Cerebral and Oculorhinal Manifestations of a Limited Form of Wegener's Granulomatosis With c-ANCA—Associated Vasculitis

Authors

  • Peterus Thajeb MD,

    Corresponding author
    1. Department of Neurology, Mackay Memorial Hospital, Taipei, Taiwan, ROC.
    2. Section of Neurology and Section of Immunology, Cathay General Hospital, Taipei, Taiwan, ROC.
      Dr. Thajeb, P.O. Box Nei-hu 6-30, Taipei, Director, Department of Neurology, Mackay Memorial Hospital, Taipei, Taiwan, ROC. E-mail: thajebp@hotmail.com.
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  • Jaw-Ji Tsai PhD

    1. Section of Neurology and Section of Immunology, Cathay General Hospital, Taipei, Taiwan, ROC.
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Dr. Thajeb, P.O. Box Nei-hu 6-30, Taipei, Director, Department of Neurology, Mackay Memorial Hospital, Taipei, Taiwan, ROC. E-mail: thajebp@hotmail.com.

ABSTRACT

The authors report on cerebral and oculorhinal manifestations in a patient with a cytoplasmic pattern of antineutrophil cytoplasmic autoantibody (c-ANCA)—associated vasculitis. Recurrent Tolosa-Hunt syndrome, cavernous sinus syndrome, Raeder's paratrigeminal neuralgia, and seizures were the major clinical manifestations. Brain MRI showed localized enhancing lesions initially in the cavernous sinus and later in the convexity pachymeninges. The lesions disappeared following 9 months of oral prednisolone (15 mg/day) and cyclophosphamide (100 mg/day) therapy. The presence of c-ANCA, demonstration of vasculitis, and depositions of immunoglobulin G (IgG) and fibrinogen in the vessel walls of pachymeninges of the patient confirmed an immune-mediated cause of the vasculitis. Cranial pathology without renal and pulmonary involvement suggests a variant of Wegener's granulomatosis, which is called the “limited” form of Wegener's granulomatosis.

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