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            : Multifocal neurologic involvement as the only manifestation of IgG4-related disease

Imbergamo, Silvia; Campagnolo, Marta; Manara, Renzo; Marino, Filippo; Adami, Fausto; Briani, Chiara

doi:10.1212/wnl.0b013e31827f08ae

Cited by 5 publications

(3 citation statements)

References 5 publications

(3 reference statements)

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“…As illustrated by our case, neurologic involvement may be predominantly or exclusively subclinical. Multifocal neurologic involvement and involvement of the spinal dura with resulting cord compression have also been described . As noted in our case, enlargement of branches of the trigeminal nerve is highly suggestive of IgG4‐RD .…”

supporting

confidence: 77%

IgG4‐related (neurologic) disease: diagnostic challenges, clinical clues and expanding spectrum

et al. 2014

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supporting

confidence: 77%

IgG4‐related (neurologic) disease: diagnostic challenges, clinical clues and expanding spectrum

et al. 2014

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“…Predictors of disease relapse were high baseline values of IgG4, IgE and the total eosinophil count. Additional reports have emerged of RTX being effective in a number of cases of IgG4-RD affecting the CNS, generally pachymeningitis [15, 61, 66, 70, 112, 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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“…Rituximab has shown promising results treating some forms of IgG4‐RD . A few cases have been described as refractory to this drug . In this case, rituximab was chosen because of the presence of systemic disease.…”

Section: Discussionmentioning

confidence: 99%

Alteration of IgG4 levels in cerebrospinal fluid in IgG4‐related disease

et al. 2015

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Teaching Neuro Images : Multifocal neurologic involvement as the only manifestation of IgG4-related disease

Abstract: Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

Cited by 5 publications

References 5 publications

IgG4‐related (neurologic) disease: diagnostic challenges, clinical clues and expanding spectrum

IgG4‐related (neurologic) disease: diagnostic challenges, clinical clues and expanding spectrum

Neurological Manifestations of IgG4-Related Disease

Alteration of IgG4 levels in cerebrospinal fluid in IgG4‐related disease

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