Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis.
We present one patient with Parry Romberg syndrome and another with linear scleroderma in coup de sabre, with focal neurologic deficits and intractable seizures arising from the hemisphere ipsilateral to the cutaneous lesion. Brain MRI showed progressive hemispheric atrophy. Pathology after functional hemispherectomy showed chronic inflammatory features suggestive of Rasmussen encephalitis.
Carreño
M
M
Department of Neurology, Hospital Clínic, Barcelona, Spain. mcarreno@clinic.ub.es
Donaire
A
A
Barceló
M I
MI
Rumià
J
J
Falip
M
M
Agudo
R
R
Bargalló
N
N
Setoain
X
X
Boget
T
T
Raspall
A
A
Pintor
L
L
Ribalta
T
T
D000328:Adult; D017668:Age of Onset; D001284:Atrophy / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D001327:Autoimmune Diseases / Q000503:physiopathology; D002648:Child; D004660:Encephalitis / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D004828:Epilepsies, Partial / Q000150:complications / Q000276:immunology / Q000503:physiopathology; D004827:Epilepsy / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D005150:Facial Hemiatrophy / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D005260:Female; D038421:Hemispherectomy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D045745:Scleroderma, Limited / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D013687:Telencephalon / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D016896:Treatment Outcome
D007166:Immunosuppressive Agents