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

Abstract

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.

Autoimmune encephalitis with anti-IgLON5 and anti-GABAB-receptor antibodies

“…Previous case reports already described the coexistence of anti-GABA B -receptor and anticollapsin response-mediator protein 5 antibodies [7] or anti-GABA B receptor and anti-CRMP5/CV2 antibodies. [4] Here, we present a patient revealing anti-IgLON5 and anti-GABA B -receptor antibodies.…”

Clinical characteristics of autoimmune encephalitis with co-existence of multiple anti-neuronal antibodies

“…Chung et al [ 9 ] reported the co-existence of IgLON5 and GABA B R antibodies in an AE patient who presented predominantly with typical IgLON5-related symptoms, including a severe sleep disorder, gait instability, dysarthria, and recurrent visual and acoustic hallucinations, but without the distinctive features of GABA B R-related symptoms of limbic encephalitis. In addition, Xia et al [ 10 ] described the co-existence of GABA B R and CV2 antibodies in a patient who presented with seizures, memory deficits, and visual hallucination, despite hallucinations not having been reported previously in AE patients with GABA B R or CV2 antibodies. Therefore, the clinical manifestations in these patients with co-existence of multiple auto-antibodies might be more complicated, making it difficult to determine which antibody is pathogenic or concomitant in these patients.…”

Clinical characteristics of autoimmune encephalitis with co-existence of multiple anti-neuronal antibodies

“…Chung et al [10] reported the co-existence of IgLON5 and GABA B R antibodies in AE patient who predominantly presented with typical IgLON5-related symptoms, including severe sleep disorder, gait instability, dysarthria, and recurrent visual and acoustic hallucinations, but without distinctive features of GABA B R-related symptoms of limbic encephalitis. Xia et al [11] described the co-existence of GABA B R and CV2 antibodies in a patient who presented with seizures, memory de cits, and visual hallucination, while the hallucinations was not reported in AE associated with GABA B R or CV2 antibodies. Therefore, the clinical manifestations in these patients with co-existence of multiple auto-antibodies might be more complicated, and it was di cult to determine which antibody was pathogenic or concomitant in these patients.…”