- Department of Neurosurgery, SCB Medical College and Hospital, Cuttack, Odisha, India
Satya Bhusan Senapati
Department of Neurosurgery, SCB Medical College and Hospital, Cuttack, Odisha, India
DOI:10.4103/2152-7806.130907Copyright: © 2014 Senapati SB. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Senapati SB, Mishra SS, Das S, Parida DK, Satapathy MC. Cranio cervical tuberculous hypertrophic pachymeningitis. Surg Neurol Int 16-Apr-2014;5:52
How to cite this URL: Senapati SB, Mishra SS, Das S, Parida DK, Satapathy MC. Cranio cervical tuberculous hypertrophic pachymeningitis. Surg Neurol Int 16-Apr-2014;5:52. Available from: http://sni.wpengine.com/surgicalint_articles/cranio-cervical-tuberculous-hypertrophic-pachymeningitis/
Background:Hypertrophic pachymeningitis is a unique clinical entity characterized by fibrosis and thickening of dura mater resulting in neurological dysfunction. It could be idiopathic or due to variety of inflammatory and infectious conditions. Tuberculous hypertrophic pachymeningitis involving cranio cervical region is rarely reported.
Case Description:A 50-year-old female presented with history of progressive quadriparesis and stiffness of neck for 2 years, dysphagia to liquid for past 3 months. Her condition rapidly deteriorated when another physician prescribed her corticosteroid. Physical examination revealed high cervical compressive myelo-radiculopathy with lower cranial nerve palsy and neck rigidity. Series of serum analysis, cerebrospinal fluid (CSF) study and contrast magnetic resonance imaging (MRI) clinched the diagnosis. She improved on antitubercular treatment.
Conclusion:In case of multilevel cervical compressive myelo-radiculopathy with lower cranial involvement, possibility of hypertrophic pachymeningitis should be kept in mind. Before diagnosing it as idiopathic, infectious causes should be excluded otherwise prescription of corticosteroid will flare up the disease process.
Keywords: Cranio cervical, hypertrophic pachymeningitis, tuberculous
Hypertrophic thickening of the meninges can be caused by a variety of pathological processes such as inflammatory (tuberculosis, fungal, Lyme's disease, syphillis, Human T-lymphotropic virus (HTLV), collagen vascular disorders (rheumatoid arthritis, Wegner's granulomatosis, systemic lupus erythematosus, mixed connective tissue disease), multifocal fibrosclerosis, neoplasia (carcinoma, lymphoma, meningioma en plaque), and miscellaneous disorders such as sarcoidosis, hemodialysis, mucopolysaccharidosis, and intrathecal drug administration.[
A 50-year-old female presented with history of progressive quadriparesis and stiffness of neck for 2 years, dysphagia to liquid for past 3 months. She had visited several doctors without any relief for the said complaints. Magnetic resonance imaging (MRI) of cervical spine was done one year back. On the basis of clinical history and supportive MRI finding, she was previously diagnosed as a case of high cervical compressive myelopathy with cord changes due to thickening of ligamentum flavum and posterior longichudinal ligament extending from foramen magnum up to C4 vertebral level. Corticosteroid was prescribed by her previous physician. Following which her condition deteriorated rapidly.Her neck became so stiff that she was unable to move it, numbness extended to all over her body; she developed dysphagia to liquid and became bed ridden. Physical examination revealed high cervical compressive myelo-radiculopathy with lower cranial nerve palsy and rigid neck. Recent contrast MRI was suggestive of circumferentially, grossly thickened, enhancing dura extending from caudal aspect of posterior fossa up to C7 level with variable compression of cord and emerging nerve roots [Figures
HPM is a rare disorder of diverse etiology. Charcot and Joffroy[
In the first stage of spinal HPM, patients experience local and radicular pain. The second stage can present signs of nerve root compression. In addition, in the third stage, patients eventually suffer from spinal cord compression.[
HPM is being increasingly recognized with the advent of computed tomography (CT) and MRI. On MRI, thickened dura mater appears isointense or hypointense on T1WI and hypointense on T2WI sequence with a hyperintense edge, which is best seen in coronal or sagittal sections.[
The natural course of IHPM is poorly understood. Spontaneous resolution, response to steroids, steroid dependency, remitting, and relapsing course have been documented.[
In case of multilevel cervical compressive myelo-radiculopathy with lower cranial involvement, possibility of HPM should be kept in mind. Before diagnosing it as idiopathic, infectious causes should be excluded; otherwise prescription of corticosteroid will flare up the disease process. Though early surgical excision has been documented for IHPM, a course of antitubercular treatment seems appropriate for tubercular causes.
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