IgG4-related inflammatory pseudotumor presenting with refractory seizures: a diagnostic pitfall.
Polyana Favero Ferreira Caetano, Vitor Ribeiro Paes, Carlos Eduardo Roelke et al.
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Case in brief
A patient with a 10-year history of immunoglobulin G4-related disease (IgG4-RD) presented with refractory focal seizures, which later progressed to a tumefactive brain lesion. The diagnosis was complicated by the condition's ability to mimic other diseases. Histopathological analysis revealed storiform fibrosis and IgG4-positive plasma cell infiltration, confirming the diagnosis. The patient's management involved addressing the underlying IgG4-RD.
What made this case unusual
The case illustrates the atypical neurological presentation of IgG4-RD, initially manifesting as functional seizures without structural brain abnormalities, complicating the diagnostic process.
Diagnostic / clinical pearl
Clinicians should maintain a high index of suspicion for IgG4-related disease in patients with refractory seizures and atypical neurological symptoms, as early diagnosis can significantly impact management.
Why it matters
This case underscores the importance of recognizing IgG4-RD as a potential cause of neurological symptoms, which may often be overlooked due to its rarity and similarity to other conditions.