume

Calcification

Enhancement

Other findings

Follow up imaging

1

ECDS

M

6/6

Headaches

Visual hallucinations

Gastro-oesophageal reflux

Normal

Preserved

Absent

Absent

 

Unchanged at 1 year

2

ECDS

F

2/2

Seizures;

Cranial nerve dysfunction

Obsessive compulsive disorder

 

Abnormal;

Mixed response

Preserved with sulcal crowding

Present;

Progressive

Present;

Leptomeningeal;

Increases

 

3 years

3

ECDS

F

12/9

Seizures;

Headaches;

Transient ischaemic attack;

Hyperaesthesia

Depression;

Anxiety

 

Abnormal;

Regressive

Preserved

Present;

Progressive

Present;

Leptomeningeal;

Increases

 

6 years

4

ECDS

M

3/3

Headaches

  

Normal

Preserved

Absent

Absent

 

Unchanged at 5 years

5

ECDS

M

0.4/0.9

   

Normal

Preserved

Absent

Absent

 

No follow up imaging

6

ECDS

F

8/9

Seizures

 

Myositis

Normal

Preserved

Absent

Absent

 

Unchanged at 1 year

7

ECDS/PRS

F

7/3

Seizures;

Cranial nerve dysfunction;

Gait disturbance;

Myoclonus

  

Abnormal; Regressive

Preserved

Absent

Absent

Left orbital myositis

6 years

8

ECDS/PRS

M

2/2

Seizures

  

Abnormal; Regressive

Preserved

Present;

In cavernomata;

Stable

Present;

In cavernomata;

Stable

 

2 years

9

ECDS/PRS

M

6/6

Headaches

  

Abnormal;

Progressive

Preserved with sulcal crowding

Present;

Progressive

Present;

Leptomeningeal;

Stable

 

11 years

10

ECDS/PRS

F

1/0.3

Seizures

 

Hypertension

Abnormal;

Progressive

Progressive atrophy

Present;

Stable

Present;

Subtle;

Leptomeningeal;

Unable to assess longitudinally

Porencephaly;

Left ocular atrophy

11 years

11

ECDS/PRS

F

9/17

 

Anxiety

 

Normal

Preserved

Absent

NA

 

2 years

12

ECDS/PRS

F

14/14

   

Normal

Preserved with sulcal crowding

NA

NA

 

No follow up imaging

13

ECDS/PRS

F

11/7

   

Normal

Preserved

Absent

NA

 

5 years

14

ECDS/PRS

M

13/14

  

Temporomandibular joint dysfunction;

Bicuspid aortic valve

Normal

Preserved

Absent

NA

 

4 years