Clinical Relapse of Anti-AMPAR Encephalitis Associated with Recurrence of Thymoma

Omi, Tsubasa; Kinoshita, Makoto; Nishikawa, Akira; Tomioka, Takahito; Ohmori, Ken; Fukada, Kei; Matsunaga, Hidenori

doi:10.2169/internalmedicine.9682-17

Cited by 19 publications

(18 citation statements)

References 6 publications

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“…In 2009, Lai [7] first reported three patients with anti-AMPAR encephalitis, and thymoma were identified among 109 cases of limbic encephalitis. To date, eleven cases of thymoma-associated anti-AMPAR2 encephalitis have been described in six publications (Table 1) [6,7,[9][10][11][12]. In most cases, thymoma was detected in oncological screening with the first episode of encephalitis, though a malignant thymoma treated with radiotherapy and chemotherapy six years prior to encephalitis was found in one case [9].…”

Section: Discussionmentioning

confidence: 99%

“…Our case was unique in that the patient initiated with the symptom of myasthenia gravis and thymoma two years prior to AE, and a complete thymectomy was performed before AE onset without recurrence of the thymoma when AE occurred. Recently, a patient with anti-AMPAR encephalitis who was reported to be in remission for 34 months showed clinical relapse three months after the detection of recurrent thymoma [11]. Hor et al reported a case of thymoma-associated myasthenia gravis and LGI1encephalitis with nephrotic syndrome after thymectomy [13].…”

Section: Discussionmentioning

confidence: 99%

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Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

2019

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Background: Autoimmune encephalitis (AE) is a newly recognized autoimmune disorders in which the targets are proteins or receptors involved in synaptic transmission and neuronal excitability. α-amino-3-hydroxy-5-methyl-4isoxazolepropionic acid receptor (AMPAR) is a subtype of glutamate receptor that mediates most of the fast excitatory neurotransmission in the brain. Case presentation: A 50-year-old woman presented with subacute onset of memory loss and behavioral changes. High levels of serum (1:1000) and CSF (1:32) antibodies against the AMPAR GluR2 were detected. A wide range of abnormalities in 6-8 Hz low to middle slow waves was found by electroencephalographs, and high-intensity signals on fluid-attenuated inversion recovery in both the medial temporal lobe and hippocampus were identified on brain magnetic resonance images. This patient presented with myasthenia gravis and type B2 thymoma (World Health Organization Thymoma Classification) at age 48. This case was unique in that the patient initiated with the symptom of myasthenia gravis and thymoma two years prior to encephalitis, and a complete thymectomy was performed before AE onset without recurrence of the thymoma when encephalitis occurred. Conclusions: Thymoma was reported to be associated with paraneoplastic neurological disease. This is the first time a thymectomy has been applied in a myasthenia gravis patient with thymoma two years prior to the onset of anti-AMPAR2 encephalitis. This case highlights the complexity of autoimmune encephalitis associated with thymoma.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

2019

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“…Our case was unique in that the patient initiated with the symptom of myasthenia gravis and thymoma two years prior to AE, and a complete thymectomy was performed before AE onset without recurrence of the thymoma when AE occurred. Recently, a patient with anti-AMPAR encephalitis who was reported to be in remission for 34 months showed clinical relapse three months after the detection of recurrent thymoma (11). Our case raises the questions of whether the autoantigens could be expressed by the thymus after thymectomy, and how these autoantigens from thymic tumors could trigger immune disorders.…”

Section: Discussionmentioning

confidence: 77%

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

Luo

Wú

Huang

2019

Preprint

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Background: Autoimmune encephalitis (AE) is a newly recognized autoimmune disorders in which the targets are proteins or receptors involved in synaptic transmission and neuronal excitability. α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) is a subtype of glutamate receptor that mediates most of the fast excitatory neurotransmission in the brain. Case presentation: A 50-year-old woman presented with subacute onset of memory loss and behavioral changes. High levels of serum (1:1000) and CSF (1:32) antibodies against the AMPAR GluR2 were detected. A wide range of abnormalities in 6-8 Hz low to middle slow waves was found by electroencephalographs, and high-intensity signals on fluid-attenuated inversion recovery in both the medial temporal lobe and hippocampus were identified on brain magnetic resonance images. This patient presented with myasthenia gravis and type B2 thymoma (World Health Organization Thymoma Classification) at age 48. This case was unique in that the patient initiated with the symptom of myasthenia gravis and thymoma two years prior to encephalitis, and a complete thymectomy was performed before AE onset without recurrence of the thymoma when encephalitis occurred. Conclusions: Thymoma was reported to be associated with paraneoplastic neurological disease. This is the first time a thymectomy has been applied in a myasthenia gravis patient with thymoma two years prior to the onset of anti-AMPAR2 encephalitis. This case highlights the complexity of autoimmune encephalitis associated with thymoma. Keywords: Autoimmune encephalitis, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, thymoma, myasthenia gravis.

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“…Neuropatias periféricas são descritas em seres humanos, secundárias a teratomas, câncer de ovário e mama, carcinoma pulmonar de pequenas células e timoma [9,26,28], entretanto são raros os relatos na medicina veterinária [4,8]. Em cães com neuropatia periférica paraneoplásica, os sinais relatados são mais relacionados às polineuropatias destacando-se tetraparesias com diminuição dos reflexos espinhais e atrofia muscular em associação ao comprometimento motor de nervos cranianos, tais como o trigêmeo, facial, glossofaríngeo e vago [8].…”

Section: Discussionunclassified

Focal Peripheral Neuropathy Associated with Lymphoma in Dogs

Silva

Lavalle

Martins

et al. 2021

Acta Scientiae. Vet.

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Background: Peripheral neuropathies result in sensory, motor or autonomic dysfunctions due to impairment of peripheral spinal or cranial nerves. Neoplasms such as lymphoma are cited as one of the many aetiological causes and it may affect the nerve directly, by compression, or indirectly, or paraneoplastic, by remote action of the neoplasm located in an extra-neural site. This study aimed to report two cases of cranial nerve neuropathy (trigeminal and facial) associated with canine lymphoma, contributing to a better understanding of its paraneoplastic effects on the nervous system, as well as the diagnosis and treatment of these conditions.Cases: Two cases of canine lymphoma associated with possible signs of paraneoplastic peripheral neuropathy were attended at the Veterinary Hospital from the Universidade Federal de Minas Gerais (HV UFMG). Case 1. A spayed mixed breed bitch, with lethargy and unilateral exophthalmos. Brain computed tomography revealed a retrobulbar mass and cytology was diagnostic for extranodal lymphoma. Subsequent to computed tomography, the dog was presented with hypotrophy of the facial musculature and difficulty in grasping food, consistent with trigeminal nerve palsy, which resolved after institution of the 19-week chemotherapy protocol from the University of Wisconsin. Nevertheless, disease reccurred and a rescue protocol was initiated. Case 2. A female Dalmatian, spayed, was diagnosed with multicentric lymphoma, after cytology of the left mandibular lymph node. Chemotherapy was initiated with the same protocol of the previous case. However, the disease progressed and it was observed facial asymmetry with ptosis of the left eyelid, pina and lips, in addition to difficulty in grasping food, suggesting facial and trigeminal cranial nerve palsy. Clinical signs resolved after institution of a rescue chemotherapy protocol. However, in both cases, disease progression and poor clinical condition resulted in decision of euthanasia and necropsy was not authorized.Discussion: Canine lymphoma is often associated with paraneoplastic syndromes, with neuropathy being one of its possible clinical manifestations. In spite of that its pathogenesis remains unclear, with little information in the veterinary literature. Diagnosis is challenging and must be initially based on recognition of neurological clinical signs and lesion localization, as in the reported cases with lesions located on the fifth and seventh cranial nerves. In the patient from the first case, the absence of clinical signs and laboratory abnormalities suggestive of endocrinopathies, associated with neurological signs restricted to the trigeminal nerve, bilaterally, before starting chemotherapy and without the identification of brain lesions in computed tomography, suggested paraneoplastic involvement as the cause of neuropathy. In the second case described, the absence of clinical signs and laboratory abnormalitiess suggestive of endocrinopathies or nutritional deficiencies, associated with neurological signs restricted to the facial and trigeminal cranial nerves, suggested direct or indirect tumour involvement. Both cases showed improvement of neurological clinical signs after chemotherapy which favored the therapeutic diagnosis. Nevertheless, failure to authorize necropsy of patients made it impossible to confirm that peripheral neuropathy is secondary to the remote effect of lymphoma.

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Clinical Relapse of Anti-AMPAR Encephalitis Associated with Recurrence of Thymoma

Cited by 19 publications

References 6 publications

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

Focal Peripheral Neuropathy Associated with Lymphoma in Dogs

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