Limbic Encephalitis Associated With Antibodies to the Nmda Receptor in Hodgkin Lymphoma

Zandi, Michael S; Irani, Sarosh R.; Follows, George; Moody, A.M.; Molyneux, Pd; Vincent, Angela

doi:10.1212/wnl.0b013e3181c55e9b

Cited by 65 publications

(37 citation statements)

References 5 publications

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“…Other known tumour associations include mediastinal teratomas and sex-cord stromal tumours [1] and there have been reported links with neuroblastomas [9] and Hodgkin's lymphoma [10]. We also found a report of a possible link with the TdaP-IPV vaccine (for tetanus, diphtheria, pertussis and polio), with the patient having a prodromal illness 24 hours post vaccine and classical psychiatric symptoms 5 weeks later [11].…”

Section: Epidemiologysupporting

confidence: 56%

Anti-NMDA Receptor Encephalitis

Ferdinand¹

2013

J Clin Cell Immunol

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In recent years, the discovery of antibodies to specific neuronal antigens that then go on to cause encephalitis has gone a long way to change the investigation and management of a potential encephalitic process. These have now become known under the umbrella term of the 'autoimmune encephalitides'. In this article we look at anti-N-methyl-D-aspartate receptor encephalitis, a condition most often found in young females and has an association with a number of malignancies, most commonly ovarian teratomas. Most patients will have a viral prodrome, followed by psychiatric, seizure, dysautonomic and dyskinetic features, but can present at any point along this pathway. Treatment involves prompt tumour identification and removal where appropriate and initiation of immunosuppressive therapy, usually commencing with corticosteroids. A substantial proportion of patients will make a full recovery, but many will need medical, psychiatric and social care following completion of the acute phase of the illness.

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Section: Epidemiologysupporting

confidence: 56%

Anti-NMDA Receptor Encephalitis

Ferdinand¹

2013

J Clin Cell Immunol

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“…Serum NMDAR antibody titers were shown to fall in patients who showed clinical improvement. Other tumors have been noted in individual patients, including a teratoma of the mediastinum [1•], teratoma of the testicle [7], sex cord-stromal tumor [6••], Hodgkin's lymphoma [8], small cell lung carcinoma [9], and neuroblastoma [10].…”

Section: Paraneoplastic Originsmentioning

confidence: 99%

“…Although contemporary cases of EL have probably morphed from the classical (von Economo-like) descriptions, it is clear that NMDAR encephalitis is not a new clinical entity [25]. A few studies have reported cases with high medial temporal lobe signal and a clinical diagnosis of limbic encephalitis, in association with NMDAR antibodies [8,9,26]. However, VGKC-complex antibodies are much more common in this clinical context [27,28].…”

Section: Encephalitis Lethargica Limbic Encephalitis and Other Encementioning

confidence: 99%

NMDA Receptor Antibody Encephalitis

Irani

Vincent

2011

Curr Neurol Neurosci Rep

100

105

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N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is a recently described immunotherapy-responsive panencephalitis with characteristic features that include a psychiatric onset and a later movement disorder. This entity was first described as a paraneoplastic phenomenon in young women with ovarian teratomata. However, more recently it has become clear that the majority of patients, particularly children, do not harbor a tumor and that males can also be affected. With the development of the NMDAR antibody assay, now available worldwide, a few patients with classical limbic encephalitis and early psychosis and epilepsy have also been found to harbor these antibodies. Early diagnosis followed by immunotherapies and tumor removal, when relevant, expedite recovery from the condition. Antibody levels correlate with the clinical severity of the disease in individual patients, and the antibodies have been shown to substantially reduce NMDA receptors on hippocampal neurons both in vitro and in vivo, supporting the likely direct pathogenicity of the NMDAR antibodies.

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“…18,20 The diagnosis is supported by the presence of a CSF pleocytosis or oligoclonal bands, the presence of the anti-NMDA receptor in the CSF, electroencephalogram (EEG) findings of slowed activity, and typical contrast-enhanced MRI findings. 19 In the case of PNS, successful tumor treatment in addition to steroids, Intravenous immunoglobulin (IVIG), or plasma exchange tends to resolve the neuropsychiatric symptoms.…”

Section: Limbic Encephalitismentioning

confidence: 99%

Paraneoplastic Syndromes in Children with Hodgkin Lymphoma

Betcherman¹,

Punnett²

2017

Oncology &Amp; Hematology Review (US)

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Paraneoplastic syndromes (PNS) refer to a phenomenon whereby certain malignancies manifest as symptoms not directly related to the tumor itself. PNS has been described in association with Hodgkin lymphoma (HL) in adults and children and may affect a number of organ systems. The pathophysiology is variable and in many cases is not well understood. Specific paraneoplastic antibodies have been isolated in some syndromes, though are not required for the diagnosis. The two best described for HL are the anti-Tr and anti-mGluR5 antibodies identified in some cases of limbic encephalitis and cerebellar degeneration respectively. A high index of suspicion for underlying malignancy is necessary when recognizing these clinical syndromes to avoid a diagnostic delay. Successful treatment of the HL often reverses the manifestations of PNS although organ function at diagnosis may limit therapeutic options. Some patients suffer devastating complications of their PNS.

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Limbic Encephalitis Associated With Antibodies to the Nmda Receptor in Hodgkin Lymphoma

Cited by 65 publications

References 5 publications

Anti-NMDA Receptor Encephalitis

Anti-NMDA Receptor Encephalitis

NMDA Receptor Antibody Encephalitis

Paraneoplastic Syndromes in Children with Hodgkin Lymphoma

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