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

Abstract：

OBJECTIVE:To 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. METHODS:Specimens 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. RESULTS:Of 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. CONCLUSIONS:DPPX-IgG is a biomarker for an immunotherapy-responsive multifocal neurologic disorder of the central and autonomic nervous systems.

journal_name

Neurology

journal_title

Neurology

authors

Tobin WO,Lennon VA,Komorowski L,Probst C,Clardy SL,Aksamit AJ,Appendino JP,Lucchinetti CF,Matsumoto JY,Pittock SJ,Sandroni P,Tippmann-Peikert M,Wirrell EC,McKeon A

doi

10.1212/WNL.0000000000000991

subject

Has Abstract

pub_date

2014-11-11 00:00:00

pages

1797-803

issue

eissn

0028-3878

issn

1526-632X

pii

WNL.0000000000000991

journal_volume

pub_type

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