Evidence map›Paper›PMID 25320100›Full record

ArticleNeurology2014

DPPX potassium channel antibody: frequency, clinical accompaniments, and outcomes in 20 patients.

William Oliver Tobin, Vanda A Lennon, Lars Komorowski, Christian Probst, Stacey Lynn Clardy, Allen J Aksamit, Juan Pablo Appendino, Claudia F Lucchinetti, Joseph Y Matsumoto, Sean J Pittock and 4 more

Open access · bronzeAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Neurology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 114 papers, 5 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
114citing papers in PubMed, 5 pooled it
13.3field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

114 citing papers in PubMed, 5 syntheses or guidelines pooled it, 295 citations in OpenAlex.

  1. Pooled it
  2. Pooled it
  3. Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple's disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022
    Pooled it
  4. Genotype-Phenotype Relations for the Atypical Parkinsonism Genes: MDSGene Systematic Review.Movement disorders : official journal of the Movement Disorder Society · 2021
    Pooled it
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  6. Autoimmune neuropsychiatric disorders manifesting with psychosis.The Journal of clinical investigation · 2025
    Review
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54 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

14 authors at 1 institution in 2 countries.

William Oliver TobinFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Vanda A LennonFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Lars KomorowskiFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Christian ProbstFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Stacey Lynn ClardyFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Allen J AksamitFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Juan Pablo AppendinoFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Claudia F LucchinettiFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Joseph Y MatsumotoFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Sean J PittockFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Paola SandroniFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Maja Tippmann-PeikertFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Elaine C WirrellFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada.
Andrew McKeonFrom the Departments of Neurology (W.O.T., V.A.L., S.L.C., A.J.A., C.F.L., J.Y.M., S.J.P., P.S., M.T.-P., E.C.W., A.M.), Laboratory Medicine and Pathology (V.A.L., S.J.P., A.M.), Immunology (V.A.L.), and Pediatrics (E.C.W.), College of Medicine, Mayo Clinic, Rochester, MN; Euroimmun AG (L.K., C.P.), Lübeck, Germany; and Neurology (J.P.A.), Faculty of Medicine, University of Manitoba, Canada. mckeon.andrew@mayo.edu.
Anna Needs Neuroblastoma Answers · US

Funding

Mechanisms of Multiple Sclerosis Tissue PathologyR01NS049577 · NINDS · MAYO CLINIC ROCHESTER · PI LUCCHINETTI, CLAUDIA F. · 2006 to 2012
$2.3M
CNS Demyelinating Autoimmunity Targeting Water Channel ComplexesR01NS065829 · NINDS · MAYO CLINIC ROCHESTER · PI PITTOCK, SEAN JOSEPH · 2010 to 2014
$1.7M
NINDS NIH HHS NS49577-R01NINDS NIH HHS R01 NS065829NINDS NIH HHS R01NS065829
6 · The paper itself

Abstract

objectiveTo describe the detection frequency and clinical associations of immunoglobulin G (IgG) targeting dipeptidyl-peptidase-like protein-6 (DPPX), a regulatory subunit of neuronal Kv4.2 potassium channels.

methodsSpecimens from 20 patients evaluated on a service basis by tissue-based immunofluorescence yielded a synaptic immunostaining pattern consistent with DPPX-IgG (serum, 20; CSF, all 7 available). Transfected HEK293 cell-based assay confirmed DPPX specificity in all specimens. Sixty-nine patients with stiff-person syndrome and related disorders were also evaluated by DPPX-IgG cell-based assay.

resultsOf 20 seropositive patients, 12 were men; median symptom onset age was 53 years (range, 13-75). Symptom onset was insidious in 15 and subacute in 5. Twelve patients reported prodromal weight loss. Neurologic disorders were multifocal. All had one or more brain or brainstem manifestations: amnesia (16), delirium (8), psychosis (4), depression (4), seizures (2), and brainstem disorders (15; eye movement disturbances [8], ataxia [7], dysphagia [6], dysarthria [4], respiratory failure [3]). Nine patients reported sleep disturbance. Manifestations of central hyperexcitability included myoclonus (8), exaggerated startle (6), diffuse rigidity (6), and hyperreflexia (6). Dysautonomia involved the gastrointestinal tract (9; diarrhea [6], gastroparesis, and constipation [3]), bladder (7), cardiac conduction system (3), and thermoregulation (1). Two patients had B-cell neoplasms: gastrointestinal lymphoma (1), and chronic lymphocytic leukemia (1). Substantial neurologic improvements followed immunotherapy in 7 of 11 patients with available treatment data. DPPX-IgG was not detected in any of the stiff-person syndrome patients.

conclusionsDPPX-IgG is a biomarker for an immunotherapy-responsive multifocal neurologic disorder of the central and autonomic nervous systems.

Indexed as

AdolescentAdrenal Cortex HormonesAdultAgedAutonomic Nervous SystemBrainDipeptidyl-Peptidases and Tripeptidyl-PeptidasesFemaleGastrointestinal DiseasesHEK293 CellsHumansImmunoglobulin GImmunotherapyMagnetic Resonance ImagingMaleMental DisordersAdrenal Cortex HormonesDipeptidyl-Peptidases and Tripeptidyl-PeptidasesDPP6 protein, humanImmunoglobulin GNerve Tissue ProteinsPotassium Channels

Identifiers

PMID25320100
PMCPMC4240433
OpenAlexW2068641392

What OpenQuestion holds

Textmetadata
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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.