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 Table of Contents  
CASE REPORTS
Year : 2021  |  Volume : 4  |  Issue : 1  |  Page : 128-132

A seemingly usual case of paraplegia with MRI diagnosis of meningioma: An unexpected course of events leading to inflammatory necrotizing myelopathy


1 Department of Orthopaedics, MM Medical College and Hospital, Maharishi Markandeshwar University, Kumarhatti-Solan, Himachal Pradesh, India and, India
2 Department of Radiodiagnosis, All India Institute of Medical Sciences, Bathinda 151001, Punjab, India

Date of Submission16-Aug-2019
Date of Decision23-Oct-2019
Date of Acceptance09-Mar-2020
Date of Web Publication01-Oct-2020

Correspondence Address:
Harmeet Kaur
Department of Radiodiagnosis, All India Institute of Medical Sciences, Bathinda 151001, Punjab.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ISJ.ISJ_58_19

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  Abstract 

Necrotizing myelopathy is a rare but potentially devastating disease and an important cause of paralysis. Its diagnosis can be challenging inspite of availability of magnetic resonance imaging (MRI) and laboratory investigations. The differential diagnoses of this condition are tuberculosis, Foix–Alajouanine syndrome, transverse myelitis, and spinal tumors. The purpose of this case report was to document the pathological findings of this entity and highlight the misleading tendency of postoperative thick fibrous scar tissue, which may complicate the MRI picture of necrotizing myelopathy and it may masquerade as an intradural meningioma. The spinal cord biopsy is the only way to confirm the diagnosis in suspected cases based on the gradual subacute progression of clinical symptoms and MRI findings of rim-like rather than solid enhancement.

Keywords: Foix–Aiajouanine syndrome, myelitis, necrotizing myelopathy, paraplegia


How to cite this article:
Tiwari P, Kaur H, Sharma G. A seemingly usual case of paraplegia with MRI diagnosis of meningioma: An unexpected course of events leading to inflammatory necrotizing myelopathy. Indian Spine J 2021;4:128-32

How to cite this URL:
Tiwari P, Kaur H, Sharma G. A seemingly usual case of paraplegia with MRI diagnosis of meningioma: An unexpected course of events leading to inflammatory necrotizing myelopathy. Indian Spine J [serial online] 2021 [cited 2021 Feb 26];4:128-32. Available from: https://www.isjonline.com/text.asp?2021/4/1/128/308213




  Introduction Top


Spinal cord inflammation can present with symptoms similar to those of compressive myelopathies: bilateral weakness and sensory changes below the spinal cord level of injury, often accompanied by bowel and bladder impairment and sparing cranial nerve and cerebral function.[1]

Although the etiopathogenesis of various types of myelopathies vary widely, the clinical presentation can be similar, therefore differentiating between them can be challenging in spite of availability of magnetic resonance imaging (MRI) and laboratory investigations.

Intradural extramedullary spinal cord tumors account for approximately two-thirds of all spinal cord tumors in adults.[2] Meningiomas, neurofibromas, and schwannomas are the most common type of tumor in this type of location.[3],[4] Meningiomas represent approximately 40% of these tumors.[2] MRI is the imaging modality of choice as it often delineates the characteristic dural origin of meningiomas. They are typically isointense or hypointense to gray matter on T1-weighted (T1W), and isointense or hyperintense on T2-weighted (T2W) magnetic resonance imaging (MRI). Intraspinal meningiomas radiographically display avid homogenous enhancement with contrast.[5]

They commonly present with pain and progressive neurological dysfunction due to spinal cord compression from the expanding tumor mass. Fortunately, the meningiomas are typically benign, and thus surgical excision represents the possibility of a curative result.[6],[7]

In affluent countries of the world, it is nowadays extremely rare to see tuberculous paraplegia. However, the most common pathology responsible for paraplegia in developing countries still remains tuberculosis.[8]

Very rarely, a small tuberculoma of the spinal cord or diffuse extradural granuloma of the cord may be responsible for the neurological complications without any radiological evidence of tuberculous involvement of the vertebrae. Such cases present as spinal tumor syndrome.[8(pp239],[245)]

Subacute necrotizing myelopathy and progressive necrotic myelopathy are uncommon disease processes without any clear-cut diagnostic criteria and boundaries. Both of them are characterized clinically by progressive neurological deterioration and pathologically by necrosis in the spinal cord. As these lesions are rare, so even experienced radiologists may be misled by the findings. It may lead to complete change in the line of management and chances of recovery.


  Case History Top


A 37-year-old female presented in orthopedic outdoor clinic of our institute with paraplegia and urinary incontinence of 2-week duration. No obvious tenderness, swelling, and redness were noted on local examination of spine. Deep tendon reflexes were absent in both lower limbs, whereas Babinski was mute. Grade 0 power was present in both lower limbs and muscle atrophy was conspicuous. Opthalmologic examination was unremarkable. Any systemic illness was ruled out after thorough clinical examination and laboratory investigations.

MRI brain was also essentially normal. The patient had a past history of progressively increasing backache in the dorsal region and paraparesis of approximately 3-month duration, 6 months back for which she underwent laminectomy and decompression surgery at D5-D6 vertebral level at some other hospital, and 6 months before visiting our institute based on inconclusive MRI findings and a clinical suspicion of “atypical tuberculosis.” Though the dura was not opened in the first operation but biopsy was sent from laminae, ligamentum flavum, vertebral body, disc, and spinal canal, which came out to be negative for any infective or neoplastic pathology.

The patient was referred to the radiology department of our institute for further workup of worsened condition and progression to paraplegia gradually after index surgery. Plain roentgenogram of the dorsolumbar spine was unremarkable. MRI of dorso-lumbar spine showed an ill-defined intradural extramedullary hypointense lesion opposite D5 and D6 vertebrae resulting in significant spinal canal compromise and compression of the dorsal cord with its anterolateral displacement toward right side [Figure 1]. Patchy hyperintense areas were seen in compressed dorsal cord along with evidence of edema noted proximally and distally [Figure 2]. Postcontrast T1 weighted fat saturated images displayed heterogenous enhancement of the intradural lesion and of the dorsal cord in sagittal and axial images [Figure 2]A and ]. Enhancement was also seen along exiting nerve roots at D5-D6, D6-D7, and D7-D8 levels, more so on the left side [Figure 3]. No abnormal signal changes or any enhancement were seen in these vertebral bodies, intervening discs, or in the adjacent prevertebral or paraspinal soft tissues.
Figure 1: Short-Tau Inversion Recovery (STIR) sagittal image of the dorsal spine showing an ill-defined intradural extramedullary hypointense lesion opposite D5 and D6 vertebrae (red arrow) with resultant significant spinal canal compromise and compression of the dorsal cord. Edema was evident in dorsal cord proximal and distal to compression. No abnormal signal was noted in dorsal vertebrae, intervening discs and prevertebral soft tissues

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Figure 2: (A) T1 fat saturated (T1FS) sagittal image of the dorsal spine showing heterogenous enhancement of the intradural lesion. (B) T1FS sagittal image of the dorsal spine showing heterogenous enhancement in the neural foramina along the exiting nerve roots (yellow arrows)

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Figure 3: T1FS axial image of the dorsal spine revealed heterogenous enhancement of the intradural lesion and of dorsal cord which also shows anterolateral displacement to the right side due to mass effect of the lesion. Enhancement also extended to the bilateral neural foramina and paravertebral region on the right side. Laminectomy defect of the previous surgery was also evident in this image

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On the basis of the aforementioned MRI findings, provisional diagnosis of intradural extramedullary meningioma with resultant compressive myelopathy was considered. Second, but very less likely possibility of tuberculosis was also considered.

Excision of meningioma with decompression surgery of the cord was planned. Intraoperatively, a very thick layer of fibrous tissue was found, which when incised, a clear and transparent dura was seen without any epidural granulation tissue. No vascular malformation or thrombosed vessels were seen. When dura was opened to excise the meningioma, no clear plane of dissection could be identified, and necrotic, semiliquid abnormal tissue was seen in the region of spinal cord, which was sent for pathologic analysis. Intraoperative quick frozen section histopathological examination of the tissue was suggestive of granulomatous inflammation with ill-formed epithelioid cell granulomas and Langhans type giant cells. In light of these findings, a decision was made not to proceed with further surgery and management with anti tuberculosis treatment was contemplated at that stage.

However, the detailed and final pathology report was consistent with the diagnosis of inflammatory necrotizing myelopathy. Areas of necrosis, thick-walled vessels with acute and chronic inflammatory cell infiltrates were seen [Figure 4] A-C. This was in total contrast to frozen section findings.
Figure 4: (A) Thick walled blood vessels. (B) Plasma cell infiltrate in fibrocollagenous tissue. (C) Chronic inflammatory (Lymphocytic) infiltrate in glial tissue

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The pathological findings are overlapping between subacute necrotizing myelopathy and progressive necrotic myelopathy.[9]

The tissue supposed to be meningioma was actually fibrocollagenous tissue resulting from previous biopsy and decompressive laminectomy procedure.

CBNAAT (cartridge-based nucleic acid amplification test) report from tissue sample was negative too for tuberculosis.

It has been more than 1 year and there has been no clinical improvement till the writing of this report and further chances of recovery are minimal as the segment of cord at the site of lesion has necrosed completely.


  Discussion Top


Myelopathy is a broad term that refers to spinal cord involvement of multiple etiologies. Spinal cord diseases often have devastating consequences, ranging from quadriplegia and paraplegia to severe sensory deficits due to its confinement in a very small area.[10]

In a large study conducted by Cohen-Gadol et al.,[11] of 38 patients with questionable spinal lesions who underwent surgical biopsy, 53% of the lesions were consistent with inflammatory processes, whereas only 21% were neoplasms. In that study, the inflammatory processes included demyelinating diseases (21%), sarcoidosis (13%), chronic nonspecific inflammation (5%), eosinophilic vasculitis (3%), noncaseating granulomatous angitis (3%), nonspecific histiocytic reaction (3%), schistosomiasis (3%), and tuberculosis (3%).

The differential diagnoses in our case were tuberculosis, transverse myelitis, spinal tumors, and Foix–Alajouanine syndrome.

In this case, the very thick fibrous tissue resulting from the previous laminectomy and tissue biopsy operation appeared hypointense on T2W MR and showed intense enhancement on postcontrast T1FS images. It was mistaken as intradural meningioma, and the bright signal of the compressed cord was mistaken as edema due to compressive myelopathy, which was actually necrotic tissue.

The patient reported here does not meet the classic diagnostic radiological criteria for Foix–Alajouanine syndrome[12] as no arteriovenous malformation of the spinal cord was evident on contrast-enhanced MRI.

MRI shows the spinal cord lesion stability or atrophy over months or years and peripheral rim-like enhancement after contrast administration, together with the clinical findings of gradual progressive deterioration, should raise the diagnostic possibility of subacute necrotizing myelopathy (SNM).[13]

Mirich et al.[13] considered abnormal thick-walled vessels within spinal cord parenchyma to be pathognomic of SNM with hyalinization of vessel walls with almost occluded lumen on higher magnification.

In our case, though abnormal thick-walled vessels were seen in spinal cord parenchyma but they were not found to be hyalinized.

Katz and Ropper,[9] in their paper, had published findings of nine patients who had progressive idiopathic myelopathy with the evidence of spinal cord necrosis. In that study only one of nine patients had reactive lymphocytosis without a perivascular infiltrate, whereas six had marked gliosis without any inflammation.[9]

The case discussed in this report had marked lymphocytic inflammatory infiltrate.

Our purpose was to document the clinical and pathological findings in a case of necrotizing myelopathy, which do not meet the criteria of any classical nosologic entity.

The preoperative diagnosis of necrotizing myelitis is difficult to determine and perhaps impossible, and a biopsy is usually necessary to confirm the diagnosis.

This case report also highlights the notorious nature of postoperative fibrous tissue to further complicate the MRI picture of rare entities such as necrotizing myelitis, which may be misdiagnosed as a benign spinal tumor. The prognosis is stark opposite in them.

Acknowledgement

We acknowledge the help and support of Dr Anuj Sharma, Associate Professor, Department of Pathology, MM Medical College and Hospital, Maharishi Markandeshwar University, Kumarhatti-Solan, Himachal Pradesh, India (e-mail: [email protected]).

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Cree BA Acute inflammatory myelopathies. Handb Clin Neurol 2014;122:613-67.  Back to cited text no. 1
    
2.
Winn HR Youmans Neurological Surgery. 6th ed. Philadelphia, PA: Elsevier Health Sciences; 2011.  Back to cited text no. 2
    
3.
Bucci MN, McGillicuddy JE, Taren JA, Hoff JT Management of anteriorly located C1-C2 neurofibromata. Surg Neurol 1990;33:15-18.  Back to cited text no. 3
    
4.
Hu HP, Huang QL Signal intensity correlation of MRI with pathological findings in spinal neurinomas. Neuroradiology 1992;34:98-102.  Back to cited text no. 4
    
5.
Weber DC, Lovblad KO, Rogers L New pathology classification, imagery techniques and prospective trials for meningiomas: The future looks bright. Curr Opin Neurol 2010;23:563-70.  Back to cited text no. 5
    
6.
Aghayev K, Vrionis F, Chamberlain MC Adult intradural primary spinal cord tumors. J Natl Compr Canc Netw 2011;9:434-47.  Back to cited text no. 6
    
7.
Chamberlain MC, Tredway TL Adult primary intradural spinal cord tumors: A review. Curr Neurol Neurosci Rep 2011;11: 320-8.  Back to cited text no. 7
    
8.
Tuli SM (Chapter 22) Neurological complications. In: Tuberculosis of the Skeletal System. 4th ed. New Delhi, India: Jaypee Publishers; 2010. p. 239.  Back to cited text no. 8
    
9.
Katz JD, Ropper AH Progressive necrotic myelopathy: Clinical course in 9 patients. Arch Neurol 2000;57:355-61.  Back to cited text no. 9
    
10.
Granados Sánchez AM, García Posada LM, Ortega Toscano CA, Lopez AL Diagnostic approach to myelopathies. Rev Colomb Radiol 2011;22:1-21.  Back to cited text no. 10
    
11.
Cohen-Gadol AA, Zikel OM, Miller GM, Aksamit AJ, Scheithauer BW, Krauss WE Spinal cord biopsy: A review of 38 cases. Neurosurgery 2003;52:806-15; discussion 815-6.  Back to cited text no. 11
    
12.
Foix C, Alajouanine T La myelite necrotique subaique. Rev Neural (Paris) 1926;2:1-42.  Back to cited text no. 12
    
13.
Mirich RD, Kucharczyk W, Keller MA, Deck , John . Subacute necrotizing myelopathy: MR imaging in four pathologically proved cases. American J Neuroradiol 1991;12:1077-83.  Back to cited text no. 13
    


    Figures

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



 

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