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Washington University Experience | MISCELLANEOUS | Idiopathic Hypertrophic Pachymeningitis (IgG4 Disease) | 3A0 Case 3 History
Case 3 History ---- The patient is a 60-year-old man who presented with left sub-occipital abnormal neural enhancement, suggestive of underlying neoplasm. Operative procedure: Left sub-occipital craniotomy biopsy. ---- The findings were those of a chronic pachymeningitis with a heterogenous inflammatory infiltrate. This histologic picture includes increased IgG4-positive plasma cells; originally a form of pachymeningitis, now designated autoimmune/sclerosing IgG4 disease. Criteria for diagnosis varies significantly from organ to organ and has been a point of disagreement between experts. The association of phlebitis described in many sites suggests the possibility that underlying involvement of the transverse sinus in this case may have resulted explaining its partial obstruction. The patient is currently alive with persistent MRI findings more than two years later. Compared to more classical cases this patient's disease is modest in dural thickening and inflammation but does show prominence of IgG4 containing elements
