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Ligia Brzezińska-wcisło - One of the best experts on this subject based on the ideXlab platform.

  • Połowiczy zanik twarzy bez zmian w ośrodkowym układzie nerwowym i twardzina en coup de sabre z guzem zewnątrzmózgowym – opis przypadków
    Termedia Publishing House, 2011
    Co-Authors: Ligia Brzezińska-wcisło, Anna Lis-Święty, Beata Bergler-czop
    Abstract:

    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

  • "Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom) without any changes in CNS and linear scleroderma "en coup de sabre" with CNS tumor
    BMC Neurology, 2009
    Co-Authors: Beata Bergler-czop, Anna Lis-Święty, Ligia Brzezińska-wcisło
    Abstract:

    Background Hemifacial atrophy (Parry-Romberg Syndrome) is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear Syndrome. In a number of patients, neurological affection is the medium of the disease. The treatment of both scleroderma varieties is similar to the treatment of limited systemic sclerosis. Case presentation We present two cases of a disease: a case of a 49-year-old woman with a typical image of hemifacial atrophy, without any changes of the nervous system and a case of a 33-year-old patient with an "en coup de sabre" scleroderma and with CNS tumor. Conclusion We described typical cases of a rare diseases, hemifacial atrophy and "en coup de sabre" scleroderma. In the patient diagnosed with Parry-Romberg Syndrome, with Borrelia burgdoferi infection and with minor neurological symptoms, despite a four-year case history, there was a lack of proper diagnosis and treatment. In the second patient only skin changes without any neurological symptoms could be observed and only a precise neurological diagnosis revealed the presence of CNS tumor.

Ligia Brzezinskawcislo - One of the best experts on this subject based on the ideXlab platform.

Sobia Hassan - One of the best experts on this subject based on the ideXlab platform.

  • Retinal vasculitis the first clue in the diagnosis of progressive hemifacial atrophy.
    European journal of rheumatology, 2019
    Co-Authors: Atefeh Vafa, Ofelya Gevorgyan, Sobia Hassan
    Abstract:

    Retinal vasculitis is a sight-threatening condition that can occur as an isolated ocular disorder or in association with a number of systemic diseases. Parry-Romberg Syndrome, also known as progressive hemifacial atrophy (PHA), is a rare disorder of unknown etiology characterized by unilateral facial atrophy and is associated with multiple ophthalmologic and neurologic manifestations. Here we report the case of a 17-year-old man with no prior diagnosis of PHA, who presented with a sudden onset of floaters and decreased vision in the right eye; he was found to have retinal vasculitis and uveitis in the right eye. Routine workup did not reveal the cause of retinal vasculitis. However, thorough physical examination demonstrated features of PHA overlapping with linear scleroderma en coup de sabre. The patient was started on treatment with systemic steroids with a later addition of methotrexate; he responded to treatment with considerable improvement in his symptoms and ophthalmologic examination.

Beata Bergler-czop - One of the best experts on this subject based on the ideXlab platform.

  • Połowiczy zanik twarzy bez zmian w ośrodkowym układzie nerwowym i twardzina en coup de sabre z guzem zewnątrzmózgowym – opis przypadków
    Termedia Publishing House, 2011
    Co-Authors: Ligia Brzezińska-wcisło, Anna Lis-Święty, Beata Bergler-czop
    Abstract:

    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

  • "Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom) without any changes in CNS and linear scleroderma "en coup de sabre" with CNS tumor
    BMC Neurology, 2009
    Co-Authors: Beata Bergler-czop, Anna Lis-Święty, Ligia Brzezińska-wcisło
    Abstract:

    Background Hemifacial atrophy (Parry-Romberg Syndrome) is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear Syndrome. In a number of patients, neurological affection is the medium of the disease. The treatment of both scleroderma varieties is similar to the treatment of limited systemic sclerosis. Case presentation We present two cases of a disease: a case of a 49-year-old woman with a typical image of hemifacial atrophy, without any changes of the nervous system and a case of a 33-year-old patient with an "en coup de sabre" scleroderma and with CNS tumor. Conclusion We described typical cases of a rare diseases, hemifacial atrophy and "en coup de sabre" scleroderma. In the patient diagnosed with Parry-Romberg Syndrome, with Borrelia burgdoferi infection and with minor neurological symptoms, despite a four-year case history, there was a lack of proper diagnosis and treatment. In the second patient only skin changes without any neurological symptoms could be observed and only a precise neurological diagnosis revealed the presence of CNS tumor.

Beata Berglerczop - One of the best experts on this subject based on the ideXlab platform.