hemifacial atrophy—progressive

GENETICS, PAEDIATRICS

Definition A condition (OMIM:141300) characterised by slowly progressive atrophy of the soft tissues of half the face, accompanied usually by contralateral Jacksonian epilepsy, trigeminal neuralgia, and changes in the eyes and hair.

Pathogenesis Causative genetic defects have not been identified. Parry-Romberg syndrome may share a common autoimmune aetiology and pathology with Rasmussen encephalitis. 

Synonyms Facial hemiatrophy, Parry-Romberg syndrome, progressive hemifacial atrophy, Romberg syndrome, Romberg’s disease, trophoneurosis facialis progressiva 

Reference http://www.omim.org/entry/141300