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• W1569853514 abstract "OBJECTIVE:To report the detection frequency of Dipeptidyl-Peptidase-Like Protein-6 (DPPX) antibody and associated clinical findings.BACKGROUND:Encephalitis and hyperexcitable phenotypes (seizures, myoclonus, agitation and diarrhea) have been described in 4 patients with IgG targeting DPPX, a regulatory subunit of the neuronal Kv4.2 potassium channel.DESIGN/METHODS:DPPX-IgG was identified in 14 patients by mouse tissue-based immunofluorescence (serum, 14; CSF, 5 of 5 patients tested). Positivity was confirmed by DPPX-transfected HEK293 cell-based assay. Ten of these patients were identified retrospectively by re-testing 123 patients for whom an unclassified synaptic autoantibody suggestive of DPPX-IgG had been recorded in the period 1991-2012. Four were identified prospectively in 2013 (one every 10 weeks). Medical records were available for 10 patients.RESULTS:The median age at symptom onset was 56 years (range, 27-75); 8/14 were male. Prodromal weight loss, reported in 8/10 patients, was often severe (40-50 kg). Neurological disorders were multifocal in 9/10. Cerebral disorders included amnesia (7), delirium (5), psychosis (2), seizures (1), headache and depression (1). Brainstem disorders included respiratory failure (5), dysphagia (3), eye movement disorders (2), dysarthria (2), and ataxia (1). Disturbed sleep was reported by 4 patients, 3 of whom had sleep studies demonstrating periodic limb movements of sleep (2), intrusion of REM sleep into non-REM sleep (1) and central sleep apnea (1). Other encephalomyelitic findings were myoclonus (5), diffuse rigidity with brisk reflexes (4), and exaggerated startle (3). Autonomic disorders affected gastrointestinal tract (6; diarrhea [3], gastroparesis and constipation [3]), bladder (2), and temperature regulation (1). A neoplasm was detected in 1 patient (B cell gastrointestinal lymphoma). Of 7 patients for whom longitudinal data were available, 5 had substantial neurological improvements attributable to: corticosteroids (3), rituximab alone (1) and cyclophosphamide plus rituximab (1).CONCLUSIONS:DPPX-IgG is a biomarker for a treatable, autoimmune CNS and autonomic disorder with predominant (but not exclusive) hyperexcitable phenotype. Disclosure: Dr. Lennon stands to receive royalty payments for commercial assays to detect of Aquaporin 4-specific Autoantibody. Dr. Komorowski has received personal compensation for activities with EuroImmun AG as an employee. Dr. Probst has received personal compensation for activities with EuroImmun AG as an employee. Dr. Aksamit has nothing to disclose. Dr. Lucchinetti stands to receive royalty payments from Biogen Idec. Dr. Pittock9s institution has received compensation for activities with Alexion Pharmaceuticals, MedImmune, and Chugai Pharma. Dr. Pittock stands to receive royalty payments from the technology entitled Neuromyelitis Optica Autoantibodies as a Marker for Neoplasia. Dr. Pittock has received research support from Alexion Pharmaceuticals, Inc. Dr. Tippmann-Peikert has nothing to disclose. Dr. Wirrell has received research support from the Mayo Foundation. Dr. McKeon has nothing to disclose." @default.
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• W1569853514 date "2014-04-08" @default.
• W1569853514 modified "2023-09-23" @default.
• W1569853514 title "DPPX Autoantibody: Frequency, Clinical Accompaniments and Outcomes (S40.001)" @default.
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