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Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia

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Abstract

A 12-year-old girl presented with a sudden decrease in her right visual acuity and homonymous hemianopsia. An angiography of the retinal arteries demonstrated recanalized occlusion of the right retinal artery. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation. Laboratory evaluations revealed dual antineutrophil cytoplasmic antibodies (ANCA) positivity [anti-proteinase (anti-PR3) ANCA and anti-myeloperoxidase (anti-MPO) ANCA], anticardiolipin (aCL) antibodies, and low titers of antinuclear antibodies (ANA). There was no evidence of active systemic lupus erythematosus (SLE), ANCA-related vasculitis, or other risk factors for cerebral occlusion, such as antiphospholipid syndrome (APS). Dual positivity for both cytoplasmic (c-ANCA) and perinuclear (p-ANCA) antineutrophil antibodies has been found previously in a small number of reports, but to our knowledge, this case represents the first case of moyamoya disease associated with dual ANCA positivity.

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Authors and Affiliations

  1. Department of Pediatric Nephrology, Faculty of Medicine, Başkent University, Ankara, Turkey

    Hale Sakalli & Esra Baskin

  2. Department of Pediatric Nephrology, Numune Teaching and Research Hospital, Adana, Turkey

    Hale Sakalli

  3. Department of Pediatric Neurology, Faculty of Medicine, Başkent University, Ankara, Turkey

    Füsun Alehan

  4. Department of Radiology, Faculty of Medicine, Başkent University, Ankara, Turkey

    Muhteşem Agıldere

  5. Department of Ophthalmology, Faculty of Medicine, Başkent University, Ankara, Turkey

    Yonca Aydin Akova

  6. Department of Neurosurgery, Faculty of Medicine, Başkent University, Ankara, Turkey

    Hakan Caner

Authors
  1. Hale Sakalli
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  2. Esra Baskin
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  3. Füsun Alehan
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  4. Muhteşem Agıldere
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  5. Yonca Aydin Akova
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  6. Hakan Caner
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Correspondence to Hale Sakalli.

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Sakalli, H., Baskin, E., Alehan, F. et al. Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia. Rheumatol Int 32, 3295–3298 (2012). https://doi.org/10.1007/s00296-011-2064-y

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  • DOI: https://doi.org/10.1007/s00296-011-2064-y

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