Non-paraneoplastic limbic encephalitis associated with anti-glutamic acid decarboxylase antibodies

Matà, Sabrina; Muscas, Giovanni; Naldi, Ilaria; Rosati, Eleonora; Paladini, Stefanía; Cruciatti, Barbara; Bisulli, Francesca; Paganini, Marco; Mazzi, Giorgio; Sorbi, Sandro; Tinuper, Paolo

doi:10.1016/j.jneuroim.2008.05.015

Cited by 113 publications

(89 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…9 Recently, Mata et al described the association between non-paraneoplastic limbic encephalitis and anti-GAD antibodies. 10 Antibodies against GAD were first reported in stiffperson syndrome. This finding was later linked to its pathogenesis.…”

Section: Discussionmentioning

confidence: 99%

Limbic encephalitis associated with anti‐GAD antibody and common variable immune deficiency

Akman¹,

Patterson²,

Rubinstein³

et al. 2009

Develop Med Child Neuro

View full text Add to dashboard Cite

A variety of autoantibodies have been identified with complex neurological disorders including limbic encephalitis. The underlying trigger for the immune‐mediated process and the role of autoantibodies in the pathogenesis of limbic encephalitis remain to be clarified. Here, we report a 16‐year‐old female who was diagnosed with acute‐onset non‐neoplastic limbic encephalitis. The initial treatment with pulse doses of i.v. methylprednisolone improved the neurological symptoms. During the next 12 months, progressive decline was reported in her academic functioning and seizure control. Additional diagnostic evaluation revealed no evidence of malignancy or central nervous system infection but circulating anti‐GAD antibodies were present in the serum and cerebrospinal fluid. Intravenous gammaglobulin infusion was initiated and continued monthly. Intravenous and oral steroids were added to the intravenous immunoglobulin treatment because of the worsening course and seizures, despite treatment with antiepileptic medications. Screening for quantitative immunoglobulins demonstrated hypogammaglobulinaemia with low immunoglobulin M and G in addition to low immunoglobulin A levels. There was a lack of protective pneumococcal antibody titers before and after immunization. Therefore, common variable immunodeficiency was suspected despite there being no history of recurrent infections. To our knowledge, this is the first report describing a possible link between immune‐mediated limbic encephalitis and immune deficiency.

show abstract

Section: Discussionmentioning

confidence: 99%

Limbic encephalitis associated with anti‐GAD antibody and common variable immune deficiency

Akman¹,

Patterson²,

Rubinstein³

et al. 2009

Develop Med Child Neuro

View full text Add to dashboard Cite

show abstract

“…EEG may show paroxysmal discharge in temporal area or, most frequently, slow waves in temporal lobe one or both sides [33]. The cerebrospinal fluid analysis remains mandatory to support the diagnosis (inflammatory features in 80% LE) [37]. In LE patients, neuropathological examination shows neuronal loss in the amygdala and hippocampal area, perivascular lymphocytic infiltrates, microglial aggregation, and glycosuria [35,36].…”

Section: Dmentioning

confidence: 99%

“…EEG może uwidocznić wyładowania napadowe w okolicach skroniowych lub najczęściej fale wolne w okolicach skroniowych, jedno-lub obustronnie [33]. Badanie płynu mózgowo--rdzeniowego ma znaczenie wspomagające (cechy zapalenia w 80% LE) [37]. Neuropatologicznie u chorych z LE stwierdza się ubytek neuronów w obrębie hipokampa i ciała migdałowatego, okołonaczyniowe nacieki limfocytarne, zgrupowania mikrogleju oraz gliozę [35,36].…”

Section: Dunclassified

Non-paraneoplastic variant of limbic encephalitis – case report

Głuszak¹,

Pazderska-Głuszak²,

Gut-Lepiech³

et al. 2017

ppn

View full text Add to dashboard Cite

Purpose: Limbic encephalitis (LE) is a rare but necessary condition to consider when experiencing cognitive disorders, seizures, and psychiatric disorders. Case description: This article presents a case of a 28-year-old patient with uncontrolled disease history, with systemic symptoms of viral infection and prolonged convulsive seizure status. Based on clinical symptoms, magnetic resonance imaging of the brain and cerebrospinal fluid test results revealed LE. After treatment and rehabilitation, the patient returned to work. Comment: Limbic encephalitis was first described in 1960. In terms of aetiology, we divide LE into non-infectious (a para nitrogen syndrome primarily associated with small cell lung cancer) and infectious cases (caused by herpes simplex virus). Other cases of autoimmune diseases have also been reported -non-paraneoplastic limbic encephalitis (NPLE), in which the presence of antibodies against the antigen of the neuronal membrane is observed. Non-paraneoplastic LE variants are examples of potentially reversible LE variants. Key words: limbic encephalitis, paraneoplastic syndrome, onconeuronal antibodies. StreszczenieCel: Limbiczne zapalenie mózgu (LE) jest chorobą rzadką, którą należy jednak uwzględnić w przypadku wystąpienia u pacjenta zaburzeń poznawczych, napadów padaczkowych i zaburzeń psychicznych. Opis przypadku: W pracy przedstawiono przypadek 28-letniej pacjentki z nieobciążonym wywiadem chorobowym, u której wystąpiły objawy ogólnoustrojowe infekcji wirusowej oraz przedłużający się drgawkowy stan padaczkowy. Na podstawie objawów klinicznych, obrazu rezonansu magnetycznego mózgu i wyników badań płynu mózgowo-rdzeniowego rozpoznano LE. Po leczeniu i rehabilitacji pacjentka wróciła do poprzednio wykonywanej pracy. Komentarz: Limbiczne zapalenie mózgu zostało opisane po raz pierwszy w 1960 r. Pod względem etiologicznym wyróżniamy przypadki nieinfekcyjne LE (o charakterze zespołu paranowotworowego związanego głównie z rakiem drobnokomórkowym płuc) oraz przypadki infekcyjne LE (spowodowane przez wirusa opryszczki pospolitej). Opisano również przypadki związane z chorobami

show abstract

“…Antibodies to voltage-gated potassium channels (VGKCs) were identified in a number of patients with non-paraneoplastic limbic encephalitis, but further studies have shown that the real antigens in these patients are leucine-rich glioma inactivated 1, contactinassociated protein-like 2, and contactin-2, all of which are associated with VGKCs [230,231]. The spectrum of autoantigens associated with in limbic encephalitis, however, continues to expand, and now includes the N-methyl-D-aspartate receptor (NMDAR), the AMPA receptor, the GABA = gamma-aminobutyric acid receptor, and glutamic acid decarboxylase [232][233][234][235]. It is quite likely that many other pathologic autoantigens will be identified.…”

Section: Limbic Encephalitismentioning

confidence: 99%

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Geldern¹,

McPharlin

Becker

2012

Neurotherapeutics

View full text Add to dashboard Cite

This chapter will review the spectrum of immunemediated diseases that affect the nervous system and may result in an admission to the neurological intensive care unit. Immunomodulatory strategies to treat acute exacerbations of neurological diseases caused by aberrant immune responses are discussed, but strategies for long-term immunosuppression are not presented. The recommendations for therapeutic intervention are based on a synthesis of the literature, and include recommendations by the Cochrane Collaborative, the American Academy of Neurology, and other key organizations. References from recent publications are provided for the disorders and therapies in which randomized clinical trials and large evidenced-based reviews do not exist. The chapter concludes with a brief review of the mechanisms of action, dosing, and side effects of commonly used immunosuppressive strategies in the neurocritical care unit.

show abstract

Non-paraneoplastic limbic encephalitis associated with anti-glutamic acid decarboxylase antibodies

Cited by 113 publications

References 15 publications

Limbic encephalitis associated with anti‐GAD antibody and common variable immune deficiency

Limbic encephalitis associated with anti‐GAD antibody and common variable immune deficiency

Non-paraneoplastic variant of limbic encephalitis – case report

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Contact Info

Product

Resources

About