Age / Sex : 59 / F

Chief complaint : neck and shoulder pain, right upper extremity numbness

What is your diagnosis?

Two weeks later, you can see the final diagonosis with a brief discussion of this case (Please submit only one answer).

Courtesy : 김보람, Bo Ram Kim 분당서울대학교병원 (Seoul National University Bundang Hospital)

Hypertrophic spinal pachymeningitis

Discussion

Findings:

T2 weighted MR images shows a hypointense dural-based intradural extramedullary and extradural mass spanning vertebral levels C6 through T1. The mass is hypointense to the spinal cord on T1 weighted image. Contrast enhanced T1 weighted image shows intense enhancement. There is also abnormal increased cord signal at the level of compression.

Differential Diagnosis:

Epidural hematoma, spinal meningioma, lymphoma

Discussion:

Hypertrophic spinal pachymeningitis (HSP) is a subtype of hypertrophic pachymeningitis, a rare disease characterized by localized or diffuse hypertrophic thickening of the dura matter. It has been implicated in a variety of inflammatory and infectious processes, including rheumatoid arthritis, Wegener granulomatosis, sarcoidosis, heavy chain disease, tuberculosis, syphilis, and fungal infections, as well as in trauma

 HSP is thought to have a predilection for the cervical and thoracic regions of the spine. As the dura mater becomes inflamed and hypertrophic, it gradually occupies more space within the spinal canal, and signs and symptoms of neurologic compromise may manifest. 

 At histologic analysis, HSP shows active inflammation in the lesion periphery, with a central zone of dense fibrosis. Specific findings include an extramedullary mass extending over multiple vertebral levels, a strongly hypointense signal on T2-weighted images, and a pattern of intense peripheral margin enhancement. All three of these findings were present in this patient. On T1-weighted images, HSP is often iso- to hypointense to the spinal cord, and on T2-weighted images, a thin edge of hyperintensity sometimes can be seen. A clinical history of chronic gradually progressive myelopathic symptoms provides an additional clue to the diagnosis.

References:

1. Pai S, Welsh CT, Patel S, Rumboldt Z. Idiopathic hypertrophic spinal pachymeningitis: report of two cases with typical MR imaging findings. AJNR Am J Neuroradiol 2007; 28(3):590–592


2. Smucker JD, Ramme AJ, Leblond RF, Bruch LA, Bakhshandehpour G. Hypertrophic spinal pachymeningitis with thoracic myelopathy: the initial presentation of ANCA-related systemic vasculitis. J Spinal Disord Tech 2011;24(8):525–532


3. Yunokawa K, Hagiyama Y, Mochizuki Y, Tanaka N, Ochi M. Hypertrophic spinal pachymeningitis associated with heavy-chain disease. Case report. J Neurosurg Spine 2007; 7(4):459–462