Contralateral head leading turning accompanied by ipsilateral eye staring in a patient with seizure onset from posterior inferior temporal sulcus, a SEEG case report

The epileptic eye and head movements during epileptic seizures may be much more complicated thanpeople originally understood, which can be ipsilateral or contralateral to the electroencephalographyfocus. Here, we describe a male patient with drug resistant focal seizures associated with a directionalseparation between head and eye movement before evolving into generalized tonic-clonic seizure.His contralateral head leading turning showed forced, sustained, and unnatural features companiedby ipsilateral eye staring. Stereoeletroencephalography monitoring was performed, and 4 habitualseizures were recorded over 5 days. Three seizures showed left head leading turning and generalizedtonic-clonic seizure, and only one showed dizziness and ringing in the ears. All the seizures showedthat the ictal onset contacts were located in the posterior inferior temporal sulcus which borders onthe anterolateral part of medial superior temporal area. The patient underwent a resection includingtemporooccipital region, and the histopathology showed focal cortical dysplasia type Ic. He has beenseizure free for two years after operation. The scores of the intelligence and memory quotient improvedhalf year after operation.

Treatment of epilepsy with bipolar electro-coagulation: an analysis of cortical blood flow and histological change in temporal lobe / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Bipolar electro-coagulation has a reported efficacy in treating epilepsy involving functional cortex by pure electro-coagulation or combination with resection. However, the mechanisms of bipolar electro-coagulation are not completely known. We studied the acute cortical blood flow and histological changes after bipolar electro-coagulation in 24 patients with intractable temporal lobe epilepsy.</p><p><b>METHODS</b>Twenty-four patients were consecutively enrolled, and divided into three groups according to the date of admission. The regional cortical blood flow (rCBF), electrocorticography, the depth of cortex damage, and acute histological changes (H and E staining, neuronal staining and neurofilament (NF) staining) were analyzed before and after the operation. The t-test analysis was used to compare the rCBF before and after the operation.</p><p><b>RESULTS</b>The rCBF after coagulation was significantly reduced (P < 0.05). The spikes were significantly reduced after electro-coagulation. For the temporal cortex, the depth of cortical damage with output power of 2-9 W after electro-coagulation was 0.34 ± 0.03, 0.48 ± 0.06, 0.69 ± 0.06, 0.84 ± 0.09, 0.98 ± 0.08, 1.10 ± 0.11, 1.11 ± 0.09, and 1.22 ± 0.11 mm, respectively. Coagulation with output power of 4-5 W completely damaged the neurons and NF protein in the molecular layer, external granular layer, and external pyramidal layer.</p><p><b>CONCLUSIONS</b>The electro-coagulation not only destroyed the neurons and NF protein, but also reduced the rCBF. We concluded that the injuries caused by electro-coagulation would prevent horizontal synchronization and spread of epileptic discharges, and partially destroy the epileptic focus.</p>

The clinical and EEG features and mutation analysisin a Chinese patient with severe hypoplasia of the cerebellum and pons

We report here a Chinese female infant with severe hypoplasia of the cerebellum and pons, and heterozygous mutation (c.18G >T, p.E6D) in the TSEN54 gene. This mutation was not present in her parents and the 100 Chinese controls, which proved to be a de novo missense mutation. MR imaging of the patient revealed severe hypoplasia of the bilateral cerebellar hemispheres and vermis with moderate flattening of the pons. A video EEG during hospitalization demostrated abnormal background activities and generalized burst and attenuation patterns during interictal stage. The spasms and tonic spasms occurred frequently in clusters with generalized voltage attenuation.

Treatment of drug-resistant epilepsy with vagus nerve stimulation -- review of 45 cases / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Vagus nerve stimulation (VNS) is an alternative treatment for drug-resistant epilepsy (DRE). The study aimed to explore the potential factors of prognosis, safety and effect of VNS treatment in patients with DRE.</p><p><b>METHODS</b>We retrospectively examined 45 cases of DRE that received VNS treatment in our center from June 2004 to June 2010 and analyzed the parameters (age of patient receiving VNS, seizure frequency before and after VNS as well as treatment duration) by Student's t test, Fisher's exact and Mann-Whitney U tests, and multivariate Logistic regression.</p><p><b>RESULTS</b>The overall response rate was 64% (29/45), 67% (6/9) for adults and 64% (23/36) for children, with no significant difference (P = 0.28). Twenty-two cases had been in VNS therapy for over 1 year with a treatment efficacy of 73% (16/22), whereas 23 cases had been in VNS therapy no more than 1 year with a treatment effecacy of 57% (13/23), and has statistically significant difference (P = 0.03). The main side effect included hoarseness of voice and cough. One patient's device was removed due to infection. One patient's VNS was half-way terminated due to seizure aggravation. One patient died due to status epilepticus.</p><p><b>CONCLUSIONS</b>VNS is a safe and effective treatment for DRE. Duration of VNS therapy may be a crucial factor on prognosis.</p>

Pediatric intractable epilepsy surgery / 中华外科杂志

<p><b>OBJECTIVE</b>To explore the strategy of pediatric intractable epilepsy surgery.</p><p><b>METHODS</b>The clinical data of 96 pediatric cases with intractable epilepsy and epilepsy syndromes underwent surgical treatment from April 2004 to April 2006 were retrospectively analyzed.</p><p><b>RESULTS</b>The surgical treatments were performed based on the results of comprehensive data from neurological, neurosurgical and pediatric departments. Among of them, 78 cases were performed with curative procedure, 17 cases with palliative procedure and 1 case with stereotactic damage procedure. The surgical effect was judged with Engel's standard, 58 cases had no seizure during 14 to 26 months follow-up, 26 cases had significantly improved in seizure control and the total efficiency was 87.5%. 81 cases had improvements in neuropsychological tests. 22 cases had postoperative complications such as neuro-dysfunctions and 15 cases were gradually recovered after the period of follow-up ended, 1 case died of CSF over drainage after operation 3 months.</p><p><b>CONCLUSIONS</b>Pediatric patients with symptomatic epilepsy and epilepsy syndromes are suitable to surgical treatment, the results are satisfactory in seizure control and neuropsychological function tests.</p>

Clinical characteristics and treatment of Rasmussen syndrome in 16 children / 中华儿科杂志

<p><b>OBJECTIVE</b>Rasmussen syndrome (RS) is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. The present study aimed to analyze the electroclinical characteristics and treatment of RS.</p><p><b>METHODS</b>The medical records of 16 children with RS were retrospectively reviewed.</p><p><b>RESULTS</b>Of the 16 children, 8 were males and 8 were females. The age of onset was from 1 year and 11 months to 11 years and 6 months. The first symptom was seizure in all patients. The main seizure type was partial motor seizures. In all the patients, seizures gradually became frequent and in the form of epilepsia partialis continua (EPC). Thirteen cases developed hemiparesis. Fixed hemiparesis occurred from 2 months to 3 years after the onset of seizures. The cognitive deterioration was present in 14. The EEG background activity was abnormal in all the cases, asymmetric slow wave disturbances were bilateral but with unilateral predominance in 11, unilateral delta or theta wave in 8. The presence of interictal epileptiform discharges were found in all cases, unilateral in 11 and bilateral in 5. Seizures were recorded in all patients, no electroclinical correlation was found in 5. Serial magnetic resonance imaging (MRI) showed progressive unihemispheric or focal cortical atrophy in all cases. Six cases transiently showed focal cortical swelling or T2/FLAIR hyperintense signal on early scans. Antiepileptic drugs were not effective in any of the patients. Three of 10 patients receiving immunoglobulin, and 4 of 8 receiving corticosteroids, had some reduction of seizure frequency for a short period. Six patients accepted functional hemispherectomy, in 4 of them seizure no longer occurred and cognitive function was improved. The results of multiple subpial transection in 2 cases and focal resection in one patient were disappointing.</p><p><b>CONCLUSION</b>The clinical features of RS were refractory partial epilepsy, progressive hemiplegia and cognitive deterioration. The EEG background was asymmetric with slow wave activity, interictal epileptiform discharges were unilateral or bilateral, no electroclinical correlation occurred. Serial MRI showed progressive unihemispheric focal cortical atrophy. Antiepileptic drugs were not effective for RS. In some patients, immunoglobulin or corticosteroids could reduce seizure frequency in the short term. Functional hemispherectomy could lead to seizure control and prevent further development of neurological impairment and cognitive deterioration.</p>

Psychiatric and central nervous system involvement in Sjogren's syndrome / 中华口腔医学杂志

<p><b>OBJECTIVE</b>To study psychiatric features and the manifestations of central nervous system involvement in Chinese patients with primary Sjogren's syndrome (PSS).</p><p><b>METHODS</b>The Minnesota Multiphasic Personality Inventory (MMPI) was used to study 27 PSS female patients and 57 healthy women. The results from two groups were compared. Ten SS patients were evaluated by electroencephalography (EEG), transcranial doppler ultrasound (TCD) and magnetic resonance imaging (MRI).</p><p><b>RESULTS</b>Of 27 patients tested, the three highest clinical mean scales included Hypochondriasis (Hs), Hysteria (Hy), Psychasthenia (Pt) were found. When PSS group was compared with normal control, T scores of SS patients were significantly higher than the healthy women in Hs, Hy. Abnormal EEG was found in 3 of 9 PSS patients. TCD was abnormal in 5 of 9 patients. MRI in 1 patient showed abnormality.</p><p><b>CONCLUSIONS</b>This study shows that the personality of PSS patients is abnormal, the features are neuroticism. Central nervous system was markedly involved in Chinese patients with PSS, including abnormal EEG and TCD. The changes of central nervous system may be related to abnormalities of psychiatric changes.</p>

Treatment of intractable epilepsy with hemispherectomy / 中华外科杂志

<p><b>OBJECTIVE</b>To explore the clinical effectiveness of hemispherectomy for patients with intractable epilepsy.</p><p><b>METHODS</b>Thirty-six patients with intractable epilepsy were treated by modified hemispherectomy and their clinical results were followed up after operation.</p><p><b>RESULTS</b>The seizures of the 36 patients were effectively controlled and 34 (94.4%) were thoroughly controlled. Meanwhile, the dysfunction of the central nervous system in 30 patients (83.3%) was aggravated, and 5 patients (13.9%) showed better improvement than before. Neuropsychological assessment revealed much improvement in the patients.</p><p><b>CONCLUSIONS</b>Modified hemispherectomy is advisable for patients with wide-range lesion of the half-brain associated with intractable epilepsy.</p>