Pseudotumor cerebri as a cardinal sign of the polyangiitis overlap syndrome

Abstract

Pseudotumor cerebri is usually thought of as an idiopathic disorder of obese young women. A variety of systemic illnesses have been described as having pseudotumor cerebri as an associated condition. This syndrome has been infrequently reported as a primary feature of central nervous system involvement by vasculitides or collagen vascular disease such as systemic lupus erythematosus. The authors report a nine-year-old child who presented with pseudotumor cerebri as a major sign of systemic vasculitis. Although a vasculitis was suspected and high dose gluco-corticosteroids administered, it was only after a sudden fatal pulmonary hemorrhage that the postmortem examination revealed the overwhelming nature of the vasculitis, consistent with the polyangiitis overlap syndrome. This is the first reported case of pseudotumor cerebri in association with the polyangiitis overlap syndrome. Familiarity with ocular signs of vasculitides may facilitate early diagnosis and specific treatment.

Key Words:

• polyarteritis nodosa
• polyangiitis overlap syndrome
• pseudotumor cerebri
• vasculitis