Połowiczy zanik twarzy bez zmian w ośrodkowym układzie nerwowym i twardzina en coup de sabre z guzem zewnątrzmózgowym – opis przypadków

Introduction. Progressive facial hemiatrophy (Parry-Romberg syndrome)is a relatively rare disease of unclear aetiology. Some authorspostulate its relation with linear scleroderma.Objective. To present 2 cases: facial hemiatrophy and linear sclerodermalocalized on the head with different clinical picture.Case report. We present two cases: a case of a 49-year-old woman witha typical picture of hemifacial atrophy, without any changes in the centralnervous system; and a case of a 33-year-old patient with sclerodermaen coup de sabre and extracerebral tumour. In a patient diagnosedwith Perry-Romberg syndrome, with Borrelia burgdorferi infectionand minor neurological symptoms, for the 4-year duration of thedisease the proper diagnosis and therapy have not been established. Inthe second patient only skin changes without any neurological symptoms were observed, but through investigations of the central nervoussystem revealed the presence of an extracerebral tumour.Conclusions. The presented cases, especially the second one, indicatethe necessity of detailed neurological examination in patients with progressivefacial hemiatrophy and scleroderma en coup de sabre in spite ofa lack of subjective complaints