The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies

Irani, Sarosh R.; Vincent, Angela

doi:10.1590/s0004-282x2012000400015

Cited by 24 publications

(15 citation statements)

References 26 publications

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“…Approximately 31.70% of patients in this study rst showed FBDS, 67.50% EEG showed slow waves, focal discharges, temporal origins were more common, and even rare three-phase waves and the whole brain low voltage; 10.00% showed fast Increased activity; 10.00% normal EEG; 2.50% manifested as sharp wave delivery; it has been reported [9] 60% of patients with head MRI showed bilateral temporal lobe high signal, 40% is abnormal signal in one hippocampus or normal, our The report is generally consistent with the above.…”

Section: Discussionsupporting

confidence: 57%

Clinical Characteristics and Prognostic Analysis of Anti-LGI 1 Receptor Encephalitis in Northeast China

Zhao

et al. 2020

Preprint

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Abstract Background: To investigate the clinical characteristics and prognosis of Anti-LGI1 receptor encephalitis.Methods: This retrospective study enrolled forty -one patients with anti LGI1 receptor encephalitis diagnosed for the first time in the Department of Neurology, the first hospital of Jilin University from June 2015 to November 2019. Results: There are twenty-two males and nineteen females, with a median age of fifty-four years. The first symptom of male patients showed cognitive impairment in nine (40.91%) cases,facial muscle dystonia in six (27.27%) cases.Of the female patients,seven (36.84%)patients had facial-arm dystonia-like seizures,six (31.58%) patients had cognitive impairment.seven (18.42%) patients had abnormal tumor markers,but no tumor was found in all patients.Thirty patients (73.17%) had hyponatremia with a minimum of 115.4 mmol/L.Except for one patient,eighteen (45.00%) patients had normal cerebrospinal fluid indicators,twenty-two (55.00%) patients had abnormal cerebrospinal fluid indicators,Eleventh (50.00%) patients had elevated protein,and nine (40.91%) patients had elevated IgG,six (27.27%) patientys of white blood cells increased,and four (18.18%) patients of increased pressure. Except for four patients,twenty-two (59.46%) patients had abnormal signals of hippocampus and temporal lobe,seventeen (77.27%) of them had bilateral abnormal signals of hippocampus,five(22.73%)cases had unilateral abnormal signals of hippocampus;Eleventh(29.73%)had lacunar Cerebral infarction and ischemic focus;Two(5.41%)cases of frontal and insular abnormal signals;one(2.70%)case of temporal and occipital abnormal signals;one(2.70%) case of white matter lesions. Except for one patient,the EEG showed twenty-seven(67.50%)cases of slow waves and focal discharges,(six(22.22%) of which were left-temporal spiked slow waves,and five (18.52%) were of right-temporal spikes Slow wave,four (14.81%) cases with double temporal sharpened slow wave,six (22.22%) cases with frontotemporal sharpened slow wave, one (3.70%) case with frontal sharpened slow wave,one(3.70%)case with Three-phase waves; In addition,four(10.00%)cases of fast activity increased;three (7.50%) cases of background α generalization). Patients received first-line immunotherapy except six patients who gave up treatment. Conclusions: Anti-LGI1 receptor encephalitis is more common in middle-aged and older people. In male,the first manifestation is cognitive impairment, and the female is more common with the facial-arm dystonia-like seizures,and rarely can be manifested as discharge sensation, hallucinations and autonomic symptoms.lumbar puncture pressure increased more than decreased;protein was significantly higher than leukocytes;EEG showed slow waves and focal discharges were more common, and abnormal background and normal EEG can occur.MRI shows that hippocampus and temporal lobe abnormal signal have the highest proportion.There is no significant difference in the prognosis of hormones, globulin,or both.

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Section: Discussionsupporting

confidence: 57%

Clinical Characteristics and Prognostic Analysis of Anti-LGI 1 Receptor Encephalitis in Northeast China

Zhao

et al. 2020

Preprint

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“…An accurate and early diagnosis is essential because both types of encephalitis may be fatal. Prognosis in both cases depends on the early institution of specific treatment, acyclovir in the case of HE, and immunotherapy or treatment of an underlying neoplasm in AE . Further knowledge on the clinical‐radiological differences and similarities between HE and AE may help in early clinical decisions.…”

Section: Introductionmentioning

confidence: 99%

Limbic encephalitis: a clinical‐radiological comparison between herpetic and autoimmune etiologies

Oyanguren

Sánchez

González

et al. 2013

Euro J of Neurology

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“…3,6,7 These neuronal- or glial-surface–directed antibodies8 are detected by demonstration of antibody binding to the surface of human embryonic kidney cells that have been made to express the specific target (Fig 1A–C), to the surface of cultured live neurons (Fig 1D), and to fixed brain sections (see Fig 1E; these techniques have different merits and will be discussed later) 3,7,9. The clinical syndromes associated with neuroglial surface-directed antibodies (NGSAbs) frequently show a rapid onset, response to immunotherapies, good correlations between symptom course and antibody levels, and are less frequently associated with malignancies 2,7,10,11…”

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confidence: 99%

Cell‐surface central nervous system autoantibodies: Clinical relevance and emerging paradigms

Irani

Gelfand

Al-Diwani

et al. 2014

Annals of Neurology

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166

149

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The recent discovery of several potentially pathogenic autoantibodies has helped identify patients with clinically distinctive central nervous system diseases that appear to benefit from immunotherapy. The associated autoantibodies are directed against the extracellular domains of cell-surface–expressed neuronal or glial proteins such as LGI1, N-methyl-D-aspartate receptor, and aquaporin-4. The original descriptions of the associated clinical syndromes were phenotypically well circumscribed. However, as availability of antibody testing has increased, the range of associated patient phenotypes and demographics has expanded. This in turn has led to the recognition of more immunotherapy-responsive syndromes in patients presenting with cognitive and behavioral problems, seizures, movement disorders, psychiatric features, and demyelinating disease. Although antibody detection remains diagnostically important, clinical recognition of these distinctive syndromes should ensure early and appropriate immunotherapy administration. We review the emerging paradigm of cell-surface–directed antibody–mediated neurological diseases, describe how the associated disease spectrums have broadened since the original descriptions, discuss some of the methodological issues regarding techniques for antibody detection and emphasize considerations surrounding immunotherapy administration. As these disorders continue to reach mainstream neurology and even psychiatry, more cell-surface–directed antibodies will be discovered, and their possible relevance to other more common disease presentations should become more clearly defined.

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The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies

Cited by 24 publications

References 26 publications

Clinical Characteristics and Prognostic Analysis of Anti-LGI 1 Receptor Encephalitis in Northeast China

Clinical Characteristics and Prognostic Analysis of Anti-LGI 1 Receptor Encephalitis in Northeast China

Limbic encephalitis: a clinical‐radiological comparison between herpetic and autoimmune etiologies

Cell‐surface central nervous system autoantibodies: Clinical relevance and emerging paradigms

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