Limbic encephalitis associated with AMPA receptor and CRMP5 antibodies: A case report and literature review

Jia, Yongping; Wang, Jie; Xue, Lanping; Hou, Yuli

doi:10.1002/brb3.1528

Cited by 17 publications

(11 citation statements)

References 24 publications

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“…This is likely related to the frequent presence of concurrent paraneoplastic antibodies and associated mechanisms, particularly CRMP5 autoimmunity. 34 , 35 In a series of 22 patients with anti-AMPAR encephalitis not focused on thymoma, a shorter survival was observed in patients with concurrent onconeural antibodies compared with those harboring isolated AMPAR antibodies. 19 …”

Section: Discussionmentioning

confidence: 99%

Thymoma and Autoimmune Encephalitis

Guasp

Landa

Martínez‐Hernández

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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ObjectiveTo report the clinical, neuroimaging, and antibody associations in patients with autoimmune encephalitis (AE) and thymoma.MethodsA retrospective cohort study of 43 patients was conducted. Antibody determination and immunoprecipitation to characterize novel antigens were performed using reported techniques.ResultsPatients' median age was 52 years (range: 23–88 years). Forty (93%) had neuronal surface antibodies: gamma-aminobutyric acid receptor A (GABAAR) (15), amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) (13), contactin-associated protein-like 2 (CASPR2) (4), leucine-rich, glioma inactivated 1 (LGI1) (3), glycine receptor (GlyR) (3), and unknown antigens (2). Concurrent antibodies against intracellular antigens occurred in 13 (30%; 9 anti–collapsin response mediator protein 5 [CRMP5]) and were more frequent in anti-AMPAR encephalitis (54% vs 20%; p = 0.037). The most common clinical presentation was encephalitis with multiple T2/fluid-attenuated inversion recovery hyperintense lesions in 23 (53%) patients (15 GABAAR, 5 AMPAR, and 1 unknown neuropil antibody), followed by encephalitis with peripheral nerve hyperexcitability in 7 (16%; 4 CASPR2, 2 LGI1, and 1 unknown antibody), limbic encephalitis in 6 (14%; 4 AMPAR, 1 LGI1, and 1 antibody negative), progressive encephalomyelitis with rigidity and myoclonus in 4 (9%; 3 GlyR and 1 AMPAR antibodies), and encephalitis with normal MRI in 3 (7%; AMPAR antibodies). Anti-GABAAR encephalitis was more prevalent in Japanese patients compared with Caucasians and other ethnicities (61% vs 16%; p = 0.003). In anti-AMPAR encephalitis, 3/4 patients with poor and 0/6 with good outcome had concurrent CRMP5 antibodies (p = 0.033). Immunoprecipitation studies identified metabotropic glutamate receptor 3 antibodies that were additionally found in 5 patients (3 with and 2 without encephalitis).ConclusionsAE in patients with thymoma include several clinical-radiologic syndromes that vary according to the associated antibodies. Anti-GABAAR encephalitis was the most frequent AE and occurred more frequently in Japanese patients.

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Section: Discussionmentioning

confidence: 99%

Thymoma and Autoimmune Encephalitis

Guasp

Landa

Martínez‐Hernández

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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“…Rarely, ≥2 autoantibodies coexist in the same patient. Patients with LE having double or triple antibodies have worse outcomes than those with single antibody because of a higher chance of concomitant tumors [ 27 , 28 , 29 ].…”

Section: Antibodiesmentioning

confidence: 99%

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

Kao

Lin²,

Weng

et al. 2020

IJMS

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Limbic encephalitis (LE) is a rare cause of encephalitis presenting as an acute and subacute onset of neuropsychiatric manifestations, particularly with memory deficits and confusion as core features, along with seizure occurrence, movement disorders, or autonomic dysfunctions. LE is caused by neuronal antibodies targeting the cellular surface, synaptic, and intracellular antigens, which alter the synaptic transmission, especially in the limbic area. Immunologic mechanisms involve antibodies, complements, or T-cell-mediated immune responses in different degree according to different autoantibodies. Sensitive cerebrospinal fluid markers of LE are unavailable, and radiographic findings may not reveal a typical mesiotemporal involvement at neurologic presentations; therefore, a high clinical index of suspicions is pivotal, and a neuronal antibody testing is necessary to make early diagnosis. Some patients have concomitant tumors, causing paraneoplastic LE; therefore, tumor survey and treatment are required in addition to immunotherapy. In this study, a review on the molecular and immunologic aspects of LE was conducted to gain awareness of its peculiarity, which we found quite different from our knowledge on traditional psychiatric illness.

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“…The presence of psychiatric symptoms and concurrent onco-neuronal antibodies were associated with poorer outcomes while younger age and confusion at presentation were linked with favorable prognosis [ 5 , 7 ]. This was also observed in patients of anti-AMPAR encephalitis with concurrent CRMP5 antibodies [ 26 ]. Additionally, fulminant encephalitis was associated with a poor prognosis.…”

Section: Discussionmentioning

confidence: 56%

Clinical characteristics and prognosis of anti-alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic acid receptor encephalitis

et al. 2021

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Background Encephalitis associated with antibodies against alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) is an extremely rare type of antibody-mediated encephalitis. This research aims to investigate the clinical characteristics and prognosis of anti-AMPAR encephalitis. Methods This retrospective study enrolled nine patients with anti-AMPAR encephalitis. Demographic information, clinical manifestations, laboratory and radiological findings, treatment and response were collected and analyzed. These patients were followed up with an average period of 72 weeks to gather prognostic information. Results Nine patients (7 females and 2 males) were enrolled with a mean age at disease onset of 59 years old. Three clinical pictures, including limbic encephalitis (n = 7; 78%), pure amnesia (n = 1; 11%) and fulminant encephalitis (n = 1; 11%) were identified. New symptoms of dysphagia and deafness were identified in the clinical spectrum of anti-AMPAR encephalitis. All patients had positive blood AMPAR antibodies, and six of them (67%) had paired positive antibodies in cerebrospinal fluid (CSF). Brain magnetic resonance imaging (MRI) was abnormal in 75% of the patients with no specific patterns recognized. Six patients (67%) had tumors, including lung cancers and thymomas. After immunotherapy and oncotherapy, partial improvement of neurological symptoms was observed among all 6 patients with available records during their hospitalization. After a mean follow-up of 72 weeks, 3 patients had marked decrease of modified Rankin Scale (mRS) score, 1 patient had unchanged mRS score, 4 patients died and the other one was lost. Conclusions Anti-AMPAR encephalitis mainly presents as limbic encephalitis, and is paraneoplastic in 67% of cases. Thus, intensive screening for tumors is recommended for all anti-AMPAR patients. Although patients showed a good short-term therapeutic response, the overall prognosis was not satisfactory.

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Limbic encephalitis associated with AMPA receptor and CRMP5 antibodies: A case report and literature review

Cited by 17 publications

References 24 publications

Thymoma and Autoimmune Encephalitis

Thymoma and Autoimmune Encephalitis

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

Clinical characteristics and prognosis of anti-alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic acid receptor encephalitis

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