Weekly Case

Title : Case 654

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)


 


Diagnosis:

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



Correct Answer
Total applicants 28 Correct answers 5
Name Institution
김기욱 국군수도병원, 전문의
김지은 서울대학교병원, 전문의
김동언 국군양주병원, 전문의
한유비 병무청, 전문의
박경리 한양대학교병원, 전공의
Semi-Correct Answer
Total applicants 28 Semi-Correct answers 11
이진영 전문의
이규정 고대안암병원, 전문의
최지영 전공의
장성원 중앙보훈병원, 전공의
배재흥 전공의
김유진 전문의
권기언 명지병원, 전공의
최현일 전공의
백승진 분당차병원, 전공의
박영태 충북대학교병원, 전공의
조영민 전문의


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