Patient characteristics and outcome associations in AMPA receptor encephalitis

Laurido-Soto, Osvaldo; Brier, Matthew R.; Simón, L; McCullough, Austin; Bucelli, Robert C.; Day, Gregory S.

doi:10.1007/s00415-018-9153-8

Cited by 67 publications

(64 citation statements)

References 54 publications

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“…In a case series, the sparing of the primary motor cortex occurred in half of the abnormal FDG‐PET scans of limbic encephalitis (Masangkay, Basu, Moghbel, Kwee, & Alavi, ). Bilateral motor cortex sparing was also observed in the FDG‐PET of a case of anti‐AMPAR encephalitis (Laurido‐Soto et al, ). In our patients, cortical hypometabolism also did not include primary motor cortex.…”

Section: Discussionmentioning

confidence: 82%

Different FDG‐PET metabolic patterns of anti‐AMPAR and anti‐NMDAR encephalitis: Case report and literature review

Wei

Tseng

et al. 2020

Brain and Behavior

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Introduction 18F‐fluorodeoxyglucose (FDG)‐PET metabolic patterns of brain differ among autoimmune encephalitis with different neuronal surface antigens. In this case report, we compared the topographical relationship of cerebral glucose metabolism and antigen distribution in the patients with anti‐NMDAR and anti‐AMPAR encephalitis. Literature review summarized the common features of brain metabolism of autoimmune encephalitis. Methods The cerebral glucose metabolism was evaluated by FDG‐PET/CT during acute‐to‐subacute stage of autoimmune encephalitis and after treatment. The stereo and quantitative analysis of cerebral metabolism used standardized z‐score and visualized on three‐dimensional stereotactic surface projection. To map NMDAR and AMPAR in human brain, we adopted genetic atlases from the Allen Institute and protein atlases from Zilles's receptor densities. Results The three‐dimensional stereotactic surface projection displayed frontal‐dominant hypometabolism in a 66‐year‐old female patient with anti‐AMPAR encephalitis and occipital‐dominant hypometabolism in a 29‐year‐old female patient with anti‐NMDAR encephalitis. Receptor density maps revealed opposite frontal–occipital gradients of AMPAR and NMDAR, which reflect reduced metabolism in the correspondent encephalitis. FDG‐PET hypometabolic areas possibly represent receptor hypofunction with spatial correspondence to receptor distributions of the autoimmune encephalitis. The reversibility of hypometabolism was in line with patients' cognitive improvement. The literature review summarized six features of metabolic anomalies of autoimmune encephalitis: (a) temporal hypermetabolism, (b) frontal hypermetabolism and (c) occipital hypometabolism in anti‐NMDAR encephalitis, (d) hypometabolism in association cortices, (e) sparing of unimodal primary motor cortex, and (e) reversibility in recovery. Conclusions The distinct cerebral hypometabolic patterns of autoimmune encephalitis were representative for receptor hypofunction and topographical distribution of antigenic receptors. The reversibility of hypometabolism marked the clinical recovery of autoimmune encephalitis and made FDG‐PET of brain a valuable diagnostic tool.

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Section: Discussionmentioning

confidence: 82%

Different FDG‐PET metabolic patterns of anti‐AMPAR and anti‐NMDAR encephalitis: Case report and literature review

Wei

Tseng

et al. 2020

Brain and Behavior

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“…The fourth case was a 44‐year‐old man with thymoma who was treated with thymectomy, methylprednisolone, IVIg, and rituximab. Finally, he successfully returned to work as a business manager (Laurido‐Soto et al, ). In contrast to the other four cases, our patient was a 26‐year‐old male who was diagnosed with suspected lymphoma.…”

Section: Discussionmentioning

confidence: 99%

“…His results showed that the mortality rate of anti‐AMPAR encephalitis was 16% (9/55). While tumor was identified in 67% cases (Laurido‐Soto et al, ). Unfortunately, there are no similar studies on anti‐CRMP5 encephalitis.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2020

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Aims AMPA receptor (AMPAR) and CRMP5 antibodies are relatively uncommon in limbic encephalitis, and patients with both antibodies are rare. We recently treated such a patient, but the patient died after active treatment. To further understand this disease, we conducted a case report and literature review. Discussions To date, five encephalitis patients, including our patient, have been found to be positive for AMPAR and CRMP5 antibodies. The male‐to‐female ratio of the reported cases is 4:1, and the age range is 26 and 62 years old. All five patients presented with various neuropsychiatric symptoms, including insomnia, abnormal behavior, seizures, extrapyramidal symptoms, and autonomic dysfunction. Four patients had tumors (three invasive thymomas and one suspected lymphoma), and three cases died within a short period of time. No tumor was detected in one of the patients during the follow‐up period; however, after active treatment, the outcome was poor, and the patient developed cachexia. One patient had good response to immunotherapy and tumor therapy and successfully returned to work. Conclusions The prognosis of encephalitis associated with AMPAR and CRMP5 antibodies is worse than that of the encephalitis associated with AMPAR antibodies alone. The most likely cause is that this encephalitis is more likely to be accompanied by malignant tumors, leading to a poor prognosis. In addition, it may also be due to some synergistic mechanisms between the two antibodies. Further studies aimed at the prognosis of this type of encephalitis are warranted.

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“…These antibodies bind to the α1 subunit of glutamate receptors. Anti-glutamate receptor antibody-positive encephalitis patients have been considered variants associated with progressive encephalomyelitis, stiffness, myoc- [20,23,28,36] LGI1 [7,11] Caspr2 [7,16] AMPAR [37,39,48] GABAR [40][41][42][43] Glutamate R [45,46,49 lonus, and other symptoms of stiff-person syndrome. However, these diseases are characterized by stiff stimulus-sensitive spasms, myoclonus, autonomic dysfunction, and brainstem lesions [45].…”

Section: Anti-glutamate Receptor Encephalitismentioning

confidence: 99%

Neuronal Surface Antibody Syndrome: A Review of the Characteristics of the Disease and Its Association with Autoantibodies

Li¹,

Zhang²,

Liu³

et al. 2020

Neuroimmunomodulation

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Several studies have certified that autoantibodies play an important role in the manifestation of neuromuscular diseases. Scientists have discovered specific neuronal tumor antibodies in patients with typical paraneoplastic neurological disorders. But in some clinical cases, it is not useful to cure this disease with common treatments unless the autoantibodies are addressed. In addition, recent studies have shown a close relationship between certain antibodies and neuronal surface proteins in some special cases. These antibodies, which act on the surface of neurons, mainly include voltage-gated calcium channel (VGKC) antibodies. VGKC antibodies are further divided into several types including antileucine-rich glioma inactivated 1 (LGI1), anti-contactin-associated protein-like 2 (Caspr2), anti-N-methyl-D-aspartate receptor (NMDAR), anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), anti-γ-aminobutyric acid receptor (GABAR), and glycine receptor. For the purpose of this review, cases of clinical studies of autoantibody-associated encephalitis were collected, the key points regarding the pathogenesis were summarized, the clinical manifestation was discussed, and all this information was organized as this review in order to introduce the relationship between autoantibodies and autoimmune encephalitis. Furthermore, it is hoped that it can effectively direct the development of diagnostic and therapeutic approach in the future.

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Patient characteristics and outcome associations in AMPA receptor encephalitis

Cited by 67 publications

References 54 publications

Different FDG‐PET metabolic patterns of anti‐AMPAR and anti‐NMDAR encephalitis: Case report and literature review

Different FDG‐PET metabolic patterns of anti‐AMPAR and anti‐NMDAR encephalitis: Case report and literature review

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

Neuronal Surface Antibody Syndrome: A Review of the Characteristics of the Disease and Its Association with Autoantibodies

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