Nishel Kothari1, Mohammad Naseem2, Mary Feldman3
1Dartmouth-Hitchcock Medical Centre, 2Maulana Azad Medical college, 3Neurology/movement disorder, Dartmouth Hitchcock Medical Center
Objective:
This case outlines IgG4-RD manifesting as an enhancing left temporal mass in a man who presented with the symptoms of confusion and headaches. Also emphasis on the responsiveness to rituximab as a treatment modality to prevent recurrence.
Background:
A 69-year-old man with emphysema presents with months of progressively worsening headaches along with increasing confusion. Findings on neurologic examination demonstrated disorientation to place/time, mild dysarthria, and poor naming and recall. MRI showed a left anterior temporal lesion with heterogeneous hyperintensity on T2-weighted imaging with gyri form contrast enhancement along with extensive leptomeningeal enhancement and surrounding vasogenic edema thought to be consistent with primary brain neoplasm. LP demonstrated an elevated protein with lymphocytic predominance with a negative infectious and autoimmune workup. CT chest showed multiple lung nodules, the largest one measuring 10 mm with a spiculated appearance. He underwent mass resection, pathology showed extensive leptomeningeal fibrosis, prominent lymphoplasmacytic infiltrates, and scattered eosinophils with cortical vessels with mononuclear infiltration. Immunostaining confirmed up to 50 IgG4-positive plasma cells per high-power field confirming the diagnosis of IgG4-related disease.
Results:
He was started on high-dose prednisone which was tapered over 6 months. Due to the high incidence of recurrence, Rituximab as a steroid-sparing agent. Follow-up MRI brain showed improvement of cerebral and meningeal enhancement consistent with a positive response to therapy.
Conclusions:
IgG4-RD is a systemic disease characterized by fibroinflammatory changes with infiltrations of IgG4-predominant plasma cells and eosinophils. Nervous system involvement is observed less frequently, and instances of brain parenchymal involvement, as demonstrated in this case, are even rarer, with most cases manifesting as hypertrophic pachymeningitis, and inflammatory lesions of the pituitary gland. Our case involved lung nodules which resolved after treatment, making the diagnosis crucial as they can be mistaken for infections or malignancy. This case highlights the successful management through surgical resection, corticosteroids, and rituximab.
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