Refractory status epilepticus and autoimmune encephalitis with GABA A R and GAD65 antibodies: A case report

Gagnon, Marc-Aurèle; Savard, Martin; Amari, Karim Mourabit

doi:10.1016/j.seizure.2016.02.006

Cited by 17 publications

(9 citation statements)

References 4 publications

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“…Despite medical interventions, our patient experienced a relapse within 4 months, which is consistent with Gagnon et al's report [ 10 ] of a patient with AE with anti-GABA A R and antiglutamate decarboxylase 65 antibodies who endured severe relapses. It is therefore plausible that AE with multiple antineuronal antibodies is relapse-prone.…”

Section: Discussionsupporting

confidence: 92%

Autoimmune encephalitis positive for both anti-γ-aminobutyric acid B receptor and anticollapsin response-mediator protein 5 antibodies

Xia

Yin²,

Zhu³

et al. 2018

Medicine

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Rationale:Autoimmune encephalitis (AE) is a heterogeneous group of recently identified disorders. Despite severe and even prolonged neurologic deficits, dramatic improvements may occur with proper immunotherapy in some patients with AE. Antineuronal antibodies have been discovered in patients’ serum and cerebrospinal fluid (CSF). However, AE with multiple antineuronal antibodies is rare. To date, there are no published reports of AE with both anti-γ-aminobutyric acid B receptor (GABABR) and anticollapsin response-mediator protein 5 (CV2) antibodies.Patient concerns:We describe a 46-year-old man who presented with seizures, working memory deficits, and visual hallucinations. We detected anti-CV2 and anti-GABABR antibodies in his serum and CSF. Brain magnetic resonance imaging (MRI) revealed patchy abnormal signals in his left temporal lobe and hippocampus. The patient's symptoms improved after receiving intravenous immunoglobulin injections and glucocorticoids, but his condition relapsed within 4 months, and he was readmitted to our hospital. Repeated MRI scans revealed new lesions in his right temporal lobe and hippocampus.Diagnosis:The AE diagnosis was established from the results of the preliminary physical examination, the laboratory tests, and the imaging findings.Interventions:The patient received intravenous immunoglobulins and glucocorticoids.Outcomes:We followed the patient for 9 months from the date of the patient's second hospital discharge. He experienced no seizures during this period, but his short-term memory deficits and visual hallucinations were not completely alleviated.Lessons:Coexisting anti-CV2 and anti-GABABR antibodies may have synergistic effects and worsen the clinical syndrome. AE with multiple antineuronal antibodies may be relapse-prone. Further studies investigating the relationship between anti-CV2 and anti-GABABR antibodies are warranted.

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Section: Discussionsupporting

confidence: 92%

Autoimmune encephalitis positive for both anti-γ-aminobutyric acid B receptor and anticollapsin response-mediator protein 5 antibodies

Xia

Yin²,

Zhu³

et al. 2018

Medicine

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“…Multiple antibody positivity associated with cancer has been reported in several studies [182, 183]. Petit-Pedrol et al (2014) recently reported five cases of anti-GABA A R and anti-GAD65 antibodies co-occurring in CSF and serum [17], and a single case report of a female with encephalitis was found to harbor anti-GABA A R and anti-GAD65 antibodies in CSF and serum [184]. Other double antibody positivity combinations that have been identified are anti-NMDAR and anti-GAD antibodies [185], anti-NMDAR and anti-D2R [165, 186], anti-GABA B R and anti-Hu [187], and anti-Caspr2 and anti-Lgi1 in a patient with Morvan syndrome [188].…”

Section: Potential Mechanisms Of Double Autoantibody Positivitymentioning

confidence: 99%

Mapping autoantigen epitopes: molecular insights into autoantibody-associated disorders of the nervous system

Sinmaz

Nguyen

Charabati

et al. 2016

J Neuroinflammation

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BackgroundOur knowledge of autoantibody-associated diseases of the central (CNS) and peripheral (PNS) nervous systems has expanded greatly over the recent years. A number of extracellular and intracellular autoantigens have been identified, and there is no doubt that this field will continue to expand as more autoantigens are discovered as a result of improved clinical awareness and methodological practice. In recent years, interest has shifted to uncover the target epitopes of these autoantibodies.Main bodyThe purpose of this review is to discuss the mapping of the epitope targets of autoantibodies in CNS and PNS antibody-mediated disorders, such as N-methyl-D-aspartate receptor (NMDAR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), leucine-rich glioma-inactivated protein 1 (Lgi1), contactin-associated protein-like 2 (Caspr2), myelin oligodendrocyte glycoprotein (MOG), aquaporin-4 (AQP4), 65 kDa glutamic acid decarboxylase (GAD65), acetylcholine receptor (AChR), muscle-specific kinase (MuSK), voltage-gated calcium channel (VGCC), neurofascin (NF), and contactin. We also address the methods used to analyze these epitopes, the relevance of their determination, and how this knowledge can inform studies on autoantibody pathogenicity. Furthermore, we discuss triggers of autoimmunity, such as molecular mimicry, ectopic antigen expression, epitope spreading, and potential mechanisms for the rising number of double autoantibody-positive patients.ConclusionsMolecular insights into specificity and role of autoantibodies will likely improve diagnosis and treatment of CNS and PNS neuroimmune diseases.

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“…Notwithstanding, this patient suffered from seizure recurrences upon withdrawal of the therapy underscoring the fact that the appropriate duration for immunosuppressive therapy in patients with autoimmune encephalitis is unknown. [ 15 ]…”

Section: Discussionmentioning

confidence: 99%

GABAA receptor encephalitis associated with human parvovirus B19 virus infection

Valle

Santos²,

Spinosa³

et al. 2021

Medicine

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Rationale: Human parvovirus B19 (B19) infection can produce a spectrum of clinical syndromes, including neurological manifestations, most notably encephalitis. Although symptoms suggestive of autoimmune disease in patients with B19 infection have been previously described, a clear association of autoimmune encephalitis with B19 infection has yet to be established. Patient concerns: We describe the case of a 6-year-old boy who was hospitalized due to status epilepticus, which evolved to super-refractory status epilepticus that was only mildly responsive to anticonvulsant drugs. Diagnosis: A cerebrospinal fluid study identified slight pleocytosis and B19 positivity. A subsequent autoimmunity cerebrospinal fluid study revealed the presence of anti-γ-aminobutyric acid type A (GABA A ) receptor antibodies. Interventions: After pulse therapy with methylprednisolone and continuous therapy with prednisolone with cyclosporine, the patient experiencing seizure persistence with disordered motor function manifestations and only minor improvement in consciousness, and so, plasmapheresis was performed. With continued immunosuppressive treatments with cyclosporine and prednisolone, the patient's clinical picture showed progressive improvement, with good control of seizures. Although the patient tolerated withdrawal of the anticonvulsant drugs well, he developed seizures when corticosteroid therapy withdrawal was attempted, so was started on azathioprine. Outcomes: After immunosuppressive therapy, the patient evolved with complete remission of symptoms, normal neurological examination and age-appropriate neuropsychomotor development. Lessons: The present case characteristics, together with previous findings, support the hypothesis that autoimmunity may be triggered by extensive antigen release due to degeneration of infected neurons. This case highlights the importance of early clinical suspicion and treatment.

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Refractory status epilepticus and autoimmune encephalitis with GABA A R and GAD65 antibodies: A case report

Abstract: Autoimmune encephalitis associated with GABAAR and GAD65 antibodies might be a severe and refractory disease. The appropriate treatment is currently unknown for those patients.

Cited by 17 publications

References 4 publications

Autoimmune encephalitis positive for both anti-γ-aminobutyric acid B receptor and anticollapsin response-mediator protein 5 antibodies

Autoimmune encephalitis positive for both anti-γ-aminobutyric acid B receptor and anticollapsin response-mediator protein 5 antibodies

Mapping autoantigen epitopes: molecular insights into autoantibody-associated disorders of the nervous system

GABAA receptor encephalitis associated with human parvovirus B19 virus infection

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