Acute limbic encephalitis and glutamic acid decarboxylase antibodies: A reality?

Blanc, F.; Ruppert, Élisabeth; Kleitz, Catherine; Valenti, Maria-Paola; Crétin, Benjamin; Humbel, René‐Louis; Honnorat, Jérôme; Namer, Izzie Jacques; Hirsch, Édouard; Manning, Lilianne; Seze, J. de

doi:10.1016/j.jns.2009.09.004

Cited by 63 publications

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“…The occurrence of neutropenia with AZA therapy in both cases is thought to be co-incidental. This is the second report documenting MMF usefulness in the treatment of anti-GAD Ab-associated NPLE [9].…”

Section: Discussionmentioning

confidence: 78%

“…Less than 20 cases of anti-GAD Ab-associated NPLE have been reported to date [7][8][9][10][11][12][13]. These reports suggest a relatively immunotherapy resistant disease entity with the exception of one patient (with normal MRI brain scan and without seizures) in whom memory improved with IVIg therapy alone [8], and another in whom cognitive functions stabilized and seizures resolved with MMF, corticosteroids, and AEDs [9]. The latter is the only report (excluding this one) in which MMF use was reported.…”

Section: Discussionmentioning

confidence: 99%

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Treatment of anti-glutamic acid decarboxylase antibody-associated limbic encephalitis with mycophenolate mofetil

et al. 2010

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Section: Discussionmentioning

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Treatment of anti-glutamic acid decarboxylase antibody-associated limbic encephalitis with mycophenolate mofetil

et al. 2010

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“…The most common seizure presentation was refractory status epilepticus. Mild muscular rigidity [62] and SPS [71] were diagnosed in 2 cases, as well as myoclonus [68] [73] [74] and facial cramps [62] The finding of a reduced CD8+/CD3+ ratio of the IAg-GAD cases, all without concomitant tumor, was lower than the IAg-onconeural group. It was also within the highest ratios of the SAg group of NMDAR cases consistent with the hypothesis that T-cells were a necessary aspect for neuronal loss and hippocampal atrophy in the 2 GAD-associated LE (in GAD cases 2 and 3).…”

Section: Anti-gad65 Encephalitismentioning

confidence: 99%

Autoimmune Encephalitides

Younger¹

2017

WJNS

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Autoimmune encephalitis is a potentially severe disorder of the brain of diverse causes with a complex differential diagnosis. Recent advances in the past decade have led to the elucidation of new syndromes and biological markers transforming the approach to diagnosis and management of autoimmune encephalitis. Limbic encephalitis, the commonest form of autoimmune encephalitis, combines common presentations of cognitive, psychiatric, and epileptic disorders and has until recently been considered paraneoplastic or postinfectious in origin. The autoimmune encephalitides are clinically and histopathologically associated with serum and intrathecal antibodies to intracellular and surface neuronal antigens, and constituents of the limbic system neuropil. This has led to a reconsideration of a number of neuropsychiatric and neurocognitive disorders as having shared mechanisms of origin. This chapter reviews their historical background, clinical presentation, laboratory evaluation, histopathology, diagnosis and management. KeywordsAutoimmune, Encephalitis, Hashimoto, Encephalopathy Historical PerspectiveCorsellis and colleagues [1] coined the term limbic encephalitis (LE) in 1968, noting a relation to bronchial cancer in three patients in the sixth to eighth decade of life, and showing close clinicopathologic similarity to cases described by Brierley and colleagues [2] six years earlier. All three cases had subacute temporal lobe seizures, neuropsychiatric, and memory disturbances for two years before death. Postmortem examination showed inflammatory lesions in limbic grey matter sections of the brain, especially in medial temporal lobe structures of the uncus and amygdala nuclei, and hippocampal, cingulum and dentate gyri. Case 2 had an undifferentiated non-metastatic lung carcinoma removed six months after onset of neurological symptoms, while two others had a clinically unsus- The formulation of the relation of GAD antibodies to SPS has been especially instructive in understanding how far the science of autoimmune neurologic disorder has advanced. In 1988, Solimena and colleagues [28] investigated the existence of non-paraneoplastic CNS autoimmunity in a patient with SPS, epilepsy and type-1 diabetes (T1D), and increased titers of oligoclonal CSF IgG. Both the serum and CSF produced identical intense staining of all gray-matter regions. GAD65 was thence an important autoantigen in T1D, being highly expressed in the cytoplasm of pancreatic β cells. GAD-derived peptides were presentable by main histocompatibility complex (MHC) class I molecules and recognized by CD8+ cells on the surface of β cells [29]. Activation of CD8+ GAD-specific T-cells further differentiated into memory cells suggesting a pathogenic role in T1D [30]. However, only patients with very high titers of GAD were associated with LE [31], and they typically presented with recent-onset temporal lobe epilepsy (TLE) and intrathecal secretion, defining a form of non-paraneoplastic LE. Other patients within the SPS spectrum harbored antibodies agains...

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“…L'auto-immunité anti-GAD (glutamic acid decarboxylase) a aussi été associée à des cas d'encéphalites limbiques chez des patients n'ayant pas de diabète de type 1 [24][25][26]. L'encéphalopathie de Hashimoto, caractérisée par la pré-sence d'anticorps antithyroglobuline et antipéroxydase et surtout par sa corticosensibilité, est aussi un diagnostic différentiel, mais cette auto-immunité est peu spécifique, car fréquente parmi les patients ayant une pathologie neurologique auto-immune [27].…”

Section: Diagnostic Différentiel Des Encéphalites Limbiques Avec Antiunclassified

Encéphalites paranéoplasiques et auto-immunes : quand y penser, comment en faire le diagnostic

Broucker

2011

Réanimation

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DOSSIER Encéphalites paranéoplasiques et auto-immunes : quand y penser, comment en faire le diagnostic Paraneoplastic and autoimmune encephalitis when to consider and how to diagnose it T. de Broucker Reçu le 23 janvier 2011 ; accepté le 20 février 2011 © SRLF et Springer-Verlag France 2011Résumé La dernière décennie a vu se développer et se rejoindre deux champs de recherche dans le domaine des encéphalites. D'un côté, des études de grande ampleur ont montré la variété des causes des encéphalites aiguës infectieuses, mais aussi l'absence de cause infectieuse retrouvée dans la moitié des cas. De l'autre, des équipes ont cherché la cause des encéphalites limbiques paranéoplasiques dans lesquelles aucun anticorps onconeuronal classique n'était mis en évidence. C'est ainsi que l'encéphalite avec anticorps anti-NMDAR (antirécepteur NMDA du glutamate) et l'encéphalite avec anticorps anti-VGKC (anti-canal potassique voltage-dépendant, démembrée désormais principalement en leucine-rich glioma inactivated protein [LGI-1] et contactin-associated protein 2 [CASPR2]), ont été découver-tes et décrites. De plus, il s'est avéré qu'une partie non négligeable des encéphalites aiguës sans cause infectieuse retrouvée sont dues en fait à des encéphalites auto-immunes, souvent paranéoplasiques, sensibles aux immunothérapies, et à l'ablation de la tumeur dans les cas où une tumeur est retrouvée. La gravité de ces encéphalites nécessite souvent la prise en charge en soins intensifs ou en réanimation, imposant une bonne connaissance de ces pathologies. Les syndromes neurologiques paranéoplasiques, les encéphalites limbiques classiques, les encéphalites avec anticorps dirigés contre les antigènes de surface membranaire (dont NMDAR) sont détaillés ainsi que leurs diagnostics différentiels avec une revue de la littérature la plus récente sur le sujet. Pour citer cette revue : Réanimation 20 (2011).Mots clés Encéphalite limbique · Syndrome neurologique paranéoplasique · Anticorps onconeuronaux · Anticorps anti-NMDAR Abstract The field of limbic paraneoplastic and autoimmune encephalitis has been greatly enhanced during the last decade by the discovery of neuronal cell-surface directed autoimmunity in the serum and/or CSF of limbic encephalitis patients. The main antibodies target the glutamate NMDA receptor and the voltage-gated potassium channel (VGKC), now known to be mainly LGI-1 and CASPR2 proteins. The recent discovery that a number of acute encephalitis of unknown cause are due to anti-NMDAR or anti-VGKC encephalitis has induced a great interest in these pathologies from critical care physicians because they display specific clinical patterns and can be cured by surgical removal of a tumour, if applicable, and immunotherapy. Neurological paraneoplastic syndromes, classic limbic encephalitis, cellsurface antibody-associated encephalitis are described and the most recent literature is reviewed. To cite this journal: Réanimation 20 (2011).

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Acute limbic encephalitis and glutamic acid decarboxylase antibodies: A reality?

Cited by 63 publications

References 20 publications

Treatment of anti-glutamic acid decarboxylase antibody-associated limbic encephalitis with mycophenolate mofetil

Treatment of anti-glutamic acid decarboxylase antibody-associated limbic encephalitis with mycophenolate mofetil

Autoimmune Encephalitides

Encéphalites paranéoplasiques et auto-immunes : quand y penser, comment en faire le diagnostic

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