Voltage-gated potassium channel complex antibodies in Creutzfeldt-Jakob disease

Fujita, Koji; Yuasa, Tatsuhiko; Watanabe, Osamu; Takahashi, Yukitoshi; Hashiguchi, Shuji; Adachi, Kazutaka; Izumi, Yuishin; Kaji, Ryuji

doi:10.1007/s00415-012-6554-y

Cited by 22 publications

(17 citation statements)

References 6 publications

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“…Third, the clinical features of limbic encephalitis with antibodies against voltage-gated potassium channel (VGKC) complex can also be similar to those of CJD (Geschwind et al, 2008). Although anti-VGKC complex antibodies were first reported to be absent in CJD patients (Geschwind et al, 2008), we do not exclude the association of anti-VGKC complex antibodies and CJD, as recently documented (Fujita et al, 2012b). Detection of antigens that are specifically targeted by the antibodies in CJD patients will lead to further understanding of the pathogenesis of the disease.…”

Section: Discussionmentioning

confidence: 69%

Antibodies to N-methyl-D-aspartate glutamate receptors in Creutzfeldt–Jakob disease patients

Fujita

Yuasa²,

Takahashi

et al. 2012

Journal of Neuroimmunology

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Section: Discussionmentioning

confidence: 69%

Antibodies to N-methyl-D-aspartate glutamate receptors in Creutzfeldt–Jakob disease patients

Fujita

Yuasa²,

Takahashi

et al. 2012

Journal of Neuroimmunology

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“…His neuropathological examination revealed spongiform changes and synaptic deposition of PrP in the cerebral cortex, confirming the diagnosis of definite CJD. A known pathogenic M232R mutation in the PRNP gene was detected further confirming the diagnosis of genetic CJD 6. Angus-Leppan et al reported a 68-year-old man presenting with rapidly progressive insomnia, confusion, myoclonus and stimulus-sensitive dystonia or choreoathetosis.…”

Section: Discussionmentioning

confidence: 79%

“…6 and 7) and the 15 cases of CJD-mimicking VGKC complex antibody-mediated autoimmunity reported by Geschwind et al 4…”

Section: Discussionmentioning

confidence: 96%

Presence of voltage-gated potassium channel complex antibody in a case of genetic prion disease

et al. 2014

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Voltage-gated potassium channel (VGKC) complex antibody-mediated encephalitis is a recently recognised entity which has been reported to mimic the clinical presentation of Creutzfeldt-Jakob disease (CJD). Testing for the presence of this neuronal surface autoantibody in patients presenting with subacute encephalopathy is therefore crucial as it may both revoke the bleak diagnosis of prion disease and allow institution of potentially life-saving immunotherapy. Tempering this optimistic view is the rare instance when a positive VGKC complex antibody titre occurs in a definite case of prion disease. We present a pathologically and genetically confirmed case of CJD with elevated serum VGKC complex antibody titres. This case highlights the importance of interpreting the result of a positive VGKC complex antibody with caution and in the context of the overall clinical manifestation.

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“…This wide range of syndromes includes not only diseases restricted to the central nervous system (fever-induced refractory epileptic encephalopathy, epilepsy, encephalitis in children, rapidly progressing dementia, and Creutzfeldt-Jakob disease) but also those affecting the peripheral nervous system (neuropathy, neuropathic pain). 2,[26][27][28][29] The precise antigens are unknown in all these cases: it is not even clear whether they are intracellular or extracellular. Furthermore, responses to immunosuppressive treatment in the cases described above vary greatly, which highlights the relevance of the diagnostic value and pathogenesis of these antibodies.…”

Section: Syndromes and Symptoms Associated With Antibodies Against Vomentioning

confidence: 96%

Clinical spectrum and diagnostic value of antibodies against the potassium channel-related protein complex

Montojo

Petit‐Pedrol

Graus

et al. 2015

Neurología (English Edition)

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Introduction: Antibodies against a protein complex that includes voltage-gated potassium channels (VGKC) have been reported in patients with limbic encephalitis, peripheral nerve hyperexcitability, Morvan's syndrome, and a large variety of neurological syndromes.Review summary: In this article, a review is presented of the syndromes associated with antibodies against VGKC-related proteins and the main antigens of this protein complex, the proteins LGI1 (leucine rich glioma inactivated protein 1) and Caspr2 (contactin-associated protein-like 2). The conceptual problems and clinical implications of the description of antibodies against VGKC-related proteins other than LGI1 and Caspr2 are also discussed.Although initial studies indicated the occurrence of antibodies against VGKC, recent investigations have shown that the main antigens are a neuronal secreted protein known as LGI1 which modulates synaptic excitability, and a protein called Caspr2 located on the cell surface and processes of neurons of different brain regions, and at the juxtaparanodal region of myelinated axons. While antibodies against LGI1 preferentially associate with classical limbic encephalitis, antibodies against Caspr2 associate with a wider spectrum of symptoms, including Morvan's syndrome, peripheral nerve hyperexcitability or neuromyotonia, and limbic or more extensive encephalitis. In addition there are reports of patients with antibodies against VGKC-related proteins that are different from LGI1 or Caspr2. In these cases, the identity and location of the antigens are unknown, the syndrome association is not specific, and the response to treatment uncertain.ଝ Please cite this article as: Montojo MT, Petit-Pedrol M, Graus F, Dalmau J. Espectro clínico y valor diagnóstico de los anticuerpos contra el complejo proteico asociado a canales de potasio. Neurología. 2015;30:295-301.

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Voltage-gated potassium channel complex antibodies in Creutzfeldt-Jakob disease

Cited by 22 publications

References 6 publications

Antibodies to N-methyl-D-aspartate glutamate receptors in Creutzfeldt–Jakob disease patients

Antibodies to N-methyl-D-aspartate glutamate receptors in Creutzfeldt–Jakob disease patients

Presence of voltage-gated potassium channel complex antibody in a case of genetic prion disease

Clinical spectrum and diagnostic value of antibodies against the potassium channel-related protein complex

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