Cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis: a case report and literature review.

Purpose: To report a case of cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis (IgG4-RSP).

Methods: A 43-year-old male patient presented with 'neck pain for 15 days, exacerbated accompanying motor and sensory dysfunction of lower limbs with bowel and bladder dysfunction for 4 days' was admitted to our department. Combined with the history of 'acupuncture treatment', MRI results and rapid-developing progression, we considered the great possibilities of spinal cord compression by intradural hematoma and timely performed the emergency operation of cervical double-door laminoplasty and thoracic decompression with internal fixation.

Results: After combined therapy of dexamethasone, mannitol and neurotrophic drugs, sensory recovery of lower limbs started at the fifth day after operation and the sensory function became normal at the fourteenth day after operation with still complete loss of muscle strength. Pathological examination strongly suggested the diagnosis of IgG4-related sclerosing pachymeningitis (IgG4-RSP).

Conclusions: IgG4-related sclerosing pachymeningitis (IgG4-RSP) is a newly recognized disease. This case of cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis (IgG4-RSP) has never been reported in China with merely three case reports worldwide. Prompt surgical decompression is recommended and pathological examination is essential for diagnosis and comprehensive treatment.