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

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Abstract

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.

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Authors and Affiliations

  1. Department of Neurology, Hacettepe University Faculty of Medicine, Hacettepe University Hospitals, 06230, Ankara, Turkey

    Cansu Ayvacıoğlu Cagan, Cagri Mesut Temucin, Doruk Arslan & M. Akif Topçuoğlu

  2. Department of Radiology, Hacettepe University Faculty of Medicine, Ankara, Turkey

    Rahsan Gocmen

  3. Department of Internal Medicine (Rheumatology), Hacettepe University Faculty of Medicine, Ankara, Turkey

    Ertugrul Cagri Bolek

  4. Department of Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey

    Figen Söylemezoğlu

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  1. Cansu Ayvacıoğlu Cagan
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Correspondence to M. Akif Topçuoğlu.

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On behalf of all authors, Mehmet Akif Topcuoglu, the corresponding author, states that there is no conflict of interest.

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This study does not contain any studies with human participants performed by the authors. Therefore, ethical committee aproval was waived. The patient signed informed consent for publication.

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Ayvacıoğlu Cagan, C., Temucin, C.M., Arslan, D. et al. Isolated spinal cord granulomatous angiitis: a case report and review of the literature. J Neurol 269, 3175–3179 (2022). https://doi.org/10.1007/s00415-021-10912-z

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  • DOI: https://doi.org/10.1007/s00415-021-10912-z

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