gms | German Medical Science

Objective: In clinical practice we are confronted with rare cervical processes, which can’t be attributed to a clear etiology. Pachymeningitis cervicalis hypertrophica (PCH) is an extremely rare, chronic, nonspecific, idiopathic or secondary disease causing chronic granulomatous inflammatory and fibrotic hypertrophy of the dura characterised by radiculomyelopathy resulting from dural thickening. The etiology of the PCH is usually obscure, although idiopathic, infection, trauma, toxins, metabolic disease and rheumatoid arthritis have all been implicated. Pachymeningitis hypertrophoica has been found intracranially as well as in all segments of the spinal cord. Until now, a definite diagnosis can be only made by biopsy. The treatment of choice is surgical decompression and/or immunosuppressive therapy. We are reporting a case of IPCH causing cervical radiculopathy and paralysis of the right arm, not responding to surgery, but well to immunosuppressive therapy.

Methods: A fifty-seven-year-old male presented with a five-day history of increasing pain in his upper back and the right arm, associated with paralysis and numbness of the right arm before hospitalization. On magnetic resonance imaging, these thickened dura showed homogeneous and strong enhancement in the right nerve roots between C3 and C6, especially neuroforamina C4/5. Cerebrospinal fluid (CSF) analysis and blood examination were normal.

Results: A cervical right laminectomy was performed in C4/5. After the operation, the patient had a complete paralysis of the right shoulder abduction and right elbow flexion. The histological examination of the dural thickening showed marked nonspecific chronic perivascularly inflammatory infiltration with numerous plasma cells and mixed-celled inflammation. After the histological examination the patient was subsequently treated with high dose prednisone for 3 weeks, which has been tapered in the further course. Cervical spine MRI with full contrast enhancement after 2 weeks revealed marked regression of nerve roots lesion; however, a remaining lesion is still seen. The arm pain resolved postoperatively and the right-sided paralysis resolved within 2 weeks. One month after surgery, his only neurological deficit was very mild weakness of the right shoulder abduction.

Conclusion: The thickening of the meninges in the MRI is indicative, but not proving for pachymeningitis. The majority of the reported biopsies of IPCH were taken mainly during surgery after frustrating diagnosis and therapy. The diagnosis and treatment regimens of IPCH are therefore based on empirical data and are not "evidence-based". In case of spinal forms of IPCH early surgery is recommended. In our case the surgery has not improved the paralysis, but it was necessary for the diagnosis. Laminoplasty should be preferred to laminectomy due to spine instability.