We report a fatal case of retroperitoneal hemorrhage following standard-dose enoxaparin treatment in a patient with autoimmune encephalitis presenting with new-onset refractory status epilepticus. A 61-year-old female developed deep vein thrombosis during treatment and received enoxaparin, following which she experienced sudden hypovolemic shock due to massive retroperitoneal bleeding. Despite intensive care, the patient died of multiorgan failure. To our knowledge, this is the first such case reported in South Korea. Clinicians should be aware of the rare but serious risk of hemorrhage when administering anticoagulants to patients with autoimmune encephalitis and other complex and evolving clinical conditions.

PurposeAnti–N-methyl-ᴅ-aspartate receptor encephalitis (NMDARE) is an autoimmune disorder of the central nervous system characterized by subacute-onset neurocognitive disorders, seizures, movement disorders, and prominent neuropsychiatric features affecting young adults. This case series describes the clinical profile, neuroimaging features, and outcomes in NMDARE patients and attempts to compare its findings with previously published studies in an Indian setting. MethodsAll consecutive patients diagnosed with definite NMDARE over a 2-year period were evaluated and followed up with. A comprehensive literature review was conducted in PubMed, Scopus, Embase, and Google Scholar, covering studies published up to January 2025. ResultsThe case series included six patients (four females) aged 10–19 years presenting with super-refractory status epilepticus, dystonia, rubral tremors, and psychiatric symptoms. Tumor screening was negative in all cases. Magnetic resonance imaging of the brain revealed white matter hyperintensities in the medial temporal lobe, periventricular white matter, basal ganglia, and midbrain. Electroencephalogram abnormalities included diffuse slowing, extreme delta brush, and epileptiform discharges. Patients responded to high-dose intravenous methylprednisolone and intravenous immunoglobulin in most cases, with rituximab and cyclophosphamide administered successfully in refractory cases. Our review of Indian literature on NMDARE revealed 60 studies (115 patients), among which 48 case reports were excluded and 12 clinical studies and case series were included. Included patients were aged 2–29 years and mostly female, with similar clinical and imaging profiles to our population and a comparable low yield of tumor screening. Prognosis was good with immunotherapy, with few fatality reports.ConclusionNMDARE patients present with protean clinical manifestations and difficult diagnostic and therapeutic challenges, resulting in an unclear prognosis. Early recognition, aggressive immunotherapy, and regular follow-up are key to better outcomes.

Electroconvulsive therapy (ECT) is a widely used therapeutic option of drug-refractory psychiatric disorders. ECT treats psychiatric symptoms by inducing brief controlled seizures through electrical stimulation, but ECT does not generally cause prolonged seizures or epilepsy. However, several studies have reported cases of prolonged seizures after ECT. This review aimed to determine the mechanism of epileptogenesis with neurobiological changes after ECT. Contrary to epileptogenesis by ECT, several cases have reported that ECT was successfully applied for treatment of refractory status epilepticus. In addition, ECT might be applied to hyperkinetic movement and psychiatric symptoms of encephalitis. We also investigated the anticonvulsant mechanism of ECT and how it controls encephalitis symptoms.

The suprachiasmatic nucleus (SCN) in the anterior hypothalamus is the major circadian pacemaker in humans. Melatonin is a key hormone secreted by the pineal gland in response to darkness. Light-induced stimuli are transmitted along the retinohypothalamic tract to the SCN. Activation of the SCN inhibits the production of melatonin by the pineal gland through a complex neural pathway passing through the superior cervical ganglion. Accordingly, when light is unavailable, the pineal gland secretes melatonin. The circadian rhythm modulates sleep-wake cycles as well as many physiological functions of the endocrine system, including core body temperature, pulse rate, oxygen consumption, hormone levels, metabolism, and gastrointestinal function. In neurodegenerative disorders, the sleep-wake cycle is disrupted and circadian regulation is altered, which accelerates disease progression, further disrupting circadian regulation and setting up a vicious cycle. Melatonin plays a critical role in the regulation of circadian rhythms and is a multifunctional pleiotropic agent with broad neuroprotective effects in neurodegenerative disorders, viral or autoimmune diseases, and cancer. In this review, I discuss the neuroprotective functions of melatonin in circadian regulation and its roles in promoting anti-inflammatory activity, enhancing immune system functions, and preventing alterations in glucose metabolism and mitochondrial dysfunction in neurodegenerative disorders and autoimmune central nervous system diseases.

Convulsive status epilepticus (SE) is a medical emergency associated with high morbidity and mortality. Recently, clinical trials and meta-analyses investigating medical treatment of SE have been published. Benzodiazepine is well known as the first-line treatment for SE. Recent evidence suggests the equivalence of intravenous fosphenytoin, valproate, and levetiracetam for treatment of established SE. There is lack of evidence regarding treatment for refractory SE. Intravenous midazolam is commonly used, and recent evidence supports the use of ketamine. Additional studies are needed to improve the management of convulsive SE.

The current coronavirus disease 2019 (COVID-19) pandemic is caused by severe acute respiratory syndrome coronavirus 2. Due to the increasing number of confirmed cases and accumulating clinical data, in addition to the predominant respiratory symptoms, a significant proportion of patients with COVID-19 experience neurological complications. Presumedly, several mechanisms, such as direct viral effects on the brain parenchyma and endothelium, and activation of the inflammatory and thrombotic pathways, cause these neurological disorders. Herein, the literature focusing on encephalitis among the central nervous system disorders associated with COVID-19 was reviewed.

Acute disseminated encephalomyelitis (ADEM) is a monophasic central nervous system inflammatory demyelinating disorder clinically defined by multifocal neurologic symptoms with encephalopathy. Brain magnetic resonance imaging most often reveals multiple T2 hyperintense lesions consistent with demyelination. High-dose corticosteroids are the current treatment of choice, and intravenous immunoglobulin or plasmapheresis is considered in steroid-unresponsive cases. The majority of patients show favorable outcomes with full clinical recovery and complete or partial resolution of previous lesions. For patients who are refractory to multiple immunomodulatory agents, other treatments such as rituximab have been used. We present a patient who developed ADEM after Epstein-Barr virus infection who achieved full recovery with immunotherapy including rituximab.

Paraneoplastic encephalitis is autoimmune encephalitis accompanied by tumors. Renal cell carcinoma (RCC) is a kidney cancer originating from various types of renal cells and rarely has been associated with paraneoplastic neurologic manifestation. We identified a case series of paraneoplastic encephalitis-associated RCC. From a prospective institutional cohort, we identified autoimmune encephalitis patients with RCC. The association between RCC and encephalitis was determined by the following criteria: (1) possible autoimmune encephalitis according to the operational autoimmune encephalitis diagnostic criteria and (2) RCC simultaneously diagnosed with neurological manifestation of encephalitis. A total of three patients presented encephalitis accompanied by RCC. Two patients had clear cell RCC, and one had chromophobe RCC. All patients showed limbic encephalitis with cognitive decline, memory impairment, or seizure. Brain magnetic resonance imaging showed T2 hyperintensities at the mesial temporal lobe in two patients with clear cell RCC but no remarkable findings in one patient with chromophobe RCC. While one patient who had early surgery within one month of RCC onset had a favorable response to the treatment, the other two patients showed a partial response and a detrimental result. Paraneoplastic encephalitis associated with RCC presented as limbic encephalitis and was responsive to immunotherapy combined with tumor resection. As our cases identified, RCC should be considered as a cause of seronegative autoimmune encephalitis.

Regulatory T cells (Tregs) play a crucial role in maintaining immune tolerance. Any deficiency or dysfunction of the Tregs can influence the pathogenesis of autoimmune disease. This study aimed to assess the role of Tregs among patients with autoimmune encephalitis (AE) with different autoantibody types and to evaluate their association with clinical features. This was a cross-sectional observational study involving 29 patients with AE. Peripheral blood was sampled from each patient for flow cytometric analysis. Proportions of CD4+CD25+ and CD4+CD25+Foxp3+ Tregs were calculated and compared between the antibody types (synaptic, paraneoplastic, and undetermined). Associations between the proportion of Tregs and clinical features were also evaluated. Five patients had synaptic autoantibodies, five had paraneoplastic autoantibodies, and the others were of an undetermined type. The proportion of CD4+CD25+ Tregs tended to be higher in those with paraneoplastic antibodies than in those with synaptic antibodies (post-hoc p = 0.028) and undetermined antibody status (post-hoc p = 0.043). A significant negative correlation was found between the proportion of Tregs and the initial modified Rankin score (r = -0.391, p = 0.036). Those who received intravenous immunoglobulin had lower proportions of Tregs than those who did not. The results of the present study suggest that Tregs may play different roles according to the type of AE and may be linked to disease severity.