Neurologic IgG4-Related Disease

Topiwala, Karan; Hampton, Christopher; Boland, Carl; Waitzman, David M.

doi:10.1177/1941874418801088

Cited by 4 publications

(2 citation statements)

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

Section: Discussionmentioning

confidence: 87%

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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Section: Discussionmentioning

confidence: 87%

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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“…IgG4-RD involves multiple organ systems, and has a tendency to develop multiple neoplastic lesions. The clinical presentations are nonspecific, but these primarily consist of dysfunction of the involved organs or tissues ( 45 , 46 ). Patients with IgG4-RD may also present with fever, fatigue, night sweats, and weight loss ( 47 , 48 ).…”

Section: Discussionmentioning

confidence: 99%

Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature

et al. 2023

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PurposeHypertrophic pachymeningitis associated with immunoglobulin G4-related disease (IgG4-RD) has been rarely reported, and there is little information and no clear consensus on the management of IgG4-related spinal pachymeningitis (IgG4-RSP). The present study described its possible clinical features, including the symptoms, imaging, treatment and prognosis of patients with IgG4-RSP.MethodsWe report three patients who presented with progressive neurological dysfunction due to spinal cord compression. Relevant articles were searched from the PubMed, Web of Science, and Embase databases, and the resulting literature was reviewed.ResultsThe literature review provided a summary of 45 available cases, which included three cases from our center. Progressive worsening of neurological impairment was observed in 22 patients (48.9%). The lesions involved the thoracic spine (n=28, 62.2%), cervical spine (n=26, 57.8%), lumbar spine (n=9, 20.0%), and sacral spine (n=1, 2.2%). Furthermore, the lesions were located in the dura mater (n=18, 40.0%), epidural space (n=17, 37.8%), intradural-extramedullary space (n=9, 20.0%), and intramedullary space (n=1, 2.2%). On magnetic resonance imaging (MRI), the lesions generally appeared as striated, fusiform, or less often lobulated oval changes, with homogeneous (n=17,44.7%) and dorsal (n=15,39.5%) patterns being the most common. Thirty-five patients had homogeneous T1 gadolinium enhancement. Early surgical decompression, corticosteroid treatment, and steroid-sparing agents offered significant therapeutic advantages. A good therapeutic response to disease recurrence was observed with the medication.ConclusionThe number of reported cases of IgG4-RSP remains limited, and patients often have progressive worsening of their neurological symptoms. The features of masses identified on the MRI should be considered. The prognosis was better with decompression surgery combined with immunosuppressive therapy. Long-term corticosteroid treatment and steroid-sparing agent maintenance therapy should be ensured. A systemic examination is recommended to identify the presence of other pathologies.

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IgG4-Related Disease: A Retrospective Chinese Study of Features and Treatment Response of 98 Patients Including 4 Rare Cases

et al. 2021

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Neurologic IgG4-Related Disease

Cited by 4 publications

References 4 publications

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

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

Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature

IgG4-Related Disease: A Retrospective Chinese Study of Features and Treatment Response of 98 Patients Including 4 Rare Cases

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