'Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom) without any changes in CNS and linear scleroderma 'en coup de sabre' with CNS tumor
| Autor: | Anna Lis-Święty, Beata Bergler-Czop, Ligia Brzezińska-Wcisło |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2009 |
| Předmět: |
Adult
Pathology medicine.medical_specialty Neurology Clinical Neurology Case Report Disease Scleroderma lcsh:RC346-429 Diagnosis Differential Scleroderma Limited Facial Hemiatrophy medicine Humans Linear Scleroderma skin and connective tissue diseases lcsh:Neurology. Diseases of the nervous system Neurologic Examination Lyme Disease Electromyography business.industry General Medicine Middle Aged medicine.disease Magnetic Resonance Imaging Borrelia burgdorferi Etiology Female Neurology (clinical) Neurosurgery Differential diagnosis Tomography X-Ray Computed business Cerebral Ventricle Neoplasms Tomography Spiral Computed Rare disease |
| Zdroj: | BMC Neurology, Vol 9, Iss 1, p 39 (2009) BMC Neurology |
| ISSN: | 1471-2377 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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