Clinical spectrum of voltage-gated potassium channel autoimmunity

Tan, K. Meng; Lennon, Vanda A.; Klein, Christopher J.; Boeve, Bradley F.; Pittock, Sean J.

doi:10.1212/01.wnl.0000312275.04260.a0

Cited by 261 publications

(251 citation statements)

References 36 publications

(46 reference statements)

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“…VGKC-complex antibody testing was performed using a radioimmunoassay as previously described. 8 The upper limit of normal range for the antibody was set at 0.02 nM per the Mayo Medical Laboratory. 8 Antibody panel ordering was at the discretion of treating neurologists based on patients' clinical features, without predetermined clinical criteria.…”

Section: Study Populationmentioning

confidence: 99%

“…8 The upper limit of normal range for the antibody was set at 0.02 nM per the Mayo Medical Laboratory. 8 Antibody panel ordering was at the discretion of treating neurologists based on patients' clinical features, without predetermined clinical criteria. A comprehensive retrospective review of patients positive for VGKC-complex antibodies was performed.…”

Section: Study Populationmentioning

confidence: 99%

“…8 A low cutoff level of VGKC-complex antibody increased sensitivity to a broader spectrum of potential autoimmunity. However, it also put the antibody specificity and clinical relevance in question.…”

Section: Low Vgkc-complex Antibody Titermentioning

confidence: 99%

“…22 Previous analyses reported varying tumor incidences of 10%-47% in VGKC-complex antibody-positive patients. 8,9,16,17 One study reported the highest tumor incidence of 47% in their report of 72 patients, and detection of VGKC-complex antibodies expedited tumor diagnosis in 18 patients. 8 The authors recommended that all VGKC-complex antibodypositive patients undergo comprehensive physical examination, CT of chest, abdomen, and pelvis, mammography (in women), and serum assay of prostate-specific antigen (in men).…”

Section: Tumor Associationmentioning

confidence: 99%

“…4,5 In the last decade, VGKC-complex antibodies have been detected in an expanding spectrum of neurologic disorders, including autonomic dysfunction, chronic epilepsy, peripheral neuropathy, motor neuron disease, dementia, and depression. [6][7][8][9][10] However, the clinical value of VGKC-complex antibodies in this group of disorders is less clear.…”

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Clinical utility of seropositive voltage-gated potassium channel–complex antibody

et al. 2016

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Background: Antibodies against voltage-gated potassium channel (VGKC)-complex are implicated in the pathogenesis of acquired neuromyotonia, limbic encephalitis, faciobrachial dystonic seizure, and Morvan syndrome. Outside these entities, the clinical value of VGKC-complex antibodies remains unclear. Methods: We conducted a single-center review of patients positive for VGKC-complex antibodies over an 8-year period. Results: Among 114 patients positive for VGKC-complex antibody, 11 (9.6%) carrying the diagnosis of limbic encephalitis (n 5 9) or neuromyotonia (n 5 2) constituted the classic group, and the remaining 103 cases of various neurologic and non-neurologic disorders comprised the nonclassic group. The median titer for the classic group was higher than the nonclassic group (p , 0.0001). A total of 90.9% of the patients in the classic and 21.4% in the nonclassic group possessed high (.0.25 nM) VGKC-complex antibody levels (p , 0.0001). A total of 75.0% of the patients in the high-level group had definite or probable autoimmune basis, while nonautoimmune disorders were seen in 75.6% of patients from the low-level group (p , 0.0001). A total of 26.3% of patients were found with active or remote solid organ or hematologic malignancy, but no antibody titer difference was observed among subgroups of absent, active, or remote malignancy. Compared to age-matched US national census, rates of active cancer in our cohort were higher in patients older than 45 years. Conclusions: High VGKC-complex antibody titers are more likely found in patients with classically associated syndromes and other autoimmune conditions. Low-level VGKC-complex antibodies can be detected in nonspecific and mostly nonautoimmune disorders. The presence of VGKC-complex antibody, rather than its level, may serve as a marker of malignancy. Neurol Clin Pract 2016;6:409-418 A ntibodies against voltage-gated potassium channel (VGKC)-complex were first identified in the peripheral nerve hyperexcitability disorder neuromyotonia, and subsequently in patients with Morvan syndrome, limbic encephalitis (LE), and faciobrachial dystonic seizure. 1-4 These diagnoses, constituting the classic Neurohospitalist Program (AJ),

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Section: Study Populationmentioning

confidence: 99%

Section: Study Populationmentioning

confidence: 99%

Section: Low Vgkc-complex Antibody Titermentioning

confidence: 99%

Section: Tumor Associationmentioning

confidence: 99%

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confidence: 99%

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Clinical utility of seropositive voltage-gated potassium channel–complex antibody

et al. 2016

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Voltage-Gated Potassium Channel Autoimmunity Mimicking Creutzfeldt-Jakob Disease

Geschwind¹,

Tan²,

Lennon³

et al. 2008

Arch Neurol

183

162

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Polymyoclonus, Laryngospasm, and Cerebellar Ataxia Associated With Adenocarcinoma and Multiple Neural Cation Channel Autoantibodies

Lim¹,

Mason²,

Young³

et al. 2009

Arch Neurol

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To describe and provide audiovisual documentation of a syndrome of polymyoclonus, laryngospasm, and cerebellar ataxia associated with adenocarcinoma and multiple neural cation channel autoantibodies. Design: Case report with video. Setting: University hospitals. Patient: A 69-year-old woman presented with subacute onset of whole-body tremulousness and laryngospasm attributed to gastroesophageal reflux. Results: Further evaluation revealed polymyoclonus, cerebellar ataxia, and laryngospasm suspicious of an underlying malignant neoplasm. Surface electromyography of multiple limb muscles confirmed the presence of polymyoclonus. The patient was seropositive for P/Qtype voltage-gated calcium channel antibody; subsequently, whole-body fluorine 18 fluorodeoxyglucose positron emission tomography and cervical lymph node biopsy revealed widespread metastatic adenocarcinoma. Follow-up serologic evaluation revealed calcium channel antibodies (P/Q type and N type) and potassium channel antibody. Conclusions: We highlight the importance of recognizing polymyoclonus. To our knowledge, this is also the first description of a syndrome of polymyoclonus, laryngospasm, and ataxia associated with adenocarcinoma and these cation channel antibodies.

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Clinical spectrum of voltage-gated potassium channel autoimmunity

Cited by 261 publications

References 36 publications

Clinical utility of seropositive voltage-gated potassium channel–complex antibody

Clinical utility of seropositive voltage-gated potassium channel–complex antibody

Voltage-Gated Potassium Channel Autoimmunity Mimicking Creutzfeldt-Jakob Disease

Polymyoclonus, Laryngospasm, and Cerebellar Ataxia Associated With Adenocarcinoma and Multiple Neural Cation Channel Autoantibodies

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