Hypertrophic spinal pachymeningitis with thoracic myelopathy: the initial presentation of ANCA-related systemic vasculitis

Joseph D Smucker; Austin J Ramme; Richard F Leblond; Leslie A Bruch; Gholamreza Bakhshandehpour

doi:10.1097/BSD.0b013e3182067abf

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Journal article

Hypertrophic spinal pachymeningitis with thoracic myelopathy: the initial presentation of ANCA-related systemic vasculitis

Joseph D Smucker, Austin J Ramme, Richard F Leblond, Leslie A Bruch and Gholamreza Bakhshandehpour

Journal of spinal disorders & techniques, Vol.24(8), pp.525-532

12/2011

DOI: 10.1097/BSD.0b013e3182067abf

PMID: 21436740

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Abstract

A retrospective case review combined with a review of current literature. We describe a case of antineutrophil cytoplasmic antibodies (ANCA)-related systemic vasculitis, with nearly 4 years of clinical and radiographic follow-up, initially presenting as hypertrophic spinal pachymeningitis (HSP). The diagnosis, surgical, and medical treatment of HSP are discussed in the context of a literature review. HSP is a rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. HSP has been associated with infectious, inflammatory, autoimmune, and traumatic conditions. Surgical decompression and/or corticosteroid therapy have been shown effective at managing this disorder, but identifying associated diseases can be essential to a favorable patient outcome. The medical, pathologic, and radiographic records of this case were reviewed. A computer-based search of the PubMed database was used to perform a comprehensive literature review of HSP. We report a 66-year-old male with a history of renal cell carcinoma and bladder cancer who presented with upper abdominal pain, thoracic myelopathy (progressive weakness and numbness of the lower extremities, and gait disturbances), and weight loss. A diagnosis of HSP was subsequently shown to be the initial presentation of ANCA-related systemic vasculitis. The possibility of an ANCA-related systemic vasculitis presenting as HSP without systemic signs is a diagnostic and therapeutic challenge for the spinal surgeon. The diagnosis of HSP should initiate a search for an associated disease process and close follow-up after initial treatment. With interdisciplinary collaboration between medicine, radiology, pathology, and orthopedics, the diagnosis was made and a treatment initiated which halted disease progression and has maintained remission for more than 4 years.

Spinal Cord Diseases - diagnosis

Diagnosis, Differential

Thoracic Vertebrae - diagnostic imaging

Humans

Meningitis - surgery

Male

Treatment Outcome

Meningitis - etiology

Spinal Cord Diseases - surgery

Radiography

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - diagnosis

Spinal Cord Diseases - etiology

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - complications

Meningitis - diagnosis

Aged

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - surgery

Thoracic Vertebrae - pathology

Details

Title: Subtitle: Hypertrophic spinal pachymeningitis with thoracic myelopathy: the initial presentation of ANCA-related systemic vasculitis
Creators: Joseph D Smucker - Department of Orthopaedics and Rehabilitation, University of Iowa, Iowa City, IA 52242, USA. joseph-smucker@uiowa.edu
Austin J Ramme
Richard F Leblond
Leslie A Bruch
Gholamreza Bakhshandehpour
Resource Type: Journal article
Publication Details: Journal of spinal disorders & techniques, Vol.24(8), pp.525-532
Publisher: United States
DOI: 10.1097/BSD.0b013e3182067abf
PMID: 21436740
ISSN: 1536-0652
eISSN: 1539-2465
Grant note: T32 GM007337 / NIGMS NIH HHS
Language: English
Date published: 12/2011
Academic Unit: Pathology; Neurosurgery; Internal Medicine
Record Identifier: 9984040324002771

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