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 |