Anti-Ma and anti-Ma2-associated paraneoplastic neurological syndromes

Suero, G. Ortega; Solà-Valls, Núria; Escudero, Domingo; Sáiz, Albert; Graus, Francesc

doi:10.1016/j.nrleng.2016.05.004

Cited by 35 publications

(32 citation statements)

References 31 publications

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“…Antibody screening targets were GAD65, onconeural autoantibodies with reported positive cases in the context of LE [1,28,29,33,[36][37][38]46], and neuronal surface membrane auto-antibodies. In total, 59 patients (56%) had no antibodies present in their CSF and PB, and were thus classified as antibody negative (AB−); 41 patients (38%) showed autoantibodies against intracellular antigens (IAB+).…”

Section: Le Patient Subgroupsmentioning

confidence: 99%

Specific B- and T-cell populations are associated with cognition in patients with epilepsy and antibody positive and negative suspected limbic encephalitis

et al. 2020

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Objective Neuropsychological impairments are major symptoms of autoimmune limbic encephalitis (LE) epilepsy patients. In LE epilepsy patients with an autoimmune response against intracellular antigens as well as in antibody-negative patients, the antibody findings and magnetic resonance imaging pathology correspond poorly to the clinical features. Here, we evaluated whether T- and B-cells are linked to cognitive impairment in these groups. Methods In this cross-sectional, observational, case–controlled study, we evaluated 106 patients with adult-onset epilepsies with a suspected autoimmune etiology. We assessed verbal and visual memory, executive function, and mood in relation to the presence or absence of known auto-antibodies, and regarding T- and B-cell activity as indicated by flow cytometry (fluorescence-activated cell sorting = FACS, peripheral blood = PB and cerebrospinal fluid = CSF). Results 56% of the patients were antibody-negative. In the other patients, auto-antibodies were directed against intracellular antigens (GAD65, paraneoplastic: 38%), or cellular surface antigens (LGI1/CASPR2/NMDA-R: 6%). Excluding LGI1/CASPR2/NMDA-R, the groups with and without antibodies did not differ in disease features, cognition, or mood. CD4+ T-cells and CD8+ T-cells in blood and CD4+ T-cells in CSF were prominent in the auto-antibody positive group. Regression analyses indicated the role education, drug load, amygdala and/or hippocampal pathology, and CD4+ T-cells play in verbal memory and executive function. Depressed mood revealed no relation to flow cytometry results. Conclusion Our results indicate a link between T- and B-cell activity and cognition in epilepsy patients with suspected limbic encephalitis, thus suggesting that flow cytometry results can provide an understanding of cognitive impairment in LE patients with autoantibodies against intracellular antigens.

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Section: Le Patient Subgroupsmentioning

confidence: 99%

Specific B- and T-cell populations are associated with cognition in patients with epilepsy and antibody positive and negative suspected limbic encephalitis

et al. 2020

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“…The majority of patients with the Ma2 antibody develop paraneoplastic limbic encephalitis or brainstem dysfunction. 64,65 Hypothalamic involvement with somnolence and cataplexy occurs in a few patients. 66 Cerebellar ataxia or a vestibulocerebellar syndrome is a rare feature with this antibody.…”

Section: Ma1/2 Antibodiesmentioning

confidence: 99%

“…Besides this, non-SCLCs have also been commonly reported. 65 Around a third of patients tend to have neurologic improvement, and some patients tend to stabilize with immunotherapy, although response rates are even better with tumor treatment. 71 This is considered to have a better response rate than other antibody-associated paraneoplastic syndromes.…”

Section: Ma1/2 Antibodiesmentioning

confidence: 99%

Autoimmune Vestibulocerebellar Syndromes

2020

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Autoimmune disorders affecting the vestibular end organs, vestibular pathways, vestibular nuclei, and vestibulocerebellum are often underrecognized as a cause of chronic dizziness and ataxia. Autoantibodies specific for cell-surface, synaptic, and intracellular neural antigens serve as biomarkers of these disorders. This article describes the epidemiology, clinical presentation, diagnostic considerations, imaging findings, treatment, and prognosis of autoimmune disorders, in which the vestibulocerebellar syndrome is the main or presenting clinical presentation. Antibodies specific for intracellular antigenic targets described in the article are PCA-1 (Purkinje cell cytoplasmic antibody type 1, also known as anti-Yo), ANNA-1 (antinuclear neuronal antibody type 1, also known as anti-Hu), ANNA-2 (antinuclear neuronal antibody type 2, also known as anti-Ri), Ma1/2 (anti-Kelch-like 11/12 antibody), Kelch-like 11, amphiphysin, CV2 (collapsin response 2, also known as collapsin response mediator protein-5 [CRMP5]), VGCC (voltage-gated calcium channel), GAD65 (glutamic acid decarboxylase 65-kDa isoform), AP3B2 (adaptor protein 3B2, also known as anti-Nb), MAP1B (microtubule-associated protein 1B antibody, also known as anti-PCA-2), and neurochondrin antibodies. Antibodies targeting cell-surface or synaptic antigenic targets described in the article include DNER (delta/notchlike epidermal growth factor related receptor; antigen to anti-Tr), CASPR2 (contactin-associated proteinlike 2), septin-5, Homer-3, and mGluR1 (metabotropic glutamate receptor 1). The vestibulocerebellar presentation is largely indistinguishable among these conditions and is characterized by subacute onset of cerebellar symptoms over weeks to months. The diagnosis of autoimmune vestibulocerebellar syndromes is based on a combination of clinical and serological features, with a limited role for neuroimaging. Subtle eye movement abnormalities can be an early feature in many of these disorders, and therefore a meticulous vestibulo-ocular examination is essential for early and correct identification. Cancer occurrence and its type are variable and depend on the autoantibody detected and other cancer risk factors. Treatment comprises immunotherapy and cancer-directed therapy. Acute immunotherapies such as intravenous immunoglobulin, plasma exchange, and steroids are used in the initial phase, and the use of long-term immunosuppression such as rituximab may be necessary in relapsing cases. Outcomes are better if immunotherapy is started early. The neurologic prognosis depends on multiple factors.

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“…The primary cancer in this group is testicular germ cell tumors and these patients have more favorable clinical outcomes, although the majority will deteriorate [125,126] . Patients with Ma1 activity are more likely to have complications, including cervical [127] , breast [128] , lung and bladder cancers [129] . The clinical outcomes and prognosis for this group are worse with poor response to immunotherapy.…”

Section: Antineuronal Antibodies Ma1 and Ma2mentioning

confidence: 99%

Biomarkers for epileptogenesis in patients with autoimmune epilepsy

Kirmani¹,

Au²,

Mir³

et al. 2020

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Autoimmune epilepsy (AE) is a general term to describe recurrent seizures that have an immune-mediated origin. It is increasingly being recognized as a cause of epilepsy due to accumulating evidence supporting an immunemediated pathogenesis in patients who have shown resistance to traditional antiepileptic drugs (AEDs). The diagnosis of AE is one of the exclusions. Currently, there are no strict diagnostic guidelines for AE, and it is similarly under-recognized. The importance of early diagnosis of AE cannot be overstated, as prompt immunotherapy is important for seizure reduction. Further investigations into potential biomarkers are needed for early detection of AE and include targeted immunotherapies in combination with AEDs. The goal of this review was to provide an overview of the following biomarkers that have been associated with AE:

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Anti-Ma and anti-Ma2-associated paraneoplastic neurological syndromes

Cited by 35 publications

References 31 publications

Specific B- and T-cell populations are associated with cognition in patients with epilepsy and antibody positive and negative suspected limbic encephalitis

Specific B- and T-cell populations are associated with cognition in patients with epilepsy and antibody positive and negative suspected limbic encephalitis

Autoimmune Vestibulocerebellar Syndromes

Biomarkers for epileptogenesis in patients with autoimmune epilepsy

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