Rasmussen’s encephalitis (RE) is a rare neurologic disorder, with an incidence of 0.18 per 100,000 population, primarily affecting children, with an average onset at 6-7 years of age. Clinical manifestations include focal refractory epilepsy, progressive neurological deterioration, and cognitive decline. In imaging, magnetic resonance imaging (MRI) typically shows an increase in volume and sequences with T2/fluid-attenuated inversion recovery (FLAIR) signal, followed by atrophy. Positron emission tomography (PET) demonstrates a decline in metabolism in one hemisphere. Pathologically, neuronal loss, perivascular lymphocytic cuffing, and small glial nodules are prominent, with 10% of cases exhibiting dual pathology, primarily cortical dysplasia. Functional hemispherectomy remains the only therapeutic option, albeit resulting in permanent motor and cognitive deficits. Immunomodulatory therapy provides only temporary relief. Currently, the etiology and pathogenesis of RE remain unclear, presenting three major challenges: early diagnosis before hemisphere atrophy and neurocognitive impairment, managing immune therapies targeting inflammation, and determining rehabilitation post-surgery to maximize neurological recovery. Emerging evidence suggests that alterations in the brain’s immune microenvironment play a pivotal role in disease progression. This article focuses on the immunopathological aspects of RE, elucidating the roles of T lymphocytes, small glial cells, and astrocytes in the development of RE.
Epilepsy is a complex and widespread neurological disorder that has become a global public health issue. In recent years, significant progress has been made in the use of wearable devices for seizure monitoring, prediction, and treatment. This paper reviewed the applications of invasive and non-invasive wearable devices in seizure monitoring, such as subcutaneous EEG, ear-EEG, and multimodal sensors, highlighting their advantages in improving the accuracy of seizure recording. It also discussed the latest advances in the prediction and treatment of seizure using wearable devices.
ObjectiveAnalyzing the seizure and cognitive outcome after different treatment by observation of a large group of intractable child epilepsy patients under 15 years old. MethodsCollecting data of children with Intractable epilepsy from Apirl 2008 to December 2013 in Sanbo Brain Hospital, Capital Medical University. Three historical cohorts of intractable child epilepsy defined by the final treatment including medication, curative operation and palliative operation depending on the surgical assessment and the families intension was retrospectively observed. 1 year and 3 years follow-up postoperatively were conducted including seizure outcome and cognitive outcome. ResultsThe curative operation group had significant better seizure free rate, and cognitive statement than medication group. And, the seizure free and cognitive outcome were better in palliative operation group than the medication group. ConclusionsEarly surgical intervention is highly recommended for intractable epilepsy chilelren in order to improve both the seizure and cognitive prognosis.
ObjectiveTo explore the clinical features and EEG features of gelastic seizures, and analyze its value of lateral localization of epileptogenic area. MethodsAll patients with gelastic seizures admitted to the Sanbo Brain Hospital of Capital Medical University between January 2014 and December 2023 were reviewed and analyzed for history, symptomatology, imaging, electroencephalographic features and surgical protocols in patients who met the inclusion criteria and were followed up for at least 1 year, and surgical efficacy was assessed by using the Engel grading. ResultsA total of 51 patients with gelastic seizures were included, there were 32 (62.75%) males and 19 (37.25%) females, 21 (41.18%) with hypothalamic hamartomas (HH) and 30 (58.82%) with non-hypothalamic hamartomas. The age of onset was earlier in the HH group than in the non-HH group, with a median age of onset of 24.00 (0.00 ~ 96.00) and 78.00 (1.00 ~ 396.00) months (P<0.001). There are three types of laughter according to their characteristics: smiling or pleasant expressions, laughing out loud, crying or bitter laughter, with smiling or pleasant expressions being the most common (49.02%). Simple laughter is rare in all patients and is often accompanied by other manifestations such as autonomic symptoms, automatic movements, complex movements, and tonic seizures. Most of the HH group started with laughter whereas in the non-HH group laughter appeared mostly in the mid to late stages (P=0.007). Most of the HH group (57.14%) had preserved consciousness whereas most of the non-HH group (83.33%) had loss of consciousness (P=0.003). The interictal discharges in the HH group were mostly diffuse or multiregional, whereas those in the non-HH group were mostly regional (P=0.035). The onset of EEG during the seizure period in the HH group was mostly diffuse, whereas those in the non-HH group were mostly regional, mainly in the frontal and temporal regions, but there was no significant difference between the two groups (P=0.148). The non-HH group was mostly seen in those with definite lesions, and the most common type of lesion was FCD (focal cortical dysplasia, FCD). All patients enrolled in the group underwent surgical treatment, and stereoelectroencephalogram (SEEG) electrode implantation was performed in 13 cases in the HH group and in 17 cases in the non-HH group. 61.90% of the patients in the HH group had an Engel grade I, and 73.33% of the patients in the non-HH group had an Engel grade I. ConclusionsGelastic seizures has a complex neural network, with common causes other than hypothalamic hamartomas, and is most commonly seen in frontal or temporal lobe epilepsy, as well as in the insula or parietal lobe, with the most common type of lesion being FCD. The symptomatology, stage of onset, and electroencephalographic features of gelastic seizures can help in the differential diagnosis, and SEEG can help define the origin of the seizure and its diffusion pathway. The overall prognosis of surgical treatment was better in both the hypothalamic hamartomas and non-hypothalamic hamartomas groups.