Case 342: IgG4-related Hypertrophic Pachymeningitis

Area of Science:

• Neurology
• Oncology
• Immunology

Background:

• A 70-year-old woman presented with severe, progressive midback pain unresponsive to conventional analgesics.
• The pain radiated to the lower ribs and worsened with positional changes, significantly impacting daily life.

Purpose of the Study:

• To investigate the cause of severe midback pain in a 70-year-old woman.
• To diagnose and manage a rare case of spinal leptomeningeal disease.

Main Methods:

• Diagnostic workup included lumbar puncture with cerebrospinal fluid (CSF) analysis, revealing elevated protein, mildly decreased glucose, and increased lymphocytes.
• Cerebrospinal fluid (CSF) cytology and flow cytometry showed small lymphoid cells without clonal proliferation, and oligoclonal bands were detected.
• Spinal and brain imaging (CT and MRI) were performed, along with chest, abdomen, and pelvis CT scans.

Main Results:

• Cerebrospinal fluid (CSF) analysis indicated an inflammatory process with elevated protein and lymphocyte count.
• Despite negative infectious and autoimmune panels, the presence of CSF oligoclonal bands suggested an underlying immune-mediated or neoplastic process.
• The patient underwent T6 laminectomy and initiated treatment with glucocorticoids and rituximab.

Conclusions:

• The patient was diagnosed with spinal leptomeningeal disease, a rare condition affecting the membranes surrounding the spinal cord.
• Treatment with glucocorticoids and rituximab resulted in symptom improvement, as evidenced by follow-up imaging.