Brain & development
-
Guillain-Barré syndrome and myasthenia gravis both lead to muscle weakness but the two combined is uncommon. Detection of these entities can help identify forms of autoimmune neuromuscular diseases that may respond to immunotherapy. This report sought to characterize the clinical features of these two entities when combined. ⋯ Detection of ptosis with or without ophthalmoplegia, distribution of limb weakness, and reflex can help in recognizing combined Guillain-Barré syndrome and myasthenia gravis. The early recognition of this combination of peripheral nervous and neuro-muscular junction inflammation is important for initial treatment and prognosis.
-
Focality in electro-clinical or neuroimaging data often motivates epileptologists to consider epilepsy surgery in patients with medically-uncontrolled seizures, while not all focal findings are causally associated with the generation of epileptic seizures. With the help of Hill's criteria, we have discussed how to establish causality in the context of the presurgical evaluation of epilepsy. The strengths of EEG include the ability to determine the temporal relationship between cerebral activities and clinical events; thus, scalp video-EEG is necessary in the evaluation of the majority of surgical candidates. ⋯ The significance of interictal HFOs should be assessed by taking into account the eloquent cortex, seizure-onset zone, and cortical lesions. Video-EEG and ECoG generally provide useful but still limited information to establish causality in presurgical evaluation. A comprehensive assessment of data derived from multiple modalities is ultimately required for successful management.
-
Brain & development · Sep 2013
Seizure outcomes of lesionectomy in pediatric lesional epilepsy with brain tumor -- single institute experience.
To determine the clinical characteristics, surgical strategy, and outcome in pediatric lesional epilepsy patients younger than 5years of age undergoing surgery in a single institute. ⋯ Lesionectomy in children younger than 5years of age is relatively safe and effective in controlling seizures. Short preoperative seizure periods and total removal of tumor might be associated with good outcome. Therefore, early and complete lesionectomy alone may help allow for seizure freedom and optimal brain development in pediatric patients.
-
Brain & development · Aug 2013
Review Case ReportsVGKC complex antibodies in pediatric severe acute encephalitis: a study and literature review.
Antibodies to surface proteins like voltage-gated potassium channel (VGKC) complexes are increasingly found in different neurologic diseases and encephalitis in adults and recently, in children. Detecting such antibodies can help identify forms of encephalitis that may respond to immuno-therapies. However, there are few reports on VGKC complex antibodies in pediatric severe acute encephalitis. ⋯ The incidence of VGKC complex antibodies in pediatric severe acute encephalitis is not high with only one (2.2%) of 46 children in this study. And, this is the first report on the association of VGKC complex antibodies and patients with influenza A-related severe acute encephalitis. The mechanism of VGKC complex antibodies in pediatric severe acute encephalitis warrants further study.
-
Brain & development · Aug 2013
Case ReportsA 14-year-old girl with hyperekplexia having GLRB mutations.
Hyperekplexia manifests as generalized stiffness and an excessive startle reflex to stimuli. It starts in the neonatal period and is transmitted in many cases via autosomal dominant inheritance. The etiology is an abnormality of the glycinergic neurotransmission system that is involved in inhibitory neurotransmission. ⋯ When patients have a persistent startle reflex, it is necessary to consider hyperekplexia, even if epileptic discharges are observed. Only four cases with GLRB mutations, including the present patient, have been reported. To make a definite diagnosis of hyperekplexia, it may be useful to screen for genes that are involved in the glycinergic neurotransmission system.