Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 8096  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
  »  Article in PDF (1,346 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  

 
  In this Article
 »  References
 »  Article Figures
 »  Article Tables

 Article Access Statistics
    Viewed167    
    Printed2    
    Emailed0    
    PDF Downloaded6    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents    
LETTERS TO EDITOR
Year : 2019  |  Volume : 67  |  Issue : 5  |  Page : 1380-1385

Idiopathic Hypertrophic Spinal Pachymeningitis: A Rare Cause of Spinal Cord Compression


Department of Neurosurgery, Government Medical College, Thiruvananthapuram, Kerala, India

Date of Web Publication19-Nov-2019

Correspondence Address:
Dr. Raja K Kutty
Department of Neurosurgery, Government Medical College, Thiruvananthapuram - 695 011, Kerala
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.271262

Rights and Permissions



How to cite this article:
Kutty RK, Sreemathyamma SB, Sivanandapanicker JL, Peethambaran A. Idiopathic Hypertrophic Spinal Pachymeningitis: A Rare Cause of Spinal Cord Compression. Neurol India 2019;67:1380-5

How to cite this URL:
Kutty RK, Sreemathyamma SB, Sivanandapanicker JL, Peethambaran A. Idiopathic Hypertrophic Spinal Pachymeningitis: A Rare Cause of Spinal Cord Compression. Neurol India [serial online] 2019 [cited 2019 Dec 8];67:1380-5. Available from: http://www.neurologyindia.com/text.asp?2019/67/5/1380/271262




Sir,

Diseases solely affecting the dura of the spinal cord are rare. Idiopathic hypertrophic spinal pachymeningitis (HSP) is one such progressive inflammatory fibrosing disease of the spinal cord. It was first described by Charcot and Joffroy in 1869.[1] It describes chronic inflammation of the spinal dura. This inflammation results in the thickening and hypertrophy of the dura[2] with compression of the spinal cord. There are various manifestations of this disease ranging from radicular pain[3] to weakness of limbs[4],[5],[6] depending upon the level of involvement of the spinal dura. We describe a case of idiopathic HSP with involvement of the spinal dura at the level of the cervicothoracic region resulting in weakness of both lower limbs.

A 29-year-old otherwise healthy female presented after falling on due to stairs at her home. After two weeks, she developed pain in the neck. This was followed by a gradual onset weakness of both lower limbs. There was no involvement of bowel or bladder. On examination she had a power of 4/5 in both her lower limbs. Deep tendon reflexes were exaggerated from elbow and downwards. There was sensory level at the level of third thoracic vertebra. A diagnosis of compressive myelopathy at the cervicothoracic level was made clinically. Magnetic resonance imaging (MRI) scan revealed a space occupying lesion in the level of the cervicothoracic region which was seen compressing the spinal cord. The lesion was hypointense on T1W and T2W images. A diagnosis of a subacute subdural hematoma was considered in the background of a recent fall at that time. A differential diagnosis of intradural extramedullary tumor was also kept provisional. The patient was managed with steroids after which the power in her lower limbs improved. She was discharged with a gradual course of tapering steroids.

After six months, she again presented with similar complaints as previously. A repeat imaging revealed that the space-occupying lesion in the cervicothoracic region was persisting with same dimensions. It was hypointense on both T1W and T2W images. Upon contrast study, there was a linear peripheral enhancement in the anterior aspect of the lesion [Figure 1]a, [Figure 1]b, [Figure 1]c. The lesion was thickest in the region of the C7–T1 region. There was a hyperintense signal within the cord on T2W images suggestive of myelomalacia. A diagnosis of HSP was suggested from the MRI. Screening of the neuroaxis was performed and was normal. She was investigated for HSP and tested for autoimmune disorders as well as chronic granulomatous infections. ANCA, IgG4, ACA, and VDRL were all found to be negative. Cerebrospinal fluid (CSF) tapping was done which showed increased protein (200 mg/dl), glucose 60 mg/dl, and cells mostly lymphocytes.
Figure 1: (a) T1W MRI sagittal section of cervical spine – spindle-shaped space occupying lesion in the posterior wall of the thecal sac in the region of cervicothoracic region severely compressing the spinal cord anteroposteriorly. (b) T1W sagittal section of cervical spine with contrast – there is linear enhancement in the anterior wall of the lesion representing inflammation and fibrosis (c) T1W axial section of cervical spine – hypointense lesion in the region of the C6 and C7 vertebrae severely compressing the cervical spinal cord anteroposteriorly

Click here to view


At surgery, a laminectomy of C7–T1 was done. The dura was found to be intensely thickened the lesion was incised in the midline and dissected to find the underlying spinal cord [Figure 2]a. It was not possible to find the underlying cord at C7–T1 with a reasonable 1 cm cut. At this point, the skin incision was extended from C2 to T3 vertebrae. Laminectomy was done from C3 to T3 and the lesion was incised in the midline at the superior-most point. The underlying dura was seen compressed at this region. The lesion was then slowly dissected from the underlying spinal cord from the cranial to caudal direction. During this, the cord was seen severely compressed at the region of C7–T1. The lesion was found to be adherent to the exiting roots of the spinal cord and it was excised near totally [Figure 2]b. An expansile duroplasty was done using the synthetic dura G-Patch (Surgiwear Inc.) since the cord was sufficiently decompressed. The cut ends of the laminae were replaced and anchored to the facets using miniplates and screws. After she woke up from anesthesia, we observed that the power of her lower limbs had deteriorated to Grade 3/5. She was started on methyl prednisolone and she regained power of her lower limbs to preoperative levels one week later. She was discharged with advice to continue prednisone 20 mg per day.
Figure 2: Intraoperative photograph – (a) Hypertrophied and fibrosed dura – small arrows. The normal spinal cord is seen compressed by the lesion – large arrow. (b) Decompressed spinal cord after near total excision of the hypertrophied dura

Click here to view


The histopathology of the report showed extensive sclerosis with patchy and diffuse areas of inflammatory infiltrates consisting of plasma cells, lymphocytes, and eosinophils [Figure 3]a and [Figure 3]b. A diagnosis of HSP was confirmed.
Figure 3: Photomicrograph – (a) Showing extensive areas of sclerosis with diffuse inflammatory infiltrates consisting of plasma cells, lymphocytes along with neutrophils and eosinophils (H and E, ×10). (b) Showing aggregates of plasma cells and lymphocytes (H and E, ×40)

Click here to view


She was followed up on an outpatient basis during which power both her lower limbs improved considerably and she was able to walk without support. However, the pain in her neck and brisk reflexes persisted which may improve in the future.

HSP was described by Park as a disease characterized by (1) unknown etiology, (2) specific dural involvement, (3) unique radiological features i.e., diffuse thickening of dura with encasement of CNS, and 4) unique histologic features i.e., chronic granulomatous inflammation of the dura.[7] It is an infrequent cause of myelopathy. Due to the advent of better imaging facilities, there has been a surge of reported cases of HSP in the recent literature. Before the MRI era, cases were largely suspected on myelogram and corroborated with histopathology.[8] Our search on the popular medical search engine PubMed revealed 39 cases of idiopathic HSP (IHSP) [Table 1]. Although differences exist with regards to gender predilection of this disease[5] our literature review suggests that this disease manifests equally among both males and females. The most commonly affected patients are in the fifth and seventh decades of life.[5] HSP can be either primary or secondary.[3] Primary or IHSP is a diagnosis of exclusion when other causes of HSP has been ruled out. The secondary form of this disease is frequently associated with various autoimmune disorders like Wegener's granulomatosis; IgG4 related, rheumatoid arthritis, neuro Behçet's;[2],[9],[10],[11],[12],[13] infectious diseases like tuberculosis,[14],[15] syphilis;[16] chronic granulomatous diseases like sarcoidosis;[17] and heavy chain diseases,[18] etc., The most common location of spinal forms of this disease is cervical followed by thoracic region.[3],[19] There have been reports of combined spinal and cranial form of this disease.[14],[20],[21],[22],[23] The cranial form of this disease is mostly located in the base of skull and posterior fossa and presents often as multiple cranial nerve palsies.[24] The most commonly involved cranial nerve is the seventh cranial nerve.[25]
Table 1: Summary of cases of IHSP in the literature

Click here to view


In the spinal form of HSP there is progressive fibrosis and sclerosing hypertrophy of the dura. The dura is thickened and compresses the underlying spinal cord which over the time manifest as myelopathy. The average onset of the disease symptoms varies in individuals from few weeks to months.[5] According to a nationwide survey in Japan, nearly 50% of affected persons have a subacute onset of disease and in the remaining, hypertrophic pachymeningitis exhibits an acute or chronic pattern.[23] There have been many instances in the literature where the disease has been misdiagnosed for other lesions. The most common differential diagnosis considered were epidural abscess,[26],[27],[28] epidural hematoma,[29] epidural lymphoma,[30],[31] subdural hematoma,[5],[31] and intradural extramedullary tumor.[7],[32],[33],[34] Our patient had a history of fall following which she developed pain in the neck associated with weakness of both lower limbs. The possibility of an epidural hematoma was strongly considered in our patient in the first instance. However, she improved with steroids during the first admission only to be readmitted with recurrence of the symptoms after six months. The second MRI showed the lesion of the same dimension and characteristics, which prompted us to seek for an alternate diagnosis.

Radiology of IHSP is vital to the diagnosis. The entire neuroaxis need to be screened as this disease can coexist in both brain and spine concomitantly. In the cranial form, the symptoms can precede the development of the disease by as long as 2 years. The most common finding in this form is thickening of the falx and tentorium.[25] The characteristic imaging findings of IHSP have been described in the review by Olubajo et al. A typical lesion is hypointense on T1- as well as T2-weighted images.[5] However, there have been reports of lesions being hyperintense[35] or isointense[31] on T1W images and hyperintense[7],[31] on T2W images. Upon contrast administration, varied observations have been reported from homogenous, nodular to linear peripheral pattern of enhancement.[3] Our patient had a linear peripheral enhancement in the anterior region of the lesion. This type of peripheral enhancement has been suggested to represent active inflammatory zone and the non-enhancing central part to be fibrosis.[6] The linear pattern of enhancement has also been correlated to be associated with with a better prognosis than homogenous or nodular.[36]

Surgery for IHSP is controversial. Surgical decompression has to be performed in a setting of spinal cord compression. Early surgery has been advocated by Mikawa et al. to minimize the damage to the spinal cord.[37] There have been many instances of patients undergoing multiple surgeries for recurrent disease.[38] The dura in IHSP is thickened and closely adherent to the exiting roots of the spinal cord. Most authors in the literature have limited their maneuvers from, simple biopsy of the lesion[27],[29],[32],[39],[40],[41] to partial excision of the lesion.[5],[7],[28],[24],[34],[42] Very few have attempted a total excision. Early surgery helps to diagnose early and halt the progression of the disease.[43] In our case, during surgery the lesion was found to be very firm and severely compressing the dura. We split the lesion in the midline and excised it near totally and augmented the dural space with duroplasty. This would give adequate room for expansion of the cord.

Steroids are the mainstay in the management of IHSP. Nearly all cases in the literature have been treated with steroids in addition to biopsy/surgery. The management of recurrences of this disease after failure of steroid therapy or cases in which steroids could not be given due to systemic intolerance is debatable. The most commonly used drugs in this scenario are methotrexate,[44],[45] cyclophosphamide,[22] melphalan,[18] and anti-tuberculous therapy.[46]

The outcome of IHSP is variable among the reported literature. Mostly the lesions have remained stable with steroids and surgery, whereas others have had multiple relapses. The recurrence of the lesions is suggested due to the inflammation of the dura present before the surgery and not due to the residual lesion.[26] Tsutsui et al., observed in their review the chronological events in IHSP and suggested that they have either sustained remissions or relapse with corticosteroid resistance or dependence.[41] Increased levels of CSF protein and cells have also been correlated to the recurrence of the disease.[44] Mikawa et al. suggested in their review that patients with IHSP can be divided into two groups according to the presence or absence of systemic inflammation. The patients in the group with systemic signs of inflammation fared worse than without.[37]

IHSP is a rare cause of spinal cord compression. Polyradiculopathy with myelopathy should alert the physician about this possibility. Early surgery in the setting of compression should prevent further damage and enhance recovery. The predictive factors for long-term cure are yet to be ascertained in large cohorts. However, steroids can successfully control the symptoms up to certain extent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Charcot JM, Joffroy A. Deux cas d'atrophie musculaire progressive avec lésions de la substance grise et des faisceaux antérolatéraux de la moelle épinière. Arch Physiol Norm Pathol 1869;2:744-60.  Back to cited text no. 1
    
2.
Rosenfeld JV, Kaye AH, Davis S, Gonzales M. Pachymeningitis cervicalis hypertrophica. Case report. J Neurosurg 1987;66:137-9.  Back to cited text no. 2
    
3.
Fukuda A, Punaro E, Rogério F, de Souza Queiroz L, Reis F. Idiopathic hypertrophic pachymeningitis as a rare cause of cervical compressive myelopathy. J Craniovertebr Junction Spine 2017;8:387-9.  Back to cited text no. 3
    
4.
Kao KP, Huang CI, Shan DE, Ho JT, Chang T, Chu FL, et al. Non-obstructive idiopathic pachymeningitis cervicalis hypertrophica. J Neurol Neurosurg Psychiatry 1986;49:1441-4.  Back to cited text no. 4
    
5.
Olubajo F, Yermakova T, Highley JR, Arzoglou V. Concomitant idiopathic hypertrophic spinal pachymeningitis and Guillain-Barré syndrome in a patient: Coincidence or a triggering mechanism? J Neurosurg Spine 2017;27:335-40.  Back to cited text no. 5
    
6.
Tang R, Li F, Chen Q. A case report of atypical long segmental thoracic hypertrophic pachymeningitis with ossification of ligamentum flavum and literature review. Eur Spine J 2017;26:202-6.  Back to cited text no. 6
    
7.
Park SH, Whang CJ, Sohn M, Oh YC, Lee CH, Whang YJ, et al. Idiopathic hypertrophic spinal pachymeningitis: A case report. J Korean Med Sci 2001;16:683-8.  Back to cited text no. 7
    
8.
Wirth FP Jr., Gado M. Incomplete myelographic block with hypertrophic spinal pachymeningitis. Case report. J Neurosurg 1973;38:368-70.  Back to cited text no. 8
    
9.
Yoon BN, Kim SJ, Lim MJ, Han JY, Lee KW, Sung JJ, et al. Neuro-Behçet's disease presenting as hypertrophic pachymeningitis. Exp Neurobiol 2015;24:252-5.  Back to cited text no. 9
    
10.
Nishino H, Rubino FA, Parisi JE. The spectrum of neurologic involvement in Wegener's granulomatosis. Neurology 1993;43:1334-7.  Back to cited text no. 10
    
11.
Li X, Zhao J, Wang Q, Fei Y, Zhao Y. ANCA-associated systemic vasculitis presenting with hypertrophic spinal pachymeningitis: A report of 2 cases and review of literature. Medicine (Baltimore) 2015;94:e2053.  Back to cited text no. 11
    
12.
Kim SH, Kim JS. Immunoglobulin G4-related hypertrophic pachymeningitis mimicking chiari malformation. J Clin Neurol 2016;12:238-40.  Back to cited text no. 12
    
13.
Ezzeldin M, Shawagfeh A, Schnadig V, Smith RG, Fang X. Hypertrophic spinal pachymeningitis: Idiopathic vs. IgG4-related. J Neurol Sci 2014;347:398-400.  Back to cited text no. 13
    
14.
Yamashita K, Suzuki Y, Yoshizumi H, Takahashi JB, Nogawa T. Tuberculous hypertrophic pachymeningitis involving the posterior fossa and high cervical region – Case report. Neurol Med Chir (Tokyo) 1994;34:100-3.  Back to cited text no. 14
    
15.
Senapati SB, Mishra SS, Das S, Parida DK, Satapathy MC. Cranio cervical tuberculous hypertrophic pachymeningitis. Surg Neurol Int 2014;5:52.  Back to cited text no. 15
[PUBMED]  [Full text]  
16.
Vale TC, Moraes TE, Lara A, Cota GF, Christo PP. Hypertrophic cervical spinal cord pachymeningitis due to treponema pallidum infection. Neurol Sci 2012;33:359-62.  Back to cited text no. 16
    
17.
Subrati N, Vaqas B, Peterson D, Patel MC. Hypertrophic pachymeningitis with sarcoidosis: A rare cause of craniocervical compression. BMJ Case Rep 2015;2015. pii: bcr2014208604.  Back to cited text no. 17
    
18.
Yunokawa K, Hagiyama Y, Mochizuki Y, Tanaka N, Ochi M. Hypertrophic spinal pachymeningitis associated with Heavy-Chain disease. Case report. J Neurosurg Spine 2007;7:459-62.  Back to cited text no. 18
    
19.
Ashkenazi E, Constantini S, Pappo O, Gomori M, Averbuch-Heller L, Umansky F, et al. Hypertrophic spinal pachymeningitis: Report of two cases and review of the literature. Neurosurgery 1991;28:730-2.  Back to cited text no. 19
    
20.
Zhu R, He Z, Ren Y. Idiopathic hypertrophic craniocervical pachymeningitis. Eur Spine J 2015;24 Suppl 4:S633-5.  Back to cited text no. 20
    
21.
Bang OY, Kim DI, Yoon SR, Choi IS. Idiopathic hypertrophic pachymeningeal lesions: Correlation between clinical patterns and neuroimaging characteristics. Eur Neurol 1998;39:49-56.  Back to cited text no. 21
    
22.
Aburahma SK, Anabtawi AG, Al Rimawi HS, Elheis MA, Mohtaseb AH. Idiopathic hypertrophic pachymeningitis in a child with hydrocephalus. Pediatr Neurol 2009;40:457-60.  Back to cited text no. 22
    
23.
Yonekawa T, Murai H, Utsuki S, Matsushita T, Masaki K, Isobe N, et al. Anationwide survey of hypertrophic pachymeningitis in Japan. J Neurol Neurosurg Psychiatry 2014;85:732-9.  Back to cited text no. 23
    
24.
Maheshwari V, Gill M, Kumar M, Srivastava C. Idiopathic hypertrophic cervical pachymeningitis – A rare report. Neurol India 2016;64:1038-40.  Back to cited text no. 24
[PUBMED]  [Full text]  
25.
Lee YC, Chueng YC, Hsu SW, Lui CC. Idiopathic hypertrophic cranial pachymeningitis: Case report with 7 years of imaging follow-up. AJNR Am J Neuroradiol 2003;24:119-23.  Back to cited text no. 25
    
26.
Ito Z, Osawa Y, Matsuyama Y, Aoki T, Harada A, Ishiguro N, et al. Recurrence of hypertrophic spinal pachymeningitis. Report of two cases and review of the literature. J Neurosurg Spine 2006;4:509-13.  Back to cited text no. 26
    
27.
Takahashi H, Wada A, Yokoyama Y, Ishii M, Shibuya K, Suguro T, et al. Idiopathic hypertrophic spinal pachymeningitis: A case report. J Orthop Surg (Hong Kong) 2010;18:113-7.  Back to cited text no. 27
    
28.
Bang JH, Cho KT, Kim EJ. Idiopathic hypertrophic pachymeningitis in the craniocervical junction. Korean J Spine 2015;12:169-72.  Back to cited text no. 28
    
29.
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. 29
    
30.
Hsu HT, Hsu SS, Chien CC, Lai PH. Teaching neuroImages: Idiopathic hypertrophic spinal pachymeningitis mimicking epidural lymphoma. Neurology 2015;84:e67-8.  Back to cited text no. 30
    
31.
Claus E, Rutgers M, Sindic C, Raftopoulos C, Godfraind C, Duprez T, et al. Remitting/relapsing idiopathic hypertrophic spinal pachymeningitidis: Comprehensive imaging work-up and MR monitoring. Eur Radiol 2005;15:53-8.  Back to cited text no. 31
    
32.
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. 32
    
33.
Jee TK, Lee SH, Kim ES, Eoh W. Idiopathic hypertrophic spinal pachymeningitis with an osteolytic lesion. J Korean Neurosurg Soc 2014;56:162-5.  Back to cited text no. 33
    
34.
Tosa M, Hara M, Morita A, Ninomiya S, Ebashi M, Kamei S, et al. Idiopathic hypertrophic spinal pachymeningitis. Intern Med 2015;54:1923-6.  Back to cited text no. 34
    
35.
Khadilkar SV, Menezes K, Parekh HN, Ursekar M, Bhagwati SN. Idiopathic hypertrophic cervical pachymeningitis: A case report with 5 year follow up. J Assoc Physicians India 2003;51:391-3.  Back to cited text no. 35
    
36.
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;28:590-2.  Back to cited text no. 36
    
37.
Mikawa Y, Watanabe R, Hino Y, Hirano K. Hypertrophic spinal pachymeningitis. Spine (Phila Pa 1976) 1994;19:620-5.  Back to cited text no. 37
    
38.
Dumont AS, Clark AW, Sevick RJ, Myles ST. Idiopathic hypertrophic pachymeningitis : A report of two patients and review of the literature. Can J Neurol Sci 2000;27:333-40.  Back to cited text no. 38
    
39.
Lowden MR. Section Editor Teaching Neuro Image: Idiopathic hypertrophic spinal pachymeningitis Teaching Neuro Image: Idiopathic hypertrophic spinal pachymeningitis. Neurology 2009;72:27-8.  Back to cited text no. 39
    
40.
Ranasinghe MG, Zalatimo O, Rizk E, Specht CS, Reiter GT, Harbaugh RE, et al. Idiopathic hypertrophic spinal pachymeningitis. J Neurosurg Spine 2011;15:195-201.  Back to cited text no. 40
    
41.
Tsutsui M, Yasuda T, Kanamori M, Hori T, Kimura T. Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis. Eur Spine J 2012;21 Suppl 4:S404-7.  Back to cited text no. 41
    
42.
Qin LX, Wang CY, Hu ZP, Zeng LW, Tan LM, Zhang HN, et al. Idiopathic hypertrophic spinal pachymeningitis: A case report and review of literature. Eur Spine J 2015;24 Suppl 4:S636-43.  Back to cited text no. 42
    
43.
Digman KE, Partinton CR, Graves VB. MR imaging of spinal pachymeningitis. J Comput Assist Tomogr 1990;14:988-90.  Back to cited text no. 43
    
44.
Park TJ, Seo WD, Kim SY, Cho JH, Kim DH, Kim KH, et al. Effective response of methotrexate for recurrent idiopathic hypertrophic spinal pachymeningitis. Korean J Spine 2016;13:200-3.  Back to cited text no. 44
    
45.
Voller B, Vass K, Wanschitz J. Hypertrophic chronic pachymeningitis as a localized immune process in the craniocervical region Hypertrophic chronic pachymeningitis as a localized immune process in the craniocervical region. Neurology 2001;56:107-9.  Back to cited text no. 45
    
46.
Sridhar K, Vasudevan MC. Idiopathic chronic hypertrophic pachymeningitis causing thoracic cord compression. Br J Neurosurg 2004;18:515-7.  Back to cited text no. 46
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

Top
Print this article  Email this article
   
Online since 20th March '04
Published by Wolters Kluwer - Medknow