IgG4-related disease causing facial nerve and optic nerve palsies: Case report and literature review

Wick, Cameron C.; Zachariah, Joseph; Manjila, Sunil; Brown, William C.; Malla, P; Katirji, Bashar; Cohen, Mark L.; Megerian, Cliff A.

doi:10.1016/j.amjoto.2016.08.005

Cited by 27 publications

(18 citation statements)

References 40 publications

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“…MTX was specifically assessed in a recent retrospective small study by Della-Torre et al and was reported to be effective in maintaining clinical and serological responses induced by glucocorticoids, enabling a substantial reduction in the overall dose of steroids in all patients and their withdrawal in six of ten patients within 6–12 months [161]. None of these patients had neurological disease secondary to IgG4-RD, and it should be noted that others have reported conflicting results of SSA use (predominantly MTX) in neurological disease caused by IgG4-RD [15, 63, 64, 69–71, 83, 118, 162, 163]. …”

Section: Treatmentmentioning

confidence: 99%

Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

106

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Opinion statementIgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.

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Section: Treatmentmentioning

confidence: 99%

Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

106

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“…4 To date, the rarity of this disease has limited characterization of skull base manifestations largely to case reports. [5][6][7][8][9][10] To this end, the first case of temporal bone IgG4-RD was not reported until 2012. 4 Motivated by both skull base IgG4-RD's considerable overlap with other cranial base pathology as well as overall disease rarity, the primary objective of this multi-institutional study was a description the cranial base manifestations of IgG4-RD with specific emphasis on disease aspects most pertinent to otolaryngologists and neurosurgeons who may be referred such patients for primary evaluation.…”

Section: Introductionmentioning

confidence: 99%

“…To date, the rarity of this disease has limited characterization of skull base manifestations largely to case reports . To this end, the first case of temporal bone IgG4‐RD was not reported until 2012 .…”

Section: Introductionmentioning

confidence: 99%

Manifestations of Skull Base IgG4‐Related Disease: A Multi‐Institutional Study

et al. 2019

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IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. Methods: Review of all cases at three tertiary-referral centers since disease description in 2003. Results: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy. Conclusions: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients.

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“…IgG4-RD cases are currently more frequently reported in Asian countries. [6,12,18,31,[34][35][36][37][38] In our case, the patient was a 54-yearold female with IgG4-RD invasion of the right cavernous sinus and orbit.…”

Section: Discussionmentioning

confidence: 77%

Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

Mayeku

Deisch

López-González

2021

Surgical Neurology International

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Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic disease of unknown etiology. It is characterized by tissue infiltration caused by IgG4 plasma cells and sclerosing inflammation of various body organs. At present, there are very few reported cases of IgG4-RD invasion of cavernous sinus and the orbit. Case Description: A 56-year-old female with a history of rheumatoid arthritis was presented with a gradual onset of right oculomotor, abducens, and trigeminal nerve deficits. Four weeks after the onset of symptoms, the patient developed gradual visual deficit. Following this, a trial of steroids was administered to the patient. However, the treatment did not work as expected and patient’s condition worsened. She progressed on to suffer complete visual loss in the right eye. Extensive work-up conducted on her turned out to be nondiagnostic. After this, the patient was referred to us for our evaluation. Neuroimaging revealed a right-sided cavernous sinus and orbital apex lesion. Given the lack of diagnosis and response to steroid treatment, we recommended surgical intervention and performed a modified pterional and pretemporal approach with extradural anterior clinoidectomy and transcavernous approach. We performed a lesion biopsy and cavernous sinus decompression, which helped in the partial recovery of visual function. The pathology report was consistent with IgG4-RD. Conclusion: IgG4-RD is a rare disease that occurs even less in combination with cavernous sinus and orbit invasion. The rarity of the disease and the diverse presentation of symptoms have sometimes caused delayed diagnosis and intervention. Patients who failed to respond to conservative management and patients in the fibrotic stage of the disease without other organ involvement may benefit from surgical intervention if amenable. Early suspicion, diagnosis, and intervention can facilitate better prognosis.

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IgG4-related disease causing facial nerve and optic nerve palsies: Case report and literature review

Cited by 27 publications

References 40 publications

Neurological Manifestations of IgG4-Related Disease

Neurological Manifestations of IgG4-Related Disease

Manifestations of Skull Base IgG4‐Related Disease: A Multi‐Institutional Study

Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

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