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Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1176-1183

IgG4-Related Disease in Intradural Extramedullary Location- Detailed Case Illustration and Literature Review with Special Emphasis on Role of Surgery in its Management

1 Department of Neurosurgery, AIIMS, New Delhi, India
2 Department of Pathology, AIIMS, New Delhi, India
3 Department of Rheumatology, AIIMS, New Delhi, India

Date of Submission22-Aug-2020
Date of Decision15-Sep-2020
Date of Acceptance23-Sep-2020
Date of Web Publication30-Oct-2021

Correspondence Address:
Rajeev Sharma
Department of Neurosurgery, AIIMS, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.329549

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 » Abstract 

Background: IgG4-related disease (IgG4RD) is a multisystemic progressive fibroinflammatory and lymphoproliferative autoimmune disorder of unknown etiopathogenesis; and its occurrence in intradural extramedullary (IDEM) location is extremely rare.
Aim and Objective: The aim of this study was to review all IDEM IgG4RD cases described in English literature and to signify the role of surgery in its management at this rare location along with the surgical technique and intraoperative findings.
Methods: An Internet-based search (PubMed) for the published reports describing spinal cases of IgG4RD with IDEM involvement was done using the keyword: spinal IgG4-related disease. An illustration of a case of cranial IgG4-related disease in a young man who developed metachronous cervical involvement in intradural extramedullary (IDEM) location is also presented.
Results: Amongst 45 spinal IgG4RD cases reported in English Literature, only 3 cases were IDEM IgG4RD. In the illustrated case, portion of the cervical lesion causing cervical cord encasement was intradural but extraarachnoidal- located between arachnoid and dura, without any pial involvement. All these 4 cases improved following surgery.
Conclusion: Timely meticulous resection of mass lesion in situations of medical treatment failure or progressive neurological decline can lead to reversibility of mass effect-associated neurological manifestations of IgG4RD.

Keywords: Hypertrophic pachymeningitis, idiopathic pachymeningitis, IgG4-related disease, intradural, intradural extramedullary, intraspinal, spinal cord encasement
Key Message: Adequate decompression sufficient enough to relieve neural compression is acceptable, residue being amenable to medical management.

How to cite this article:
Sharma R, Kaur K, Sasidharan A, Gupta R, Laythalling RK. IgG4-Related Disease in Intradural Extramedullary Location- Detailed Case Illustration and Literature Review with Special Emphasis on Role of Surgery in its Management. Neurol India 2021;69:1176-83

How to cite this URL:
Sharma R, Kaur K, Sasidharan A, Gupta R, Laythalling RK. IgG4-Related Disease in Intradural Extramedullary Location- Detailed Case Illustration and Literature Review with Special Emphasis on Role of Surgery in its Management. Neurol India [serial online] 2021 [cited 2021 Dec 3];69:1176-83. Available from:

IgG4-related disease (IgG4RD) is a multisystemic progressive fibroinflammatory and lymphoproliferative autoimmune disorder of unknown etiopathogenesis characterized by tumefactive lesions in multiple sites.[1],[2],[3] Central nervous system involvement usually presents as hypertrophic pachymeningitis with cranial involvement more commonly than spine.[4],[5] Cranial or spinal involvement may be accompanied an active disease elsewhere in the body. Spinal involvement is commonly seen in epidural location and intradural extramedullary (IDEM) involvement is extremely rare. Excision of IDEM lesion requires more meticulous surgical techniques due to close proximity to neural structures. A detailed disease course in a young man, a known case of cranial IgG4-related disease who developed metachronous cervical involvement in the rare IDEM location is presented along with the details of the surgical technique employed for relieving pressure on the cord due to encasement.

 » Case Illustration Top

A 27- years-old gentleman developed rapidly progressive painless vision decline in left eye three years back. His clinical examination at another hospital was normal except for the presence of perception of hand movements close to face in left eye. There was no significant family history or atopy. Contrast enhanced magnetic resonance imaging (CEMRI) of brain and orbits [Figure 1] showed a sellar- suprasellar lesion sized 14 × 8 × 11 mm involving foraminal and intradural portion of left optic nerve and pituitary gland encasing left internal carotid artery with anterior cranial fossa base and clival dural enhancement. Hormonal profile was normal, and the patient had no diabetes insipidus. At his previous medical institution, provisional diagnosis of en-plaque meningioma was made; and thus was operated there by left pterional craniotomy and subtotal excision of the left optic nerve lesion. Postoperatively, he developed complete left eye blindness which failed to improve till date. As immunohistopathological analysis of biopsy was suggestive of IgG4-related disease (IgG4 positive plasma cells >10/high-power field); he was started on oral steroids, but the patient stopped treatment and was lost to follow-up. Seventeen months after cranial surgery, the patient presented to the same hospital with new complaints of insidious onset gradually progressive numbness of the whole body below the neck and slight weakness in both upper limbs without any pain. CEMRI cervical spine with whole spine screening [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d,[Figure 2]e showed a C2-4 dural-based multilobulated circumferential lesion encasing the cord with thinning of cervical cord with other enhancing foci: linear dural thickening along clivus and C6-D1 without any mass effect on adjacent neural tissue (cord or nerve roots). As imaging was atypical of meningioma or schwannoma, and in view of past history of IgG4-related cranial disease; a possibility of IgG4-related cervical spinal intradural disease was considered and oral steroid therapy was started. As there was no resolution of symptoms despite taking steroids regularly for five months, patient came to our hospital. Clinically, his visual acuity was 6/36 in right eye and no perception of light in left eye; and there was spastic quadriparesis of muscle strength 4/5 in all four limbs. CEMRI cervical spine with whole spine screening showed slight increase in size of the C2-4 lesion. Routine hematological and biochemical parameters including serum IgG and IgG4 levels were within normal limits. After taking Rheumatology and Hematology consultation, diagnosis of IgG4-related cervical spinal intradural disease was confirmed and patient was advised to continue oral steroid (Prednisolone 60 mg per day- gradually tapered later to 10 mg per day) along with additional Azathioprine (25 mg slowly increased to 100 mg per day) therapy for 6 months. As there was no clinicoradiological [Figure 2]f,[Figure 2]f,[Figure 2]g,[Figure 2]h,[Figure 2]i,[Figure 2]j,[Figure 2]k,[Figure 2]l,[Figure 2]m,[Figure 2]n improvement with medical management for over 6 months, the patient was planned for surgical management. He underwent C2-4 laminotomy, midline durotomy, near-total piecemeal excision of the dural-based multilobulated circumferential IDEM lesion under neuromonitoring [Figure 3]. Lesion was adhered to the inner surface of dorsal dura; plane between the inner surface of the dorsal dura and the intradural lesion was created by blunt dissection. Multiple anchoring dural stitches were taken on either side using 4-0 silk sutures for wider intradural exposure. No neural structure was visible after durotomy as the pinkish-yellow lesion was seen covering whole of the dorsal surface of the cord and roots. As the lesion-dural plane was being created laterally, we could identify the plane between the dorsal component of the lesion and the dorsal nerve roots at foramina level which was followed proximally to reach the dorsal surface of the spinal cord. There was a well defined plane between dorsal surface of the cord and the dorsal component of the lesion, there were no adhesions between the cord piamater and the lesion, and the cord with its roots was free and normal. Lesion was pinkish-yellow, firm, very minimally vascular, and not-suckable with cavitron ultrasonic aspirator even at highest possible settings. Piecemeal excision of the dorsal portion of the lesion was done, thus exposing the dorsal surface of the cord and dorsal nerve roots arising from the cord going into the neural foramina. Cervical roots at foramina were encased by the lesion- dissected free. Ventral component of the lesion was pushing the cord posteriorly, but cord was lax. Access to the ventral component of the lesion was improved by cutting ligamenta denticulata and mobilizing the cord to the contralateral side; lesion was then cut with scissors and removed piecemeal. Gross total excision of the macroscopic tumor portion was done, leaving behind the thickened arachnoid portion at some sites ventrolateral and ventral to cord. There were no adhesions between the ventral portion of the lesion and the truly ventral dura; the truly ventral cervical dura exposed after lesion excision looked apparently normal. Cord and nerve roots were lax and fell down into the thecal sac after lesion excision. There were transient drops in right upper limb MEPs, which recovered slowly and spontaneously over the next one hour. During this one hour, no dissection was done on right side- only dissection was done on left side. Lesion excision was completed on right side after complete recovery of MEPs. Primary midline dural closure and C2-4 Laminoplasty using plates and screws was done. Postoperative recovery was uneventful and his symptoms gradually improved over next few days. Postoperative imaging [Figure 4] at 2 months follow-up showed good decompression of cervical cord and stable residual cranial disease. Rituximab was added to maintenance steroid therapy after confirming immunohistopathological diagnosis [Figure 5]. Immunohistopathological findings of the spinal lesion were similar to that of the cranial lesion [Figure 5]. He was asymptomatic at 11 months follow-up with continued medical (steroids) management.
Figure 1: (a-f) CEMRI Brain and orbits showed a T1 isointense, T2 hypointense brilliantly enhancing lobulated lesion involving left optic nerve of size 14 × 8 × 11 mm widening left optic foramen, extending into planum, suprasellar and sellar region with encasement of left internal carotid artery, presence of dural tail with anterior cranial fossa base and clival dural enhancement. Pituitary gland could not be identified separately from the lesion, but pituitary stalk was enhancing in midline. Anterior cranial fossa base and clival dura showed enhancement

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Figure 2: (a-e) Initial CEMRI Cervical spine with whole spine screening showed a T1 isointense, T2 iso- to hypo-intense C2-4 dural based multilobulated circumferential lesion encasing the cord with thinning of cervical cord, and enhancing linear dural thickening along clivus upto C2 and C6-D1 without any mass effect on adjacent neural tissue- cord or nerve roots. (f-n) Serial CEMRI after 5 months of steroid and azathioprine therapy (just prior to cervical surgery) showed slightly increase in size of C2-4 intradural lesion encasing the cord

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Figure 3: Intraoperative photographs showing (a) absent plane [black arrow] after durotomy, (b) dorsal lesion component covering cord, black arrow showing D-wave electrode, (c) after incising lesion's dorsal component [black arrow] on left side to expose the underlying cord, (d) arachnoid [black arrow] identified, (e) ligamentum denticulatum cut- black arrows pointing to lesion, (f) dissecting root encased by lesion [black arrows], (g) lesion's ventral component [black arrow], (h) left side and ventrally, (i) right side and ventrally, and (j) lax cord and nerve roots at closure

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Figure 4: (a-j) Postoperative CEMRI brain, cervical spine with whole spine screening at 2 months follow-up showed good decompression of cervical cord with stable residual cranial and spinal disease

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Figure 5: (a-d) Histopathological examination of both cranial (optic nerve sheath) and spinal excisions revealed (a) storiform fibrosis, with (b-d) dense infiltration by plasma cells and lymphocytes in clusters and infiltrating the vessel walls with prominence of endothelial cells; and (e-f) Immunohistochemistry for (e) IgG and (f) IgG4 revealed ~60% of plasma cells to be IgG4 immunopositive

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 » Materials and Methods Top

Spinal IgG4RD is most commonly described in epidural location in the spine, with intradural extramedullary involvement being extremely rare. As only a few single patient- case reports of intradural extramedullary involvement are available in the literature, we planned to review all these spinal cases described in English Literature. An Internet based search (PubMed) for the published reports describing spinal cases of IgG4RD with intradural extramedullary involvement was done using keyword: spinal IgG4-related disease. We included all such immunohistochemically proven intradural extramedullary IgG4RD cases described in English Literature, and cases lacking immunohistochemical confirmation were excluded. From each published case report, we excerpted the following information- number of cases described, age, sex, presentation, duration of symptoms, comorbidities, history of atopy, vertebral level, intradural location of the lesion with respect to the spinal cord, neuroimaging features, biochemical and serological parameters, disease location elsewhere in body besides spinal involvement, initial medical management and its response, surgical procedure performed, extent of resection, intraoperative lesion characteristics, histopathological and immunohistochemical features, maintenance treatment, follow-up, and final outcome. If certain information was not available in the research paper, we recorded that parameter as NR: “not reported”; and the case in question was not included in the denominator for that particular parameter during the analysis.

 » Results Top

Out of the initial search of 45 articles using keyword “spinal IgG4-related disease”, we finally selected three articles fulfilling our inclusion and exclusion criteria. All these three articles were published as case reports. The information was recorded in a tabular form [Table 1]. The present case was also included in the statistical analysis. Analysis of four patients (including the present case) showed four lesions in the age range of 27-68 years with 75% patients being >50 years of age. Three lesions were seen in males, and one lesion in female patient (M: F = 3:1). Presentation was acute in one, chronic in two, and NR in one patient. Most of them presented with paraparesis as the most prominent symptom for a duration ranging from two days to six months. None of them had atopy. Lesion was hypointense on T2 MRI sequence in three out of four cases. Only cervical and upper dorsal spine involvement was noted in these four cases. Lesion was ventral to the cord in two cases, dorsal to the cord in one case, and was circumferentially encasing the cord in the presently illustrated case. Medical management was tried initially in two cases (including the present case) unsuccessfully. Significant neural compression causing neurodeficits was the indication of surgery in all the four cases. Nerve root was cut to reach the ventral lesion in both the patients with ventral lesions. However, nerve roots were not cut to remove the ventral portion of the lesion in the present case. Total/near-total resection could be achieved in only two out of four lesions. Postoperative medical management with steroids was continued in three of them and was avoided in the fourth patient due to diabetes. All four of them showed neurological improvement after surgery despite subtotal removal in two patients.
Table 1: Comparison of various reported spinal IgG4RD cases in intradural extramedullary location

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 » Discussion Top

IgG4-related pachymeningitis can present either as a linear dural thickening with nonspecific neuro-symptomatology (memory impairment, cognitive decline, seizures etc) or as a bulging pseudotumor mass compressing adjacent neural structures like nerves, brain, or spinal cord.[6],[7] Mass effect of the lesion over the cranial nerves rather than the cranial nerve invasion is the cause of cranial neuropathies.[8] Constitutional symptoms and submandibular/lacrimal enlargement may or may not be present. Hypointense lesion on T2-weighted MRI sequence (neoplastic pathology less likely), restricted diffusion on DWI, absence of hyperostosis, and brilliant contrast enhancement with dural tail points towards the possibility of IgG4-RD.[8] Diagnosis is confirmed by histopathological and immunohistochemical (IgG and IgG4 immunostaining) analysis of biopsy specimens simultaneously excluding the other differential diagnoses.[4],[9] However, obtaining biopsy specimen may not always be feasible (like cavernous sinus disease), thus necessitating empirical treatment.[10] Diagnosis of IgG4RD can be classified as definite, probable, or possible based on serum IgG4 levels (>135 mg/dl), presence of classic histological findings (dense lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis), immunostaining findings (absolute IgG4+ plasma cell count > 10/HPF, IgG4+/IgG+ plasma cell ratio >40%), and presence of pseudotumors in one or more organs.[3],[11] Whole-body clinico-radiological assessment using 18F-fluorodeoxyglucose positron emission tomography/CT identifies areas of systemic involvement.[8]

Medical treatment (steroids, steroid-sparing immunomodulators, Rituximab, etc) with steroids is the treatment of choice of this highly treatable disease in the neurologically intact patients, but can be tried as initial therapy even in patients with neurological deficits. It is preferred to obtain tissue diagnosis before starting medical treatment.[12] Besides providing tissue sample for immunohistopathological analysis, surgical decompression of the lesion causing neural compression is indicated in cases not responding to medical treatment or in cases with progressive neurological decline despite adequate medical management.[4] For diagnostic purposes; fine needle aspiration technique is not recommended, but is useful in excluding malignancy.[12] Open surgical biopsy from multiple sites is preferred over needle biopsy as the former provides more amount of tissue sample and open biopsy sample shows more numbers of IgG4 + plasma cells compared to needle biopsy.[8],[13] Also, needle biopsy sample may not show storiform fibrosis and if taken from the periphery of a malignant lesion (peritumoral tissue being rich in IgG4 + plasma cells) may be misdiagnosed as IgG4RD.[13] Longstanding “burnt out” disease having major fibrotic component is usually corticosteroid-resistant, thus indicating need of excision of the tumefactive mass causing neural compression.[12] Though total excision is not mandatory, it can be achieved in isolated pseudotumors (not involving critical neurovascular structures) causing mass effect-related neurodeficits thereby facilitating long-term remission.[8] However, dense fibrous change in a long-standing lesion may limit the extent of decompression, adversely affecting neurological recovery.[8] Prolonged medical therapy is continued after surgical decompression to reduce residual disease burden, thereby reducing recurrence. Relapses are common especially after tapering or stopping steroids.[14] IgG4-RD responder index can be used to define response to treatment, remission, and relapse during follow-up.[6],[12]

Majority of the spinal case reports documented in the Literature involve the epidural location. As it is a pachymeningeal disease, we extrapolate that focal dural thickening bulging on the outer surface of spinal dura presents as epidural disease, and that bulging on the inner surface of spinal dura presents as dural-based IDEM disease. Also, we postulate possibility of occurrence of combined epidural and IDEM disease which is yet to be reported. Only three cases of IDEM Spinal IgG4RD have been described in Literature [Table 1].[2],[5],[15] We hereby report this fourth case of IDEM Spinal IgG4RD. Also, this is the first reported case of IgG4RD in IDEM location presenting as cervical cord encasement, not responded to steroids and azathioprine, and was excised nearly completely leading to good neurological recovery. Pseudotumor location ventral to the spinal cord was noted in three out of these four cases as the ventral component of the lesion was much thicker than the dorsal component in our case. Three out of these four cases were males, and only one out of them was less than 30 years of age. All four of them presented with neurological deficits. None of them improved with initial medical management, but three out of these four cases showed good neurological improvement after subtotal to near total resection. Minimal improvement despite total resection by Kim et al.[15] may be due to D4 watershed area.

Intradural extramedullary location is important because of the mass effect-associated neurological deficits and special surgical techniques required in excising the ventral lesion. Bridges et al.[5] and Kim et al.[15] have reported one case each of ventrally placed intradural extramedullary lesion. To gain circumferential access, Bridges et al.[5] cut bilateral D3 and D4 nerve roots along with the ligamenta denticulata. We gained circumferential access by wide vertical midline durotomy and bilateral ligamenta denticulectomy without cutting any cervical root. Like Bridge et al.[5] case, lesion was firm and not suckable with ultrasonic aspirator even at its highest settings in our case. However, in contrast to Bridge et al.[5] case where only debulking was achieved, we could achieve near total excision of the lesion causing spinal cord encasement and compression. Patient is following up with rheumatology department and is currently on corticosteroids and Rituximab. Bridges et al.[5] in their literature review of all reported Spinal IgG4RD cases (n = 16), found that extent of resection was subtotal resection or biopsy in majority (>90%) of cases; complete resection was done only in one case reported by Kim et al.[15] So, decompression sufficient enough to relieve neural compression is needed, the residue being amenable to medical therapy. Kim et al.[15] have reported a case of ventrally located C7-D5 IDEM IgG4RD in a 52-year-old lady presenting with rapidly progressive acute paraplegia which was managed by intravenous steroid pulse therapy followed by C7-D4 laminotomy and total excision of the lesion after sacrificing thoracic rootlets. However, there was minimal improvement at two months follow-up despite undertaking rehabilitation program. Levraut et al.[2] have reported a case of dorsally located D2-D3 IDEM IgG4RD presenting as paraparesis in 58-year-old diabetic man. Lesion was originating from dorsal duramater and was densely adherent to piamater of spinal cord- subtotal excision was achieved. Without initial steroid treatment (due to diabetes), lesion was excised leading to near complete recovery. There was no residue on MRI at three months follow-up. In our case, the multilobulated lesion encasing the cervical cord was dural-based; densely adhered to the inner surface of dorsal, bilateral and anterolateral dura without any adhesion to the truly ventral dura. Lesion was intradural but extra-arachnoidal; located between arachnoid and dura, without any pial involvement. Tumor thickness of the lesion component dorsal to cord was less compared to the lesion component ventral to the cord (thicker and firmer). The various steps which helped us in achieving adequate decompression without neurological decline are: 1) widest possible laminotomy and durotomy for wide exposure, 2) identifying and maintaining correct dissection plane by meticulous dissection, 3) piecemeal decompression preserving all nerve roots, 4) cutting of ligaments denticulata bilaterally for cord mobilization without traction, and 5) withholding decompression and waiting for evoked potentials to recover following drop and then completing decompression. We also reviewed literature for synchronous and metachronous multisite neural involvement. Ishihara et al.[16] have reported a case of metachronous involvement of left parietal lobe and intramedullary cervical spinal cord by IgG negative inflammatory pseudotumor. Like in our case, both the cranial and spinal lesions were managed by surgical resection in their case. Lin et al.[17] have reported a case of synchronous involvement of brain and spine in a known case of rheumatoid arthritis, diagnosed by axillary lymph node and brain needle biopsies, and managed successfully by medical therapy. Though we have reported it as metachronous involvement, but smaller synchronous (initially asymptomatic) involvement which progressed with time and became symptomatic cannot be ruled out in our case as spine imaging was not done at the time of cranial surgery (operated at another center two years prior) and patient was not on regular steroid therapy after first surgery at another center.

While reviewing literature for circumferential encasement of spinal cord (irrespective of pathology); we could only found Javed et al.[18] paper describing circumferential epidural encasement of the spinal cord at cervicothoracic junction in a 83-year-old lady with rheumatoid arthritis who presented with spastic paraparesis. Compartment syndrome of spinal cord due to encasement was successfully relieved by C6-D3 laminectomy and complete posterior epidural decompression of spinal cord in their case.[18]

 » Conclusion Top

Timely meticulous resection of mass lesion in situations of medical treatment failure or progressive neurological decline can lead to reversibility of mass effect-associated neurological manifestations of IgG4RD. Post-resection prolonged medical therapy helps in reducing recurrence.

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 » References Top

Radotra BD, Aggarwal A, Kapoor A, Singla N, Chatterjee D. An orphan disease: IgG4-related spinal pachymeningitis: Report of 2 cases. J Neurosurg Spine 2016;25:790-4.  Back to cited text no. 1
Levraut M, Cohen M, Bresch S, Giordana C, Burel-Vandenbos F, Mondot L, et al. Immunoglobulin G4-related hypertrophic pachymeningitis: A case-oriented review. Neurol Neuroimmunol Neuroinflamm 2019;6:e568.  Back to cited text no. 2
Ryu G, Cho HJ, Lee KE, Lee JJ, Hong SD, Kim HY, et al. Clinical significance of IgG4 in sinonasal and skull base inflammatory pseudotumor. Eur Arch Otorhinolaryngol 2019;276:2465-73.  Back to cited text no. 3
Winkel M, Lawton CD, Sanusi OR, Horbinski CM, Dahdaleh NS, Smith ZA. Neuro-surgical considerations for treating IgG4-related disease with rare spinal epidural compression. Surg Neurol Int 2018;9:209.  Back to cited text no. 4
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Bridges KJ, DeDeaux CH, Than KD. IgG4-related disease presenting as intradural extramedullary lesion: A case report and review of literature. Br J Neurosurg 2019;33:570-6.  Back to cited text no. 5
Vasaitis L. IgG4-related disease: A relatively new concept for clinicians. Eur J Intern Med 2016;27:1-9.  Back to cited text no. 6
Ferreira NR, Vaz R, Carmona S, Mateus S, Pereira P, Fernandes L, et al. IgG4-related disease presenting with an epidural inflammatory pseudotumor: A case report. J Med Case Rep 2016;10:61.  Back to cited text no. 7
Marinelli JP, Marvisi C, Vaglio A, Peters PA, Dowling EM, Palumbo AA, et al. Manifestations of skull base IgG4-related disease: A multi-institutional study. Laryngoscope 2019. doi: 10.1002/lary. 28478. [Epub ahead of print].  Back to cited text no. 8
Alrashdi MN. Immunoglobulin G4-related spinal pachymeningitis. Saudi Med J 2020;41:652-6.  Back to cited text no. 9
Mageau A, Shor N, Fisselier M, Aboab J, Lecler A, Rebours C, et al. Rituximab for corticosteroid-resistant relapsing IgG4-related intracranial pachymeningitis: Report of two cases. Pract Neurol 2018;18:159-61.  Back to cited text no. 10
Obiorah IE, Henao Velasquez A, Özdemirli M. The Clinicopathologic spectrum of IgG4-related disease. Balkan Med J 2018;35:292-300.  Back to cited text no. 11
Slade SJ, Bauer EM, Stone VV, Dave AJ. Spinal IgG4-related hypertrophic pachymeningitis with spinal cord compression: Case report and literature review. World Neurosurg 2019;130:65-70.  Back to cited text no. 12
Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181-92.  Back to cited text no. 13
Chwalisz BK, Stone JH. Neuro-ophthalmic complications of IgG4-related disease. Curr Opin Ophthalmol 2018;29:485-94.  Back to cited text no. 14
Kim SH, Kang Y, Oh SH, Paik S, Kim JS. Paraplegia in a patient with IgG4-related sclerosing disease: A case report. Ann Rehabil Med 2014;38:856-60.  Back to cited text no. 15
Ishihara M, Izumoto S, Iwatsuki K, Yoshimine T. Immunohistochemical study of multiple inflammatory pseudotumors with both brain and spinal cord involvement: Case report. Neurol Med Chir (Tokyo) 2010;50:246-50.  Back to cited text no. 16
Lin J, Zheng L, Zhou D, Hong Z. Immunoglobulin G4-related disease involving both cerebral parenchyma and spinal cord: A case report. J Neuroimmunol 2019;335:577018. doi: 10.1016/j.jneuroim. 2019.577018.  Back to cited text no. 17
Javed S, Selvaratnam V, Babori A, Wigfield C. Circumferential encasement of the spinal cord in a patient with rheumatoid arthritis. Br J Neurosurg 2011;25:308-9.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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