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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
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Pediatric Rheumatology

ISSN: 1546-0096

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