Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Print this page Email this page Users Online: 364

  Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 15  |  Issue : 4  |  Page : 587-590  

Idiopathic hypertrophic pachymeningitis as a rare cause of spinal cord compression


Department of Neurology, Dr DY Patil Medical College, Hospital and Research Centre, Dr DY Patil Vidyapeeth, Pune, Maharashtra, India

Date of Submission06-Jan-2021
Date of Decision25-Apr-2021
Date of Acceptance25-Apr-2021
Date of Web Publication28-Jan-2022

Correspondence Address:
Furqan Mohd Akram Khan
Department of Neurology, Dr. D. Y. Patil Vidyapeeth, Dr. D. Y. PatilMedical College, Hospital and Research Centre, Pimpri, Pune - 411 018, Maharashtra
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_9_21

Rights and Permissions
  Abstract 


Idiopathic hypertrophic pachymeningitis (IHP) is a rare inflammatory disease characterized by inflammation and hypertrophy of the dura mater. Spinal form of IHP is extremely rare. We report an idiopathic hypertrophic spinal pachymeningitis case, a 67-year-old female who presented with neck and upper back pain with progressive paraparesis and sensory level. Her magnetic resonance imaging cervico-dorsal spine showed abnormal epidural hypointensity on T2-weighted images from C5 to T4 with significant cord compression and intramedullary cord signal changes at T2 and T3 levels. The lesional biopsy was done suggestive of chronic inflammatory changes. She was treated with steroids and was able to walk unaided.

Keywords: Compressive myelopathy, idiopathic hypertrophic pachymeningitis, idiopathic hypertrophic spinal pachymeningitis


How to cite this article:
Dave D, Akram Khan FM, Rohatgi S, Nirhale S, Rao P, Naphade P. Idiopathic hypertrophic pachymeningitis as a rare cause of spinal cord compression. Med J DY Patil Vidyapeeth 2022;15:587-90

How to cite this URL:
Dave D, Akram Khan FM, Rohatgi S, Nirhale S, Rao P, Naphade P. Idiopathic hypertrophic pachymeningitis as a rare cause of spinal cord compression. Med J DY Patil Vidyapeeth [serial online] 2022 [cited 2022 Jul 5];15:587-90. Available from: https://www.mjdrdypv.org/text.asp?2022/15/4/587/336716




  Introduction Top


Idiopathic hypertrophic pachymeningitis (IHP) is a rare inflammatory disease characterized by inflammation and hypertrophy of the dura mater. This rare disease is mostly found intracranially. The spinal form of IHP is rarer and first described by Charcot and Joffroy with its first name as “Pachymeningitis hypertrophica cervicalis” in 1869.[1] It has varied clinical presentations from local and radicular pain to radiculopathy and also myelopathy. Although many associated diseases have been suggested, the etiology of idiopathic hypertrophic spinal pachymeningitis (IHSP) is not well understood. We report a case of a 67-year-old female who presented with neck and upper back pain with progressive paraparesis and sensory level. Her magnetic resonance imaging (MRI) cervico-dorsal spine showed abnormal epidural hypointensity on T2-weighted images from C5 to T4 with significant cord compression and intramedullary cord signal changes at T2 and T3 levels. The lesional biopsy was done suggestive of chronic inflammatory changes. She was treated with steroids and was able to walk unaided. This case report highlights the features of IHSP, focusing on its MRI features and histological findings which are important in diagnosis and its management approach.


  Case Report Top


A 67-year-old female presented with progressive asymmetric paraparesis with diminished sensation below costal margins for 1 month. There is no history of weakness in the upper limbs or bladder and bowel involvement. She had complaints of worsening neck pain radiating to both arms and upper and mid back pain radiating to the paravertebral area for 2 years. There is no history of asymmetric paraparesis with sensory level 1 year back which recovered gradually over 2 months after taking steroids. There was neither noticeable history of fever and trauma nor any contributable family medical history.

On examination, Grade II modified Ashworth scale spasticity was noted in both lower limbs with the power of 2/5 at all the joints in all range of movements, brisk knee, and ankle jerks bilaterally and extensor plantars. The motor examination of upper limbs was normal. She had diminished touch, pain, and temperature sensation with a level at T5 and vertebral level T3.

Her MRI showed abnormal epidural hypointensity on T1 and T2-weighted images from C5 to T4 along the posterior margins of the corresponding vertebral bodies [Figure 1]. This hypointensity is also noted along the posterior and lateral margin of the thecal sac, especially in the upper dorsal area. Significant cord compression is seen, most marked at T2 and T3 level [Figure 2]. This was associated with intramedullary cord signal changes. The epidural signal abnormality shows enhancement on the postcontrast study, which was especially along the inner margin of the abnormal tissue [Figure 3].
Figure 1: Sagittal (a) T1 and (b) T2-weighted MRI, showing a hypointense epidural lesion C5 to T4 along the posterior margins of the corresponding vertebral bodies

Click here to view
Figure 2: Axial T2-weighted magnetic resonance imaging, showing a hypointense epidural lesion causing significant cord compression

Click here to view
Figure 3: Sagittal gadolinium-enhanced T1-weighted magnetic resonance imaging showing enhancement on the postcontrast study, which was especially along the inner margin of the abnormal tissue

Click here to view


Her MRI brain contrast study was normal. Her routine laboratory investigations including erythrocyte sedimentation rate and C-reactive protein were normal.

Her autoimmune workup including ANA (IFA), ANA Blot, P-ANCA, and C-ANCA was normal. Workup for sarcoidosis and tuberculosis was negative and included that serum calcium, serum ACE levels, tuberculin test, high-resolution computed tomography, and contrast-enhanced computed tomography chest were normal, and there was no hilar lymphadenopathy. Ultrasonography lymph nodes screening showed no lymphadenopathy. Her serum IgG4 levels were normal. Cerebrospinal fluid CSF analysis was normal except for mildly elevated proteins with no atypical cells on cytology.

She was started on injection dexamethasone 8 mg intravenous 8 hourly, her power improved and was able to stand and walk with support by the end of 2 weeks.

She underwent laminectomy and dural biopsy.

The biopsy specimen showed fibro collagenous tissue displaying hyalinization with patchy lymphoplasmacytic infiltrate admixed with histiocytes and acute inflammatory cells [Figure 4]. There are no definite epithelioid granulomas or giant cells. Special stain for acid-fast bacilli and fungal organisms (PAS, GMS) were negative.
Figure 4: Intermediate-magnification photomicrograph (H and E, ×100) showing focal aggregates of plasma cells and lymphocytes with dense fibrous tissue

Click here to view


Immunohistochemistry showed CD3 and CD20 stains reactive T cells and B cells, respectively, and MIB-1 stain reactive few scattered inflammatory cells.

Based on her MRI findings, biopsy report, and laboratory investigations, diagnosis of IHSP causing cord compression was made.

Oral prednisolone 1 mg/kg was continued along with rehabilitation postoperative. Two months later, the patient was able to walk unaided, and almost complete recovery from the neurologic symptoms was seen by the end of 2 months.


  Discussion Top


IHP is a rare disease characterized by hypertrophic inflammation of the dura mater. It is mostly found intracranially, and spinal form of IHP is very rare. In 1869, Charcot and Joffroy first described the spinal form of IHP. Charcot and Joffroy described three stages; in the first stage, there is local and radicular pain. In the second stage, there are nerve root compression signs. In the final stage, there is spinal cord compression. Our patient experienced all stages.[1]

The etiology of hypertrophic spinal pachymeningitis (HSP) includes trauma, connective tissue disorders, autoimmune disorders, and infections. Furthermore, spinal anesthesia, intrathecal steroids, and intrathecal contrast agents for myelography have been reported to cause HSP.[2] Furthermore, IgG4-related disorders are also known to cause HSP.[3] Despite this, in many cases, no cause has been found and is then labeled as IHSP. In our case, we could not find any cause despite thorough investigations.

On MRI, the dural-based mass of low T2 signal with enhancement extending over multiple levels has been described in IHSP. The linear enhancement pattern has got a better therapeutic response as compared to the nodular form, possibly related to less fibrosis and more vascularity.[4],[5],[6],[7],[8] Friedman and Flanders described that the peripheral enhancement was highly suggestive of hypertrophic pachymeningitis. It is caused by inflammation along the periphery of lesion.[9]

Typical pathologic features of IHSP are dural thickening with chronic inflammatory infiltrate of plasma cells and lymphocytes, occasionally, eosinophils, polymorphonuclear cells, or giant cells are seen. This case also shows typical radiologic and pathologic features observed in the literature.

The management of IHSP is controversial. Surgical decompression by laminectomy and dural excision is recommended. Biopsy with steroids can decrease the thickness of dura and show improvement in neurologic deficits. In decompression, laminoplasty is recommended over extensive laminectomy to enhance the stability of the spine. Furthermore, the role of postoperative radiotherapy, immunosuppression such as azathioprine, and cyclophosphamide has been emphasized.[2] Our case has shown significant improvement with limited laminectomy for biopsy and steroid therapy. Azathioprine was added as a steroid-sparing agent to prevent relapses.


  Conclusion Top


IHSP is a rare disorder with characteristic radiological findings. On MRI, gadolinium-enhancing thickened dura gives a clue to the diagnosis. However, IHSP is a diagnosis of exclusion. Connective tissue and autoimmune disorders need to be ruled out as a cause of hypertrophic pachymeningitis. Therefore, a thorough workup is required before labeling it as IHSP. Radiographic and pathological confirmation with the exclusion of other known etiologies is the cornerstone for its diagnosis. At present, decompression surgery followed by immunosuppressive therapy remains the mainstay of treatment. Surgery serves the dual purpose of achieving biopsy and relieving compression over the spinal cord.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Charcot JM, Joffroy A. Two cases of muscle atrophy progressive with lesions of the gray matter and the bundles anterolateral of the spinal cord. Arch Physiol Norm Pathol 1869;2:744-60.  Back to cited text no. 1
    
2.
Kim JH, Park YM, Chin DK. Idiopathic hypertrophic spinal pachymeningitis: Report of two cases and review of the literature. J Korean Neurosurg Soc 2011;50:392-5.  Back to cited text no. 2
    
3.
Kim SH, Kim JS. Immunoglobulin G4-related hypertrophic pachymeningitis mimicking chiari malformation. J Clin Neurol 2016;12:238-40.  Back to cited text no. 3
    
4.
Ashkenazi E, Constantini S, Pappo O, Gomori M, Averbuch-Heller L, Umansky F. Hypertrophic spinal pachymeningitis: Report of two cases and review of the literature. Neurosurgery 1991;28:730-2.  Back to cited text no. 4
    
5.
Mikawa Y, Watanabe R, Hino Y, Hirano K. Hypertrophic spinal pachymeningitis. Spine (Phila Pa 1976) 1994;19:620-5.  Back to cited text no. 5
    
6.
Botella C, Orozco M, Navarro J, Riesgo P. Idiopathic chronic hypertrophic craniocervical pachymeningitis: Case report. Neurosurgery 1994;35:1144-9.  Back to cited text no. 6
    
7.
Kanamori M, Matsui H, Terahata N, Tsuji H. Hypertrophic spinal pachymeningitis. A case report. Spine (Phila Pa 1976) 1997;22:1787-90.  Back to cited text no. 7
    
8.
Sridhar K, Vasudevan MC. Idiopathic chronic hypertrophic pachymeningitis causing thoracic cord compression. Br J Neurosurg 2004;18:515-7.  Back to cited text no. 8
    
9.
Friedman DP, Flanders AE. Enhanced MR imaging of hypertrophic pachymeningitis. AJR Am J Roentgenol 1997;169:1425-8.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
   
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
   Abstract
  Introduction
  Case Report
  Discussion
  Conclusion
   References
   Article Figures

 Article Access Statistics
    Viewed307    
    Printed7    
    Emailed0    
    PDF Downloaded12    
    Comments [Add]    

Recommend this journal