Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency.

BACKGROUND: Encephalitis lethargica is an encephalitic illness with multiple nervous system symptoms. Lesions only involving substantia nigra on magnetic resonance imaging are uncommon, especially in children. A second encephalitis illness after encephalitis lethargica has never been reported before. PATIENT DESCRIPTION: We describe a 7-year-old boy with humoral immunity deficiency who developed encephalitis lethargica associated with bilateral substantia nigra lesions on magnetic resonance imaging. After a nearly complete recovery, he developed encephalitis once again. He was diagnosed with encephalitis lethargica with somnolence, akinetic mutism, and ophthalmoplegia after intermittent fever. Cerebrospinal fluid pleocytosis and positive oligoclonal bands were documented. Symmetrical substantia nigra lesions on high-intensity magnetic resonance imaging gradually evolved into a liquid signal. He had almost recovered when he developed fatigue and hypersomnia and was diagnosed with encephalitis again, supported by mild pleocytosis in cerebrospinal fluid and subcortical white matter lesions in the frontal lobes. His symptoms resolved following administration of corticosteroids and immunoglobulins. CONCLUSION: This is the first report of an immune-deficient child to develop encephalitis lethargica with isolated substantia nigra lesions on magnetic resonance imaging and a second encephalitis illness after recovery from encephalitis lethargica.

Epileptic EEG classification based on kernel sparse representation.

The automatic identification of epileptic EEG signals is significant in both relieving heavy workload of visual inspection of EEG recordings and treatment of epilepsy. This paper presents a novel method based on the theory of sparse representation to identify epileptic EEGs. At first, the raw EEG epochs are preprocessed via Gaussian low pass filtering and differential operation. Then, in the scheme of sparse representation based classification (SRC), a test EEG sample is sparsely represented on the training set by solving l1-minimization problem, and the represented residuals associated with ictal and interictal training samples are computed. The test EEG sample is categorized as the class that yields the minimum represented residual. So unlike the conventional EEG classification methods, the choice and calculation of EEG features are avoided in the proposed framework. Moreover, the kernel trick is employed to generate a kernel version of the SRC method for improving the separability between ictal and interictal classes. The satisfactory recognition accuracy of 98.63% for ictal and interictal EEG classification and for ictal and normal EEG classification has been achieved by the kernel SRC. In addition, the fast speed makes the kernel SRC suit for the real-time seizure monitoring application in the near future.

[Influence of ketogenic diet on the clinical effects and electroencephalogram features in 31 children with pharmacoresistant epileptic encephalopathy].

OBJECTIVE: To investigate the effect of ketogenic diet (KD) on the clinical and electroencephalogram features in children with pharmacoresistant epileptic encephalopathy. METHOD: Thirty-one children (19 boys, 12 girls) aged 7 months to 7 years (mean 2 years 5 month) with epilepsy refractory to conventional antiepileptic drugs (AEDs) were included in this study. In addition to their original AED treatment, the children were assigned to different ketogenic diets based on their age. The prospective electro-clinical assessment was performed prior to the KD and then one week, one month and again 3 months after the initiation of therapy, respectively. RESULT: The reduction of seizure frequency in 52%, 68% and 71% of all patients exceeded 50% one week, one month and three months after KD treatment respectively. KD is particularly effective in myoclonic astatic epilepsy (MAE; Doose Syndrome) and West syndrome with 100% and 81.25% of the patients having a greater than 50% seizure reduction, respectively. After 3 months of KD treatment, more than 2/3 patients experienced a reduction in interictal epileptiform discharges (IEDs) and improvement in EEG background. CONCLUSION: The clinical and electroencephalographic improvement confirms that KD is beneficial in children with refractory epilepsy.

Effects of ketogenic diet on the clinical and electroencephalographic features of children with drug therapy-resistant epilepsy.

The aim of this study was to investigate the effects of a ketogenic diet (KD) on the clinical and electroencephalographic (EEG) features of children with drug therapy-resistant epilepsy. A total of 31 children with drug therapy-resistant epilepsy were selected, including 19 males and 12 females. The youngest was 7 months old and the oldest was 7 years old. Clinical seizures in the children prior to and 1 week, 1 month and 3 months after the initiation of the KD were compared and the clinical effect of the KD was evaluated. The ratio of fat to carbohydrate + protein in the KD was 4:1. Following the initiation of the KD treatment, the original antiepileptic drugs were not changed. The changes in occipital region background rhythm and interictal spike wave discharge index (SI) were evaluated prior to and 1 week, 1 month and 3 months after the initiation of the KD. The efficacy had an upward trend over time, with a total efficacy rate of 51.61% 1 week later, 67.74% 1 month later and 70.97% 3 months later. Doose syndrome showed the greatest response to KD, with a 100% efficacy rate. However, since there were only two cases in the study, its efficacy remains to be assessed. Infantile spasm also showed a good response to KD; 9 of the 16 patients were seizure free and the total efficacy rate was 81.25%. As the length of the KD treatment was increased, the background rhythms of the children underwent significant changes and the SI was significantly reduced. KD not only demonstrated good clinical efficacy, but also significantly reduced the frequency of interictal epileptic discharges and improved the EEG background rhythm.