Cognitive and behavioral outcome of stereotactic laser amydalohippocampotomy in a pediatric setting.

We present neuropsychological and functional outcome data in a teenager undergoing stereotactic laser amygdalohippocampotomy (SLAH) who had drug-resistant mesial temporal lobe epilepsy due to left hippocampal sclerosis. Given strong baseline cognitive performance, there was concern for post-operative declines in language and verbal memory were this patient to undergo open resection. She was evaluated pre- and post-ablation with clinical and experimental neuropsychological measures including semantic memory, category-specific object/face recognition and naming, spatial learning, and socio-emotional processing. The patient became seizure-free following SLAH and experienced significant improvements in school performance and social engagement. She experienced improvement in recognition and naming of multiple object categories, memory functions, and verbal fluency. In contrast, the patient declined significantly in her ability to recognize emotional tone from facial expressions, a socio-emotional process that had been normal prior to surgery. We believe this decline was related to surgical disruption of the limbic system, an area highly involved in emotional processing, and suspect such deficits are an under-assessed and unrecognized risk for all surgeries involving the amygdalohippocampal complex and broader limbic system regions. We hope this positive SLAH outcome will serve as impetus for group level research to establish its safety and efficacy in the pediatric setting.

Diverse seizure presentation of acute Mycoplasma pneumoniae encephalitis resolving with immunotherapy.

We report 3 previously normal children that presented for evaluation of new onset seizures. Case 1, a 7-year-old female, presented with refractory left frontal lobe seizures associated with right arm simple motor seizures refractory to 6 antiepileptic medications at sufficient doses and levels. Case 2, a 15-year-old female, presented with left frontotemporal lobe seizures and nonconvulsive seizures, associated with neuropsychiatric symptoms refractory to 5 antiepileptic medications. Both patients received intravenous steroids and intravenous immunoglobulin. Case 3, an 11-year-old male, presented with a generalized tonic clonic seizure and worsening hallucinations responding to intravenous corticosteroids and 1 antiepileptic medication. All 3 patients had extensive infectious and metabolic evaluation and were found to be serum immunoglobulin M positive for mycoplasma pneumoniae. Despite their prolonged severe symptoms, all patients had virtually complete recovery with excellent seizure control after aggressive seizure management with immunotherapy and antiepileptic medication.

Seizures temporally associated with nitrous oxide administration for pediatric procedural sedation.

Nitrous oxide is an inhaled agent commonly used by dental staff to provide anxiolysis and analgesia for dental procedures and by anesthesia personnel as an adjunct to more potent general anesthetic gases. More recently, nitrous oxide has been used to provide sedation/analgesia for a variety of medical procedures in children outside of the operating room, including lumbar puncture, laceration repair, fracture reduction, and urologic imaging. We report 3 cases of clinical seizure activity associated with nitrous oxide administration for pediatric procedural sedation. Although temporally related, no causality is established. Review of the medical and dental literature confirm the rarity of these events.

Magnetic resonance imaging (MRI) and electroencephalographic (EEG) findings in a cohort of normal children with newly diagnosed seizures.

In the initial assessment of children with new-onset seizures, the suggestion that electroencephalography (EEG) should be standard and that magnetic resonance imaging (MRI) should be optional has been questioned. The purposes of this study were to (1) describe the frequency of EEG and MRI abnormalities and (2) explore relationships between MRI and EEG findings to determine their relevance in the assessment of children with new-onset seizures who are otherwise developing normally. As part of an ongoing, prospective study of children with new-onset seizures, we studied 181 children (90 girls and 91 boys). Children were entered into the study within 3 months of their first-recognized seizure. The association between EEG and MRI abnormalities was explored using a chi-square test. Abnormal MRI findings were found in 32.6% (n = 59) of the sample. The EEG and MRI results agreed with respect to classification into normal or abnormal in 37% (n = 67). Of the 50 children with a normal EEG, however, 21 (42%) were found to have an abnormal MRI. We found an unexpectedly high frequency of imaging abnormalities in our sample of otherwise normal children, although the significance of these findings is not clear. Follow-up of these patients will help us interpret the importance of the abnormalities. Despite our relatively small sample, however, our findings indicate that a normal EEG does not reliably predict a normal MRI in children with first seizures.

MR imaging and spectroscopy in juvenile Huntington disease.

Juvenile Huntington disease manifests differently from adult Huntington disease and has more variability in presentation. We describe a child with cognitive decline and adventitial movements in whom Huntington disease was confirmed with genetic testing. MR imaging showed abnormal T2 prolongation in the putamina and progressive caudate atrophy, and MR spectroscopy revealed elevated myoinositol and diminished N-acetyl aspartate, creatine, and phosphocreatine. Imaging findings of caudate atrophy and abnormal T2 prolongation in the putamina with MR spectroscopy findings consistent with dense gliosis can be helpful indicators of juvenile Huntington disease.