No abstract

SEEG is feasible and useful in children with drug-resistant insular epilepsy which is often characterized by autonomic symptoms as the initial symptoms and should be suspected in cases with HMS, asymmetric tonic seizures and even asymmetric spasms. Early propagation is mostly frontal and central. Analysis of a larger population is required to refine these findings.

Aim. We report our experience of stereoelectroencephalography (SEEG) in 65 children with drug‐resistant seizures, with a particular emphasis on young children. Methods. We retrospectively studied all SEEG performed between 2009 and 2011 in our centre. As SEEG can have several indications, the patients were classified into three categories, according to the probability of surgery. The contribution of SEEG to the final decision regarding surgery was evaluated for each category separately. We also compared the main demographic and surgical data of children younger than 5 years of age (Group 1; 21 children) with those older than five years of age at the time of investigation (Group 2; 44 patients). Results. MRI was not contributory in 20% of patients (9.5% in group 1; 25% in group 2). Electrical stimulations localised the motor area in all patients when performed (49% of patients), even in group 1 (62% of patients). SEEG led to surgery in 78% of patients (90.5% in group 1; 73% in group 2), after a second invasive investigation in 9.2 % of patients. The resection involved more than one lobe in 25% of patients (37% in group 1; 19% in group 2). Ultimately, 78% of patients with a low probability of having surgery before SEEG received surgery (88% in group 1). The surgical outcome of Engel class 1 was reported for 67% of patients (79% of patients in group 1 and 59% in group 2). No complications occurred. Conclusion. SEEG in children is safe and useful, and the surgical outcome in younger children is as good as, or sometimes even better than, that in older children. As a result of lower rates of complication and morbidity, SEEG appears to be more appropriate, in comparison to subdural grids, in situations where it is unclear if patients will have surgery after an invasive investigation.

No abstract

The authors report here 2 cases of subacute-onset encephalitis with N-methyl-D-aspartate (NMDA) receptor antibodies. One had a paraneoplastic syndrome associated with a neuroblastoma, whereas the other had no primary tumor. This disease was originally described as a paraneoplastic syndrome in young women with ovarian teratoma. The clinical features of both children resembled the typical symptoms reported for older patients with this disease: psychomotor deterioration, movement disorders, and seizures. One of the reported cases is the first known case of paraneoplastic encephalitis with NMDA antibodies in a child with neuroblastoma. Both cases described here were younger than any of the previously reported cases. Consistent with recently published series, this report suggests that the spectrum of symptoms of encephalitis with NMDA receptor antibodies is probably wider than previously thought.

Gadolinium-enhanced MRI provided additional information facilitating the diagnosis of vasculitis in a child with Lyme neuroborreliosis and stroke. The location of vessel wall enhancement was correlated with the topography of the acute infarct, and the lack of vessel lumen obstruction supported the diagnosis of vasculitis rather than any other cause.

No abstract

We studied four members of a family suffering from typical attacks of familial hemiplegic migraine (FHM) caused by a new mutation, R548C, of ATP1A2 gene in exon 12. One individual had also childhood absence epilepsy and generalized tonic-clonic seizures (GTCS). GTCS were followed by a severe attack of hemiplegic migraine at four times. Sodium valproate enabled control of both the epileptic seizures and the most severe FHM attacks. This association of FHM and epileptic seizures and their control with the same treatment suggest similar pathophysiological mechanisms.