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Cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis: a case report and
Rui Gu1, Peng-Yuan Hao1, Jia-Bei Liu2
1Department of Orthopedics, China-Japan Union Hospital of Jilin University, 126 Xiantai Blvd, Changchun, 130033, People's Republic of China.
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.
Insights
This case report details a rare instance of cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis (IgG4-RSP). Early surgical decompression and pathological diagnosis are crucial for managing this condition.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related sclerosing pachymeningitis (IgG4-RSP) is a rare, newly described fibrotic inflammatory condition.
- Spinal cord compression can result from various pathologies, necessitating accurate diagnosis and timely intervention.
Observation:
- A 43-year-old male presented with progressive neck pain, lower limb motor and sensory dysfunction, and bowel/bladder issues.
- Initial presentation mimicked spinal cord compression from hematoma, leading to emergency surgical decompression.
- Post-operative recovery showed sensory improvement, but motor deficits persisted.
Findings:
- Pathological examination confirmed IgG4-related sclerosing pachymeningitis (IgG4-RSP) as the cause of cervicothoracic spinal cord compression.
- This represents one of the few reported cases globally and the first in China.
- The patient received combined therapy including dexamethasone, mannitol, and neurotrophic drugs.
Implications:
- This case highlights the importance of considering IgG4-RSP in the differential diagnosis of spinal cord compression.
- Prompt surgical decompression is vital for neurological recovery.
- Accurate pathological diagnosis is essential for guiding comprehensive treatment strategies for IgG4-RSP.
