Epileptic Seizures and Right-Sided Hippocampal Swelling as Presenting Symptoms of Anti-IgLON5 Disease: A Case Report and Systematic Review of the Literature

Abstract

Background and ObjectiveAnti-IgLON5 disease is an uncommon neurological disorder characterized by diverse clinical manifestations. Although many relevant cases have been reported, our understanding of this disorder is still quite restricted. We present a rare case of anti-IgLON5 disease and performed a comprehensive systematic review of all published cases to expand the clinical spectrum of this disorder.MethodsWe report a 61-year-old woman with an atypical presentation of epileptic seizures with abnormal signals in her right hippocampus on brain magnetic resonance imaging (MRI). A systematic review was performed of electronic databases, including PubMed, EMBASE, China National Knowledge Infrastructure (CNKI), WanFang and VIP China Science.ResultsWe identified 161 cases from 65 publications. With heterogeneous clinical manifestations, we found that bulbar dysfunction, sleep apnea, gait instability and neurocognitive and behavioral symptoms are the most common symptoms of anti-IgLON5 disease. Anti-IgLON5 antibodies presented a higher positive rate and titer in the serum than in the cerebrospinal fluid (CSF). Haplotype DRB1*10:01-DQB1*05:01 is highly correlated with anti-IgLON5 disease. Only 38 patients have presented distinctive MRI alterations (26.2%). Approximately half of the cases are responsive to immunosuppressive or immunomodulatory treatment.ConclusionAnti-IgLON5 disease is characterized by various clinical manifestations and laboratory findings. Immunotherapy may be effective in treating anti-IgLON5 disease, but the results are far from satisfactory. Studies with larger sample sizes are required to improve the current understanding of this disorder.

Ischemic and Metabolic Stroke Can Co-occur in m.3243A>G Carriers: A Case Report

“…Whether seizure activity triggered the development of the SLE, particularly the neuropsychological deficits at onset, remains speculative but is conceivable since SLEs are frequently accompanied by seizures [7]. Arguments against a seizure as the cause of the lesion are that relatives or caregivers did not observe a seizure shortly before or during admission, that the initial EEG did not show any epileptiform discharges, and that the lesion did not show an increase in volume, as is often found in seizure MRIs [8]. Another argument against seizures as the trigger of the SLL is that the patient suffered a series of left focal seizures not earlier than on the 10th day of hospitalization.…”

Autoimmune antibody-induced neuronal hyperactivity triggers pathological Tau in IgLON5 disease

“…a-IgLON5 disease is a recently described, rare but severe neurological disorder characterized by the presence of AABs targeting the neuronal cell surface protein IgLON5 3 . Individuals diagnosed with a-IgLON5 disease demonstrate diverse clinical manifestations and commonly experience sleep behavior abnormalities, movement disorders, memory deficits, and seizures [4][5][6] . Brains of a-IgLON5 patients show intraneuronal accumulations of highly phosphorylated Tau protein (p-Tau) in several regions, including hippocampus, cerebellum, brain stem, hypothalamus, and basal ganglia, as well as in the spinal cord 3,7 .…”