Anti‐IgLON5 Disease Responsive to Immunotherapy: A Case Report with an Abnormal MRI

Moreno‐Estébanez, Ana; García-Ormaechea, Milagros; Tijero, Beatriz; Fernández-Valle, Tamara; Gómez‐Esteban, Juan Carlos; Berganzo, Koldo

doi:10.1002/mdc3.12679

Cited by 18 publications

(13 citation statements)

References 11 publications

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“…In line with this, previously reported therapy effects were less obvious and some authors doubted if an immunosuppressive treatment would be effective in this disease [1,2]. However, subacute disease progression has been reported 1 3 in a few other cases, probably representing a favorable timepoint for immunotherapy initiation [5][6][7]. Interestingly, up to 20% of patients have a relative rapid clinical presentation, in less than 4 months.…”

Section: Dear Sirsmentioning

confidence: 71%

Early immunotherapy is highly effective in IgG1/IgG4 positive IgLON5 disease

et al. 2020

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Section: Dear Sirsmentioning

confidence: 71%

Early immunotherapy is highly effective in IgG1/IgG4 positive IgLON5 disease

et al. 2020

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“…It is an enigmatic condition lying between autoimmune encephalitis and neurodegenerative diseases. On the one hand, patients have IgG4 Ab against the neuronal cell adhesion molecule IgLON5, usually in both serum and CSF, and some positive responses to immunotherapy have been reported [71][72][73][74][75]. These findings are in line with an immune-mediated etiology.…”

Section: Encephalitis With Anti-iglon5 Autoantibodiesmentioning

confidence: 95%

“…A strong class II HLA association, as in other IgG4mediated diseases, also suggests an autoimmune pathogenesis. Patients carrying DRB1*10:01 have been reported in several case reports and small series, along with 13/15 ( [70,71,[73][74][75][76][77][78]. HLA DQA1*01-DQB1*05 appears in more than 90% of the patients, not always linked to DRB1*10:01.…”

Section: Encephalitis With Anti-iglon5 Autoantibodiesmentioning

confidence: 99%

Associations between HLA and autoimmune neurological diseases with autoantibodies

Muñiz‐Castrillo

Vogrig

Honnorat

2020

Autoimmun Highlights

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Recently, several autoimmune neurological diseases have been defined by the presence of autoantibodies against different antigens of the nervous system. These autoantibodies have been demonstrated to be specific and useful biomarkers, and most of them are also pathogenic. These aspects have increased the value of autoantibodies in neurological practice, as they enable to establish more accurate diagnosis and to better understand the underlying mechanisms of the autoimmune neurological diseases when they are compared to those lacking them. Nevertheless, the exact mechanisms leading to the autoimmune response are still obscure. Genetic predisposition is likely to play a role in autoimmunity, HLA being the most reported genetic factor. Herein, we review the current knowledge about associations between HLA and autoimmune neurological diseases with autoantibodies. We report the main alleles and haplotypes, and discuss the clinical and pathogenic implications of these findings.

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“…The literature search initially retrieved 113 studies published between 2014 and 2019, from which 35 were excluded as duplicates, 38 after reading the title and abstract and 22 after reading the whole article (Table S1). Therefore, 18 articles met the selection criteria (see Figure 1): three case series 1,7,8 and 15 case reports, [11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] all with a SIGN level of evidence of 3, which described a total of 46 patients with IgLON5 treated with IT (Table 1).…”

Section: Searchmentioning

confidence: 99%

Response to immunotherapy in anti‐IgLON5 disease: A systematic review

et al. 2020

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The aim of this study was to evaluate the frequency of response to immunotherapy in patients with anti‐IgLON5 disease through a systematic review of the literature. MEDLINE and Embase databases were searched for studies that included patients with anti‐IgLON5 disease who received immunotherapy (IT). Review inclusion criteria were met by 18 studies. The main study variable was response to IT, defined as the frequency of patients with an improvement greater than mild in at least one of the main symptoms defined by the clinical phenotype. Data were also gathered on the rate of response to last follow‐up, the line(s) of IT received, the administration of monotherapy or combination therapy, and clinical and analytical characteristics. Selected studies included a total of 46 patients. A response to IT was observed in 20 (43.4%) and the presence of response to last follow‐up in 15 (32.6%). Response was achieved more frequently with combination therapy vs monotherapy (14/21 [66.6%] vs 7/22 [31.8%]) and second‐line therapy vs first‐line therapy (7/13 [53.8%] vs 15/46 [32.6%]). The response rate by drug was 34.2% (12/35) for steroids, 42.8% (9/21) for IVIg, 46% (7/15) for PLEX, 100% (5/5) for AZA and 75% (3/4) for MMF. Factors associated with a response to IT included the cognitive impairment and non‐classical phenotypes, presence of HLA‐DQB1*05:01 without HLA‐DRB1*10:01 and cerebral spinal fluid inflammation. Patients with anti‐IgLON5 disease respond to IT, and this response is associated with certain clinical and analytical characteristics of the patients. Also rate of response seems higher with second‐line and combination treatment. However, the quality of available studies is inadequate to allow definitive conclusions to be drawn.

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Anti‐IgLON5 Disease Responsive to Immunotherapy: A Case Report with an Abnormal MRI

Cited by 18 publications

References 11 publications

Early immunotherapy is highly effective in IgG1/IgG4 positive IgLON5 disease

Early immunotherapy is highly effective in IgG1/IgG4 positive IgLON5 disease

Associations between HLA and autoimmune neurological diseases with autoantibodies

Response to immunotherapy in anti‐IgLON5 disease: A systematic review

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