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Neurology ; 68(16): 1308-10, 2007 Apr 17.
Article in English | MEDLINE | ID: mdl-17438222

ABSTRACT

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.


Subject(s)
Encephalitis/complications , Epilepsy/complications , Facial Hemiatrophy/complications , Scleroderma, Limited/complications , Telencephalon/physiopathology , Adult , Age of Onset , Atrophy/immunology , Atrophy/pathology , Atrophy/physiopathology , Autoimmune Diseases/physiopathology , Child , Encephalitis/immunology , Encephalitis/physiopathology , Epilepsies, Partial/complications , Epilepsies, Partial/immunology , Epilepsies, Partial/physiopathology , Epilepsy/immunology , Epilepsy/physiopathology , Facial Hemiatrophy/immunology , Facial Hemiatrophy/physiopathology , Female , Hemispherectomy , Humans , Immunosuppressive Agents/therapeutic use , Magnetic Resonance Imaging , Male , Scleroderma, Limited/immunology , Scleroderma, Limited/physiopathology , Telencephalon/immunology , Telencephalon/pathology , Treatment Outcome
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