Isolated spinal cord granulomatous angiitis: a case report and review of the literature.

Autor: Ayvacıoğlu Cagan C; Department of Neurology, Hacettepe University Faculty of Medicine, Hacettepe University Hospitals, 06230, Ankara, Turkey., Temucin CM; Department of Neurology, Hacettepe University Faculty of Medicine, Hacettepe University Hospitals, 06230, Ankara, Turkey., Arslan D; Department of Neurology, Hacettepe University Faculty of Medicine, Hacettepe University Hospitals, 06230, Ankara, Turkey., Gocmen R; Department of Radiology, Hacettepe University Faculty of Medicine, Ankara, Turkey., Bolek EC; Department of Internal Medicine (Rheumatology), Hacettepe University Faculty of Medicine, Ankara, Turkey., Söylemezoğlu F; Department of Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey., Topçuoğlu MA; Department of Neurology, Hacettepe University Faculty of Medicine, Hacettepe University Hospitals, 06230, Ankara, Turkey. mat@hacettepe.edu.t.
Jazyk: angličtina
Zdroj: Journal of neurology [J Neurol] 2022 Jun; Vol. 269 (6), pp. 3175-3179. Date of Electronic Publication: 2022 Jan 09.
DOI: 10.1007/s00415-021-10912-z
Abstrakt: Introduction: Isolated spinal cord angiitis (ISCA) is very rare disease. But, it is frequently encountered in the differential diagnosis of atypical spinal cord syndromes.
Case Presentation and Review of the Literature: We present a 31-year-old male who presented with progressive paraparesis, and diagnosed with pathologically confirmed ISCA. Longitudinal cystic transverse myelitis was documented in spinal MRI. He responded well to cyclophosphamide and steroid combination, and no relapse was noted during the 4-year follow-up. A standard systematic analysis of the germane literature disclosed 15 more ISCA cases. In total 16 cases (mean age: 46.5, 10 males), ISCA was diagnosed with pathological evaluation in all (Biopsy in 11, Autopsy in 5). MRI lesion is characterized by usually multisegmental longitudinal and sometimes cystic expansile lesions. In seven cases, it was described as "(pseudo)tumoral" by the authors. Albeit absence of elevation of CSF protein/WBC or "compatible" spinal MRI lesion may aid to exclude ISCA to some extent, pathological confirmation is currently necessary for the diagnosis. In 11 cases, ISCA was treated similar to primary supratentorial vasculitis. Mortality rate is 31%.
Discussion: ISCA diagnosis, a typical example of which we have presented here, can only be established by tissue examination. However, noninvasive diagnostic criteria are critically needed. Our data suggest that this can only be possible with multinational multicenter prospective registry.
(© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)
Databáze: MEDLINE
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