Case report reveals recurrent posterior circulation stroke secondary to suspected IgG4-related disease in an older male, highlighting an unusual inflammatory etiology for cryptogenic stroke.
Background: IgG4-related disease is a chronic fibro-inflammatory disorder that infrequently affects the central nervous system, leading to a heterogeneous array of clinical syndromes. Case report: We report a case of a 64-year-old man presenting with recurrent posterior circulation stroke and lymphadenopathy secondary to suspected IgG4-RD. He was diagnosed on a combination of cerebellar leptomeningeal MRI-brain enhancement, markedly elevated serum IgG4 levels and suggestive IgG4-RD lymph node histopathology. Outcome: The patient was successfully treated with corticosteroids and rituximab. Conclusion: This case underscores the importance of recognizing IgG4-RD as a potential rare cause of cryptogenic stroke in the right clinical context. This is one of few reported cases in which recurrent posterior circulation stroke was the presenting manifestation of IgG4-RD.
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Dragaš et al. (2026) studied this question.
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