ObjectiveTo study the clinical and EEG features, therapeutic response and prognosis of eyelid myoclonia-nonconvulsive status epilepticus (EM-NCSE) in children.MethodsCollected the clinical and EEG data of 3 children with EM-NCSE that were diagnosed in department of neurology in Qilu Children Hospital of Shandong university during the January in 2015 to August in 2016.Analysed the therapeutic response to antiepletic drugs(AEDs).ResultsAmong the three children, there were 2 girls and 1 boy.The age at the onset of the disease was from 6 to 10 years old.The average age of them is 8.67 years old.The clinical manifestations include mental confusion, dysphoria, winking and scrolling up the eyes.The typical vedio electroencephalography (VEEG) in the patients showed 3~6 Hz generalized spike and waves and polyspikes burst, especially in the frontal and the anterior temporal region.In addition, the eye closure and intermittent photic stimulation helped to induce discharges and clinical events as eyelid myoclonia (EM).ConclusionsEM-NCSE is one of the idiopathic and generalized epileptic disease and characterized by EM.Video EEG monitoring plays an important role in the diagnosis of this disease.The drugs of choice for treatment was diazepam.When the event was controlled, AEDs were effective for the following therapy.
ObjectiveAicardi and Goutières syndrome was first reported as a rare hereditary encephalopathy with white matter involvement in 1984. Typical clinical manifestations include severe mental motor development retardation or regression, pyramidal and extrapyramidal symptoms and signs, epilepsy, microcephaly and frostbite.MethodsTo collect a case of patient who presented with convulsions 14 days after birth without obvious inducement. The child was diagnosed as epilepsy in the local hospital and the symptoms improved after treatment with antiepileptic drugs. At 4 months, the child presented nods and clenched fists, and was diagnosed as infantile spasm. After Adrenocorticotrophic hormone and drug treatment, the symptoms gradually improved. Due to upper respiratory track infection, the child was aggravated at the age of 1 year and 2 months, and then diagnosed as Aicardi-Goutières syndrome by video EEG, skull MRI, fundus and gene screening.ResultsSurgery and treatment with antiepileptic drugs significantly improved the symptoms of the child, and the pathological biopsy of the brain tissue supported the previous diagnosis.ConclusionsThe report of this case will help to improve the clinician's diagnosis and treatment of Aicardi-Goutières syndrome.
ObjectiveTo explore the clinical manifestation, diagnosis, treatment and prognosis of infantile spasm complicated with craniostenosis.MethodsA case of infantile spasm complicated with craniostenosis in the Department of Neurology of Qilu Children's Hospital in December 2017 was reviewed with the literature. The clinical manifestations, diagnosis, treatment and prognosis of infantile spasm with craniostenosis were analyzed.ResultsThe proband infantile spasms and craniostenosis was diagnosed by clinical, imaging examination and VEEG. Epileptic attack was prevented and craniostenosis was corrected by hormone shock therapy (corticotrophin was administered for 14 days, followed by topiramate)and surgical treatment (cranial cap reconstruction was performed), and good clinical prognosis was obtained.ConclusionThis case was the first reported case of craniostenosis with infantile spasm in China, and compared with the foreign treatment method, better treatment method and the operation opportunity were obtained. Which has a significant effect on the clinical treatment of infantile spasm complicated with transcranial disease.
ObjectiveTo investigate the efficacy and safety of Stereotactic electroencephalogram (SEEG)-guided Radiofrequency-thermocoagulation (RF-TC) in the treatment of refractory insular epilepsy in children.MethodsThe clinical data of 7 children with SEEG-confirmed insular epilepsy admitted to the Epilepsy Center of the Children’s Hospital Affiliated to Shandong University from January 2021 to May 2022, were retrospectively analyzed (3 males and 4 females; average age, 6.6±3.5 years). All patients underwent stage I pre-operative evaluation, and were implanted with SEEG electrodes for video EEG monitoring. The radiofrequency thermocoagulation contacts were determined according to SEEG and imaging results, and radiofrequency thermocoagulation was performed via electrode contacts. The patients were followed up at 3, 6, 12 and 18 months after operation by outpatient review or via telephone interview. The clinical efficacy was evaluated by Engel classification and complications were recorded. ResultsSix cases (6/7) were characterized by nocturnal seizures, and four cases (4/7) exhibited hypermotor or complex motor seizures. Three cases (3/7) showed focal ankylosis; only 1 patient had aura. All of the 7 cases showed interictal scalp EEG consistent with the side of surgery: 6 cases showed distribution in the perilateral fissure region, and 1 case showed confinement to the temporal region. In MRI, 4 cases showed negative signal, 2 cases showed unclear gray-white matter boundary, and 1 case showed thickening of the insular cortex. All of the 7 patients received electrode implantation and completed follow-up for over 6 months [6.0~22.0 (12.3±5.3) months]. At the last follow-up, 5 of the 7 children were seizure free (Engel class la), and 2 still had seizures after surgery, with no postoperative long-term complications.ConclusionChildren with insular epilepsy rarely show an aura, but have prominent motor symptoms, and the scalp electroencephalogram is mainly distributed in the perilateral fissured area. SEEG-guided RF-TC has good safety and efficacy in the treatment of drug‐resistant insular epilepsy.