Acta Epileptologica最新文献

Background: Anti-leucine-rich glioma-inactivated 1 (LGI-1) autoimmune encephalitis (AE), characterized by rapid decline of memory, seizures, and neuropsychiatric abnormalities, is a rare but devastating disorder. Early diagnosis and treatment are essential to prevent long-term sequelae. In this report, we provide a detailed description of clinical characteristics, laboratory test results, imaging, and electroencephalography (EEG) findings, as well as treatment responses of eight patients with anti-LGI-1 AE treated at our center.

Case presentation: At the onset, all eight patients presented with confusion/memory deterioration, seizures (including faciobrachial dystonic seizures or other types of seizure), and behavioral changes such as hallucination, paranoia, and anxiety. Four patients were found with severe hyponatremia. Anti-LGI1 antibodies were detected in the cerebrospinal fluid and/or serum of all patients. For patients with faciobrachial dystonic seizures, no discernible scalp EEG change was detected, while EEG recording of patients experiencing other types of seizure showed focal slowing, focal epileptiform discharges, and focal onset seizures. All patients showed abnormal brain magnetic resonance imaging signals, mainly involving the mesial temporal lobe and the hippocampus. In addition, one patient also experienced fulminant cerebral edema during the acute phase of the illness. All patients received immunotherapy and anti-seizure medications and achieved good seizure control. Nevertheless, these patients continued to experience cognitive impairment during their long-term follow-ups.

Conclusions: The care of anti-LGI1 AE patients requires rapid evaluation, prompt initiation of immunotherapy, and long-term follow-up. The long-term presence of neurocognitive complications observed in these patients underline the importance of developing reliable biomarkers that can distinguish between different subtypes of this disease with heterogeneous clinico-electrographico-radiological features. Further research is needed to understand the molecular mechanisms underlying the heterogeneity, in order to facilitate development of more effective treatments for anti-LGI1 AE.

Researchers have widely acknowledged the therapeutic value of epilepsy surgery for drug-resistant epilepsy. Nonetheless, there is a substantial gap in the surgical treatment for appropriate candidates owing to several factors, particularly in the population of young children. To standardize the protocols of preoperative evaluation and surgery of young children for epilepsy surgery, the China Association Against Epilepsy has appointed an expert task force to standardize the protocols of preoperative evaluation and surgery in pediatric epilepsy patients. It adopted the modified Delphi method and performed two rounds of surveys through an anonymous inquiry among 75 experts from four subgroups including pediatric neurologists, epileptologists, pediatric epilepsy surgeons, and functional neurosurgeons. The survey contents contained: (1) the participants, comprising children aged ≤ 6 years; (2) adopted DRE definition proposed by the International League Against Epilepsy in 2010; and (3) investigated epilepsy surgery, principally referring to curative epilepsy surgeries. The neuromodulation therapies were excluded because of the differences in treatment mechanisms from the above-mentioned surgeries. According to the Delphi process, a consensus was achieved for most aspects by incorporating two rounds of surveys including preoperative assessment, surgical strategies and techniques, and perioperative and long-term postoperative management, despite controversial opinions on certain items. We hope the results of this consensus will improve the level of surgical treatment and management of intractable epilepsy in young children.

Background: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a novel autoimmune encephalitis (AE) first identified in 2007. It provides a new direction for clinicians when encountering unexplained symptoms such as seizures, psychotic behavioral abnormalities, speech disorders, and involuntary movements. Most patients have a good prognosis after immunotherapy, but some may experience relapses.

Case presentation: We report a Chinese female patient diagnosed with anti-NMDAR encephalitis. Over the past 30 years, the patient had experienced eight episodes with seizures as the first symptom, which eventually progressed to autoimmune-associated epilepsy. In the last two episodes, both serum and cerebrospinal fluid of the patient were negative for AE-related antibodies, and brain magnetic resonance imaging (MRI) revealed abnormal hyperintensity in the bilateral hippocampi. The patient's symptoms were poorly controlled by immunotherapy but well controlled by anti-seizure medicines.

Conclusions: Patients with a long history of AE and multiple relapses that start with seizures may display alterations of brain structure. Physicians should pay attention to autoimmune-associated epilepsy.

Background: Focal cortical dysplasia (FCD) is a common cause of drug-resistant epilepsy. Electroencephalography (EEG) biomarkers that predict good postoperative outcomes are essential for identifying patients with focal epilepsies.

Case presentation: We report the case of a 21-year-old female with seizure onset at the age of 9, characterized by left-hand dystonic posturing and impaired awareness, which evolved to bilateral tonic-clonic seizures, evaluated in a neurological referral center in Lima, Peru. During 6-h video-EEG, interictal EEG revealing focal brief potentially ictal rhythmic discharges (BIRDs) over the right frontal central region, lasting less than 10 s. The ictal features were characterized by low-voltage fast activity over the same area. Brain magnetic resonance imaging (MRI) demonstrated a focal lesion of focal cortical dysplasia type II in the right frontal lobe. The patient underwent a lesionectomy guided by electrocorticography, which showed continuous polyspikes. BIRDs showing a brief burst of spikes lasting longer than 0.5 s, were also identified on intraoperative electrocorticography (ECoG) and helped define the extent of resection. The patient obtained an Engel Outcome Class IA at 6 years of follow-up.

Conclusions: The atypical BIRDs on ECoG can be used as a prognostic biomarker for prolonged seizure-freedom outcome in patients with epilepsy. Additional reports are needed in developing countries with and without brain MRI lesions to advance outpatient presurgical evaluations despite limited resources.