Voltage-Gated Potassium Channel Antibodies in Slow-Progression Motor Neuron Disease

Godani, Massimiliano; Zoccarato, Marco; Beronio, Alessandro; Zuliani, Luigi; Benedetti, Luana; Giometto, Bruno; Sette, Massimo Del; Raggio, E; Báldi, Rita; Vincent, Angela

doi:10.1159/000447715

Cited by 3 publications

(2 citation statements)

References 19 publications

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“…Cases with motor neuron disorder-like presentations may also display anti-CASPR2 [ 6 , 7 ]. Moreover, mutations of certain VGKC subunits may lead to motor neuron dysfunction, and antibodies to the VGKC complex are found in patients with motor neuron disorders [ 8 , 9 ], implying the significance of the VGKC complex in anterior horn functions.…”

Section: Discussionmentioning

confidence: 99%

Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Polyradiculopathy Associated with Epstein-Barr Virus Infection

Uzunalimoğlu¹,

Sağlam²,

Şişman³

et al. 2021

Case Rep Neurol

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Epstein-Barr virus (EBV) has been associated with a plethora of neurological manifestations including polyneuropathy and polyradiculopathy. A 27-year-old man with a recent upper respiratory system infection presented with difficulty in walking. His neurological examination revealed reduced muscle strength in both proximal and distal lower limb muscles without sensory and autonomic signs. Needle electromyography showed abnormal spontaneous activity and reduced recruitment of motor units in muscles innervated by multiple lumbo-sacral roots. Cerebrospinal examination showed increased protein levels with normal cell counts. While spinal MRI was normal, whole-body CT and PET examination showed disseminated lymph node enlargement. Anti-EBV viral capsid antigen and anti-nuclear antigen IgG but not IgM was positive, whereas EBV PCR was negative in blood. Analysis of inguinal lymph node biopsy showed reactive lymphoid hyperplasia and EBV DNA. Leucine-rich glioma-inactivated protein 1 (LGI1) antibody was found in serum but not in CSF. All clinical, imaging, and electrophysiological findings improved following steroid and intravenous immunoglobulin treatment. These findings suggested the acute involvement of lumbo-sacral spinal roots and/or motor neurons. Purely motor polyradiculopathy has been reported in both EBV-positive and LGI1 antibody-positive patients, and EBV infection is known to precede different autoimmune manifestations. Whether EBV infection may trigger LGI1 autoimmunity and cause involvement of spinal motor roots and/or motor neurons needs to be further studied.

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Section: Discussionmentioning

confidence: 99%

Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Polyradiculopathy Associated with Epstein-Barr Virus Infection

Uzunalimoğlu¹,

Sağlam²,

Şişman³

et al. 2021

Case Rep Neurol

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“…It has been developed by Neurimmune, a Swiss biotech company, and was originally isolated from B cell lines that had been generated from neurologically healthy individuals. It is very selective for the aggregated form of human aSyn that has been detected in tissue sections from PD and DLB patients [125]. A good safety and tolerance were recently demonstrated at single doses with up to 90 mg/kg in a phase I study in healthy volunteers.…”

Section: Immunotherapies To Target Alpha-synucleinmentioning

confidence: 99%

Novel Immunotherapeutic Approaches to Target Alpha-Synuclein and Related Neuroinflammation in Parkinson’s Disease

Zella

Metzdorf

Ostendorf

et al. 2019

Cells

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The etiology of Parkinson’s disease (PD) is significantly influenced by disease-causing changes in the protein alpha-Synuclein (aSyn). It can trigger and promote intracellular stress and thereby impair the function of dopaminergic neurons. However, these damage mechanisms do not only extend to neuronal cells, but also affect most glial cell populations, such as astroglia and microglia, but also T lymphocytes, which can no longer maintain the homeostatic CNS milieu because they produce neuroinflammatory responses to aSyn pathology. Through precise neuropathological examination, molecular characterization of biomaterials, and the use of PET technology, it has been clearly demonstrated that neuroinflammation is involved in human PD. In this review, we provide an in-depth overview of the pathomechanisms that aSyn elicits in models of disease and focus on the affected glial cell and lymphocyte populations and their interaction with pathogenic aSyn species. The interplay between aSyn and glial cells is analyzed both in the basic research setting and in the context of human neuropathology. Ultimately, a strong rationale builds up to therapeutically reduce the burden of pathological aSyn in the CNS. The current antibody-based approaches to lower the amount of aSyn and thereby alleviate neuroinflammatory responses is finally discussed as novel therapeutic strategies for PD.

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The presence and clinical significance of autoantibodies in amyotrophic lateral sclerosis: a narrative review

Liu,

Hong,

Wang

et al. 2024

Neurol Sci

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Voltage-Gated Potassium Channel Antibodies in Slow-Progression Motor Neuron Disease

Cited by 3 publications

References 19 publications

Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Polyradiculopathy Associated with Epstein-Barr Virus Infection

Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Polyradiculopathy Associated with Epstein-Barr Virus Infection

Novel Immunotherapeutic Approaches to Target Alpha-Synuclein and Related Neuroinflammation in Parkinson’s Disease

The presence and clinical significance of autoantibodies in amyotrophic lateral sclerosis: a narrative review

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