Pruritus in anti-DPPX encephalitis

Wijntjes, Juerd; Bechakra, Malik; Schreurs, Marco W. J.; Jongen, Joost L.M.; Koppenaal, Aart; Titulaer, Maarten J.

doi:10.1212/nxi.0000000000000455

Cited by 16 publications

(17 citation statements)

References 7 publications

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“…For all antibody-defined AIE subgroups combined, we could identify 116 publications that matched our search criteria and contained relevant information for the intended analyses. Ten were identified for AIE with AMPAR antibodies (7–16), 15 for CASPR2 (16–30), eight for DPPX (31–38), four for GABA A R (15, 39–41) with patients reported by the Pettingil et al being excluded as these were not tested for GABA A R antibodies in CSF and represented a different phenotype (42), 15 for GABA B R antibodies (12, 15, 16, 43–54), 15 for GAD antibodies (19, 22, 55–67), five for GlyR antibodies (68–72), 14 for IgLON5 antibodies (73–86), 25 for LGI1 antibodies (5, 6, 15, 16, 19–22, 30, 52, 77, 87–100), and finally 27 for NMDAR antibodies (4, 15, 16, 19, 22, 52, 62, 88, 89, 101–118). The history of the data search and the results are depicted in Figure 1.…”

Section: Resultsmentioning

confidence: 99%

“…For that purpose, we expanded our literature search to publications with <3 patients for AIE associated with AMPAR, CASPR2, DPPX, GABA B R, GAB A AR, and IgLON5 antibodies as for these <15 patients were identified with the full data set. Thereby, we increased the number of patients by 10 for AIE with IgLON5 antibodies (74, 75, 79–86), three for AIE with AMPAR antibodies (8, 10, 11), by two for AIE with DPPX antibodies (35, 38), by one for AIE with CASPR2 (21) and GABA B R antibodies each (47). However, the number of individual patients with information of all three CSF parameters remained <10 for AIE with DPPX, CASPR2, GABA B R, and GABA A R antibodies (Supplementary Table 3).…”

Section: Resultsmentioning

confidence: 99%

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Cerebrospinal Fluid Findings in Patients With Autoimmune Encephalitis—A Systematic Analysis

2019

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Autoimmune encephalitides (AIE) comprise a group of inflammatory diseases of the central nervous system (CNS), which can be further characterized by the presence of different antineuronal antibodies. Recently, a clinical approach for diagnostic criteria for the suspected diagnosis of AIE as well as definitive AIE were proposed. These are intended to guide physicians when to order the antineuronal antibody testing and/or facilitate early diagnosis even prior to the availability of the specific disease-confirming test results to facilitate prompt treatment. These diagnostic criteria also include the results of basic cerebrospinal fluid (CSF) analysis. However, the different antibody-defined AIE subtypes might be highly distinct with regard to their immune pathophysiology, e.g., the pre-dominance of specific IgG subclasses, IgG1, or IgG4, or frequency of paraneoplastic compared to idiopathic origin. Thus, it is conceivable that the results of basic CSF analysis might also be very different. However, this has not been explored systematically. Here, we systematically reviewed the literature about the 10 most important AIE subtypes, AIE with antibodies against NMDA, AMPA, glycine, GABA A , and GABA B receptors as well as DPPX, CASPR2, LGI1, IgLON5, or glutamate decarboxylase (GAD), with respect to the reported basic CSF findings comprising CSF leukocyte count, total protein, and the presence of oligoclonal bands (OCB) restricted to the CSF as a sensitive measure for intrathecal IgG synthesis. Our results indicate that these basic CSF findings are profoundly different among the 10 different AIE subtypes. Whereas, AIEs with antibodies against NMDA, GABA B , and AMPA receptors as well as DPPX show rather frequent inflammatory CSF changes, in AIEs with either CASPR2, LGI1, GABA A , or glycine receptor antibodies CSF findings were mostly normal. Two subtypes, AIEs defined by either GAD, or IgLON5 antibodies, did not fit into this general pattern. In AIE with GAD antibodies, positive OCBs in the absence of other changes were typical, while the CSF in IgLON5 antibody-positive AIE was characterized by elevated protein.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Cerebrospinal Fluid Findings in Patients With Autoimmune Encephalitis—A Systematic Analysis

2019

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“…Our case features the rare finding of oro‐bucco‐lingual dyskinesia, which is seen in the accompanying Video S1, likely the result of central nervous system hyperexcitability. Another unique aspect includes the prodrome of diffuse pruritus, which has been reported in few patients 6,8 …”

Section: Discussionmentioning

confidence: 99%

Oro‐Bucco‐Lingual Dyskinesia, Weight Loss, and Cognitive Decline in Anti‐DPPX Antibody‐Mediated Encephalitis

Deuel

Vaughan

et al. 2020

Movement Disord Clin Pract

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“…Since the disorder of anti-DPPX encephalitis was discovered, till September 2020, the number of patients reported is 44 [4,[7][8][9][10][11][12][13][14][15][16]. Combining our nine patients (a total of 53), the median age was 52 years (range 13-76 years), and 34 (63%) were male.…”

Section: Summary Of Anti-dppx Encephalitis Syndrome Spectrummentioning

confidence: 99%

Clinical and imaging analysis to evaluate the response of patients with anti-DPPX encephalitis to immunotherapy

Xiao

2021

Preprint

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Background: To report the main spectrum, new clinical and imaging characteristics of dipeptidyl-peptidase-like protein 6 (DPPX) antibody-associated encephalitis, and evaluate the effect of immunotherapy.Methods: A retrospective analysis of nine patients reported describing the clinical and immunological features was performed, and all previously reported cases were reviewed. A cell-based indirect immunofluorescence assay with human embryonic kidney 293 cells transfected with DPPX was used. Results: Nine patients were identified (median age, 51 years; range, 14–65 years) with prodromal fever, diarrhea, or weight loss, followed by a rapidly progressive encephalopathy characterized by cognitive disorder. One patient who received methylprednisolone therapy and a trail of tacrolimus showed substantial improvement and had no relapse in the six-month follow-up. Our comprehensive literature review demonstrated that a total of 53 cases were reported, of which more than half had prodromal weight loss (52.8%), and gastrointestinal disorders (58.5%). Cognitive (74.6%) and brainstem/spinal cord disorders (75.5%) were the most common major symptoms. Our study is the first to report three patients with anti-DPPX encephalitis who had sleep disorder of rapid eye movement sleep behavior disorder (RBD), limb paralysis (two), severe pleocytosis and elevated protein levels (two) in the cerebrospinal fluid, and increased T2/FLAIR signal abnormalities in the bilateral hippocampus, temporal lobe, amygdala, basal ganglia, thalamus, centrum semiovale, and frontal and parietal lobes in seven cases (77.8%).Conclusion: Our study expanded the clinical and imaging phenotype of anti-DPPX- encephalitis. We also reported the use of tacrolimus for long-term immunosuppressant therapy in anti-DPPX encephalitis, with substantial improvement and no relapse during a follow-up period of 6 months. Further studies elucidating the entire clinical spectrum of anti-DPPX encephalitis, pathogenic roles, and prognosis under long-term immunosuppressive therapy are warranted.

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Pruritus in anti-DPPX encephalitis

Cited by 16 publications

References 7 publications

Cerebrospinal Fluid Findings in Patients With Autoimmune Encephalitis—A Systematic Analysis

Cerebrospinal Fluid Findings in Patients With Autoimmune Encephalitis—A Systematic Analysis

Oro‐Bucco‐Lingual Dyskinesia, Weight Loss, and Cognitive Decline in Anti‐DPPX Antibody‐Mediated Encephalitis

Clinical and imaging analysis to evaluate the response of patients with anti-DPPX encephalitis to immunotherapy

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