scholarly journals A Patient with Double-Negative VGKC, Peripheral Nerve Hyperexcitability, and Central Nervous System Symptoms: A Postinfectious Autoimmune Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Birte Eikeland
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Autoimmune Disease ◽  
Peripheral Nerve ◽  
Skin Lesions ◽  
Double Negative ◽  
Pathogenic Potential ◽  
Peripheral Nerve Hyperexcitability ◽  
Systemic Symptoms ◽  
Kinetics Of

Research in the last few years has indicated that most voltage-gated potassium channel- (VGKC-) complex antibodies without leucine-rich glioma-inactivated protein 1 or contactin-associated protein-like 2 antibody specificity lack pathogenic potential and are not clear markers for autoimmune inflammation. Here we report on a patient with double-negative VGKC who developed severe peripheral nerve hyperexcitability, central nervous system symptoms with agitation and insomnia, dysautonomia, and systemic symptoms with weight loss, itch, and skin lesions. The disease started acutely one month after an episode of enteroviral pericarditis and responded well to immunotherapy. The patient is presumed to have developed a postinfectious immunotherapy-responsive autoimmune disease. In the setting of anti-VGKC positivity, it seems likely that anti-VGKC contributed to the pathogenesis of the patient’s symptoms of nerve hyperexcitability and that the disease was caused by an acquired autoimmune effect on the neuronal kinetics of VGKC. It is still unknown whether or not there are unidentified extracellular molecular targets within the VGKC-complex, i.e., a novel surface antigen and a pathogenic antibody that can cause affected individuals to develop a peripheral nerve hyperexcitability syndrome. This case highlights the fact that less well-characterized autoimmune central and peripheral nervous system syndromes may have infectious triggers.

P341 Painful legs and moving toes syndrome associated with peripheral nerve hyperexcitability and central nervous system involvement

2017 ◽  
Vol 128 (9) ◽  
pp. e288-e289
Author(s):  
Sara Cors-Serra ◽  
Ángeles Lloret-Alcañiz ◽  
Paula Cases-Bergón ◽  
Cristina Ipiéns-Escuer ◽  
Lara Mauri-Fábrega ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Peripheral Nerve ◽  
Central Nervous System Involvement ◽  
System Involvement ◽  
Peripheral Nerve Hyperexcitability

scholarly journals 045 Primary cutibacterium acnes central nervous system infection causing focal seizures: an unusual presentation of a rare disease

2019 ◽  
Vol 90 (e7) ◽  
pp. A15.2-A15
Author(s):  
Sophie E Waller ◽  
Sarah Browning ◽  
Elizabeth Pepper
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Infection ◽  
Cns Infection ◽  
Delayed Presentation ◽  
Pathogenic Potential ◽  
Focal Seizures ◽  
Device Infection ◽  
Cutibacterium Acnes ◽  
Neurosurgical Intervention

IntroductionCutibacterium acnes is a Gram positive, anaerobic bacterium of low pathogenic potential that forms part of the normal cutaneous flora. Although most often identified as a contaminant in culture of microbiological specimens, it is commonly implicated in both postoperative wound and implantable device infection. Neurosurgical device infections secondary to C. acnes are well recognised and are likely secondary to bacterial contamination from the skin during surgery. Indolent infection characterised by delayed presentation of weeks to months following intervention is common. C. acnes infection involving the central nervous system (CNS) in the absence of previous neurosurgical intervention is rare, but has been described following dental or mastoid infections and following facial trauma. A further case series has reported de novo C. acnes CNS infection occurring in the absence of these recognised risk factors, but with clinical features of meningitis being common to all.Methods and resultsWe describe a unique case of primary C. acnes extra-dural collection in a previously well patient with no neurosurgical history presenting with sub-acute focal seizures and progressive focal leptomeningeal thickening on MRI.ConclusionC. acnes CNS infection can occur in the immunocompetent and in the absence of neurosurgical intervention.

scholarly journals Pharmacological prion protein silencing accelerates central nervous system autoimmune disease via T cell receptor signalling

Brain ◽  
2010 ◽  
Vol 133 (2) ◽  
pp. 375-388 ◽  
Author(s):  
Wei Hu ◽  
Stefan Nessler ◽  
Bernhard Hemmer ◽  
Todd N. Eagar ◽  
Lawrence P. Kane ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
T Cell ◽  
Autoimmune Disease ◽  
Prion Protein ◽  
T Cell Receptor ◽  
Cell Receptor ◽  
Receptor Signalling

A Newly Developed Mouse Monoclonal SOX10 Antibody Is a Highly Sensitive and Specific Marker for Malignant Melanoma, Including Spindle Cell and Desmoplastic Melanomas

2014 ◽  
Vol 139 (4) ◽  
pp. 530-536 ◽  
Author(s):  
David Tacha ◽  
Weimin Qi ◽  
Seong Ra ◽  
Ryan Bremer ◽  
Charlie Yu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Malignant Melanoma ◽  
Peripheral Nerve ◽  
Immunohistochemical Staining ◽  
Spindle Cell ◽  
Breast Carcinomas ◽  
Peripheral Nerve Sheath Tumors ◽  
Highly Sensitive ◽  
Wide Range

Context Recent immunohistochemical studies have demonstrated Sry-related HMG-Box gene 10 (SOX10) expression in malignant melanomas, malignant peripheral nerve sheath tumors, a subset of breast carcinomas, and gliomas. SOX10 has shown important clinical utility in its ability to detect desmoplastic and spindle cell melanomas. To date, most publications have employed a research use–only goat polyclonal SOX10 antibody for immunohistochemical staining. Objective To describe the development of a new mouse monoclonal SOX10 antibody (BC34) and evaluate its immunohistochemical staining profile in a wide range of normal and neoplastic tissues, with an emphasis on melanoma. Design SOX10 antibody was optimized for staining using a polymer detection system and visualization with diaminobenzidine. Results In normal tissues, SOX10 was expressed in skin melanocytes and eccrine cells, breast myoepithelial and lobular epithelial cells, salivary gland myoepithelial cells, peripheral nerve Schwann cells, and central nervous system glial cells. SOX10 was expressed in 238 of 257 melanomas (92.6%), including 50 of 51 of both spindle cell and desmoplastic melanomas (98%). SOX10 was expressed in 100% of nevi (20 of 20) and schwannomas (28 of 28). In other neoplasms, SOX10 was expressed in 18 of 109 invasive ductal breast carcinomas (16.5%). All other carcinomas were negative for SOX10. SOX10 was identified in 25 of 52 central nervous system neoplasms, primarily in astrocytomas (22 of 41; 53.7%), and in 4 of 99 various sarcomas examined (4.0%). Conclusions The newly developed mouse monoclonal SOX10 antibody BC34 is highly sensitive and specific for malignant melanoma, including desmoplastic and spindle cell variants, and appears highly suitable for clinical use.

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