IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension

Williams, Thomas E.; Marta, Mónica; Giovannoni, Gavin

doi:10.1136/practneurol-2015-001275

Cited by 10 publications

(3 citation statements)

References 18 publications

(15 reference statements)

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“…Hypertrophic pachymeningitis has been recognised as part of the IgG4-related disease spectrum [3], and can present with intracranial hypertension [4], as well as leptomeningitis, hypophysitis, pseudotumor and cranial neuropathy.…”

Section: Introductionmentioning

confidence: 99%

WITHDRAWN: IgG4-related Disease Presenting With Ataxia 

Tse

Hadjivassiliou

Devgire

et al. 2021

Preprint

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Background:Immunoglobulin G4 related disease is a relatively rare multi-system disorder which can present with diverse manifestations including mass-lesions and, or, organ dysfunction. Although the orbits, salivary glands and sinuses are comparatively common sites of involvement there are few reports of isolated intracranial presentation. Case presentation:Although large vessel IgG4-RD vasculitis is an uncommon entity we report a case of a 84-year old male presenting with cerebellar ataxia. CT at presentation demonstrated bilateral low-density change in the middle cerebellar peduncles, which corelated to areas of elevated T2 signal demonstrating restricted diffusion on MRI. CT angiogram demonstrated marked thickening of the common carotid arteries and branches extending to the skull base. In addition, the vertebral arteries also demonstrated significant wall thickening and occlusive narrowing. Serum immunoglobulin IgG and IgA were elevated and in particular IgG immunoglobulin was extremely high.Conclusions:Bilateral symmetrical restricted diffusion in the middle cerebellar peduncles is a striking radiological feature and reported to occur with multiple aetiologies, we believe this is the first report to associate this with IgG4-RD and ataxia.

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Section: Introductionmentioning

confidence: 99%

WITHDRAWN: IgG4-related Disease Presenting With Ataxia 

Tse

Hadjivassiliou

Devgire

et al. 2021

Preprint

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“…IgG4‐RHP seems to have a similar course to other IgG4‐RD patients. Most IgG4‐RHP respond to corticosteroids, but rapid recurrence of symptoms of HP is reported in different case series necessitating a corticosteroid‐sparing agent such as methotrexate, azathioprine, rituximab, and rarely mycophenolate mofetil 15–17 . As we considered meningeal disease was an urgent condition, all of our cases were treated with pulse corticosteroids, showing clinical improvement.…”

Section: Age At Onset/sex Symptoms Serum Igg4 At Diagnosis (Mg/dl) An...mentioning

confidence: 87%

“…13 A case-control study, suggested that CSF IgG4 quantification could be an alternative diagnostic tool with a cut off value of 2.27 mg/dL. [15][16][17] As we considered meningeal disease was an urgent condition, all of our cases were treated with pulse corticosteroids, showing clinical improvement. Four of them received rituximab, and 1 patient received azathioprine.…”

Section: Mri Dural Thickening Locationmentioning

confidence: 95%

IgG4‐related pachymeningitis—Long term follow up and outcome of six patients

et al. 2023

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ObjectiveOur understanding of IgG4‐RD and pachymeningitis has grown substantially, but the optimal approach for diagnosis, management, and long‐term outcomes is still an area of uncertainty.MethodsHUVAC is a database for IgG4‐RD patients, this database was retrospectively evaluated for pachymeningeal disease. Demographic, clinical, serological, imaging, histopathological data, and treatment details were re‐interpreted in patients with pachymeningitis.ResultsAmong 97 patients with IgG4‐RD, 6 (6.2%) had pachymeningitis. None of these patients had extracranial features, and also, in most of the patients, serum IgG4 levels were normal. Tentorium cerebelli and transverse sinus dura were the most commonly involved in the posterior fossa. During 18 months of median follow‐up on steroid+‐rituximab, none of them relapsed as pachymeningitis.ConclusionOur patients were mainly older males with sole neurological involvement. Non‐specific headache was the most common manifestation, and serum IgG4 levels were not useful for diagnosis. Typical radiology and tentorial thickening should suggest IgG4‐RD and prompt an early biopsy. Moreover, accompanying hypophysitis could also be a clue. With steroids+ rituximab treatment, no relapse related to meningeal involvement was seen in long‐term follow‐up.

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