“Limbic Encephalitis” and Its Association With Carcinoma

Corsellis, J. A. N.; Goldberg, Geoffrey; Norton, Andrews

doi:10.1093/brain/91.3.481

Cited by 441 publications

(222 citation statements)

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“…Clinical features of LE and neuromyotonia were in keeping with original reports. 13,14 No cases of faciobrachial dystonic seizure or Morvan syndrome were encountered. The remaining 103 cases were classified as the nonclassic group.…”

Section: Resultsmentioning

confidence: 99%

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: Resultsmentioning

confidence: 99%

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

et al. 2016

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“…It classically presents with a constellation of symptoms including memory disturbances, complex partial seizures along with behavioural abnormalities and sleep disturbances [2]. Etiology wise, autoimmune limbic encephalitis can either be associated with antibodies to intracellular neuronal antigens, which include all previously known paraneoplastic antigens, and the other associated with antibodies to cell membrane antigens, including the VGKC, NMDA receptor, GAD which have no evidence of any tumour in body [10].…”

Section: Discussionmentioning

confidence: 99%

“…Viral infections, inflammatory or autoimmune disorders (lupus, Sjogren's, Hashimoto thyroiditis and CNS vasculitis), toxic and metabolic encephalopathy, paraneoplastic syndromes and autoimmunity become the possible clinical etiologies. Classically, limbic encephalitis was mostly considered a paraneoplastic disorder [2,3] but many new antibodies are being discovered in patients of Limbic Encephalitis which are not associated with tumours, thereby arising possibility of autoimmune basis to this disease [1,4].…”

Section: Introductionmentioning

confidence: 99%

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A Case of Limbic Encephalitis: Antibody LGI1 Associated Encephalitis

Bhardwaj¹,

Sharma²

2016

J Neurol Neurosci

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Limbic encephalitis (LE) is a rarely encountered disease in modern clinical practice. It is basically autoimmune in nature, with its pathophysiology determined by a number of antibodies to neural surface proteins. However, 20% of cases are paraneoplastic and have a tumour source in the body which leads to limbic encephalitis secondarily. Studies have shown that VGKC complex (Voltage Gated Potassium Channels) is one of the common antibodies found in this disease and is represented by three such proteins out of which LGI1 is most prevalent in limbic encephalitis. This entity is characterized by monophasic presentation with acute or subacute onset memory loss, confusion, seizures and psychiatric symptoms. The presence of anti LGI1 antibodies in serum or CSF confirms the diagnosis. We hereby report a case of a 65 year old woman with 8 months history of peripheral neuropathy followed by memory impairment, focal seizures with behavioural and psychiatric changes. No tumour was found on imaging and the classic paraneoplastic panel was negative. However, she was found to be positive for Antibody LGI1 proving autoimmune basis of her illness which responded majorly to immuno-modulatory therapy in the form of high dose steroids.

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“…We consider that the patient's mental alteration is a consequence of the disease rather than any adverse effect of steroid therapy. Wecould not rule out the possibility that this patient was suffering from PLE, because paraneoplastic symptoms sometimesprecede the appearance of any evident neoplasm (3). Using immunoblotting we subsequently analyzed the CSFderived from the patient to confirm whether or not there were antibodies that recognized the CNScomponents as in a previous report (4) (Fig.…”

Section: Case Reportmentioning

confidence: 93%

Steroid-Responsive Limbic Encephalitis

et al. 2003

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anti-white matter antibodies that mainly recognized astrocytes. Intravenous steroid followed by oral steroid reduced the symptoms to a remarkable degree. The patient has now been successfully sustained with steroid for more than two years. Weconsidered that this case is classified as non-paraneoplastic limbic encephalitis, and acquired autoimmunity played a major role in the pathogenesis of this case. (Internal Medicine 42: 428-432, 2003)

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“Limbic Encephalitis” and Its Association With Carcinoma

Cited by 441 publications

References 0 publications

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

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

A Case of Limbic Encephalitis: Antibody LGI1 Associated Encephalitis

Steroid-Responsive Limbic Encephalitis

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