Background Leucine-rich glioma-inactivated protein 1 (LGI1) antibody-mediated encephalitis is a rare subtype of autoimmune encephalopathy, which is associated with autoimmunity against the neuronal plasma membrane proteins. The characteristic symptoms of this disease are memory dysfunction, seizures, faciobrachial dystonic seizures, cognitive deficits, neuropsychiatric disturbances, and intractable hyponatremia. The diagnosis of this disease mainly depends on the presence of anti-LGI1 antibody in serum or cerebrospinal fluid of patients. LGI1 antibody encephalitis has been reported mostly in adults, with rare occurrences in children. Case presentation In this report, we described a 4-year-old girl with typical seizures. Seizure types included focal seizures and generalized tonic-clonic seizures. The electroencephalogram findings showed focal discharges. Brain magnetic resonance imaging (MRI) showed normal. The cerebrospinal fluid (CSF) levels of cells, glucose, and chloride were within the normal range, and the culture did not reveal growth of any pathogen. Test of serum LGI1-Ab was positive, while the tests for autoimmune encephalitis antibody series in CSF were negative. The seizures of the patient were completely controlled after the therapy of immunoglobulin, methylprednisolone and antiepileptic drugs (AEDs), and the mental state almost returned to normal. Conclusion To our knowledge, the patient described here may be the youngest case of LGI1 antibody encephalitis reported to date. Children with the LGI1 antibody-associated encephalitis may present only with single symptoms such as epileptic seizures and have good response to the therapy of immunoglobulin, methylprednisolone and antiepileptic drugs. Our case report will provide hints for pediatricians in the diagnosis and treatment of LGI1-antibody encephalitis.
ObjectiveTo examine the clinical effectiveness and tolerability of perampanel (PER) as initial monotherapy in pediatric patients with newly diagnosed focal epilepsy.MethodsA retrospective analysis was conducted on 62 children with newly diagnosed focal epilepsy who were treated with PER at the Epilepsy Center of Jinan Children's Hospital from July 2021 to July 2022. The treatment status, prognosis, and adverse reactions were followed up for a minimum of 6 months after the initiation of PER monotherapy. The effectiveness of the patients was estimated by the PER effective rate at 3-, 6-, and 12-month follow-up evaluations and adverse reactions were also recorded. The effective rates of PER in different etiologies and epilepsy syndromes were also statistically analyzed.ResultsThe effective rates of PER treatment at the different time points of evaluation were 88.7% (3 months), 79.1% (6 months), and 80.4% (12 months). With PER treatment, seizure freedom varied over time, with 61.3%, 71.0%, and 71.7% of patients at the 3-, 6-, and 12-month follow-ups, respectively. Among the etiologies of epilepsy, the effective rates of genetic etiology, structural etiology, and unknown etiology were generally above 50% at the 3-, 6-, and 12-month follow-ups. Among the epilepsy syndromes, the categories with higher treatment efficacy were self-limiting epilepsy with centrotemporal spikes (SeLECTs), self-limited epilepsy with autonomic seizures (SeLEAS), and childhood occipital visual epilepsy (COVE), with an effective rate of above 80%. Adverse events were documented in 22 patients (35.5%), but they were mild and tolerable. The most common adverse events comprised irritability, drowsiness, dizziness, and increased appetite.ConclusionPER has favorable effectiveness and tolerability as initial monotherapy for children with newly diagnosed focal epilepsy, which could be a potential option for long-term medication in the treatment of focal epilepsy in children. The current study provided potential evidence for PER as initial monotherapy in children with focal epilepsy in clinical practice.
Purpose: In this study, computed tomography (CT) radiographic measurements and common clinical scores were used to evaluate the effectiveness of percutaneous posterior full-endoscopic resection of an ossified thoracic ligamentum flavum. Methods: A prospective study was conducted on 16 patients treated with posterior endoscopy from September 2017 to November 2019. Before the operation, 3 days after the operation and 1 year after the operation, the area of ossification in the ligamentum flavum was assessed by sagittal CT scans and transected to evaluate the decompression effect of posterior endoscopic surgery. The clinical efficacy of the surgery was evaluated at the above time points by using the visual analog scale for pain, modified Japanese Orthopedic Association scale, ODI and Macnab efficacy evaluation. Results: The area of sagittal ossification in the ligamentum flavum in 16 patients was 116.62±32.72 mm2 before the operation, 15.99±12.54 mm2 3 days after the operation, and 16.78±11.49 mm2 1 year later. The sagittal canal invasive proportions were 48.10±10.04% before the operation, 6.46±4.86% 3 days after the operation, and 6.83±4.48% 1 year later. The area of transected ossification in the ligamentum flavum was 141.59±27.25 mm2 before the operation, 11.72±8.64 mm2 3 days after the operation, and 10.82±7.57 1 year later. The transected spinal canal invasive proportions were 57.58±11.37%, 4.76±3.45% and 4.40±3.01%. The mJOA score were 3.50±1.10, 6.19±0.91 and 9.19±1.38, with a n average recovery rate of 73.96±16.58%. According to the Macnab evaluation, the recovery status of the 16 patients 1 year after the operation was excellent in 9 patients, good in 5 patients, and fair in 2 patients; the excellent and good rate was 87.50%. The differences were statistically significant (P<0.05). Intraoperative dural tears occurred in 2 of 16 patients, but no complications, such as cerebrospinal fluid leakage, were observed. Conclusion:The measurements of ossification ligamentum flavum area and spinal canal invasive proportion can appropriately evaluate the degree of spinal canal stenosis in thoracic myelopathy caused by ossification of the ligamentum flavum. This method can be used together with other common clinical scores to better evaluate the efficacy of surgery.
After publication of this article [1], it is reported the below corrections need to be made with it.1. The following sentence need to be added to the end of 'Background' section:"This study was approved by the Institutional Ethics Committee of Qilu Children's Hospital of Shandong University and informed consent has been obtained from the guardian of patient prior to analysis." 2. 'Ethics approval and consent to participate' section in the back matter should be revised to:"This study was approved by the Institutional Ethics Committee of Qilu Children's Hospital of Shandong University and informed consent has been obtained from the guardian of patient prior to analysis." 3. 'Consent for publication' section in the back matter should be revised to:"All authors and the guardian of patient agreed for the publication of this study."The original article has been updated.
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