In neonatal CSVT, regional antithrombotic treatment practices demonstrate considerable variability and uncertainty about indications for antithrombotic therapy. Additional studies are warranted.

Resective pediatric epilepsy surgery in Lennox-Gastaut syndrome.

Pediatrics. 2010 Jan;125(1):e58-66

Authors: Lee YJ, Kang HC, Lee JS, Kim SH, Kim DS, Shim KW, Lee YH, Kim TS, Kim HD

OBJECTIVE: The objective of this study was to evaluate the role of resective pediatric epilepsy surgery for Lennox-Gastaut syndrome (LGS). METHODS: We analyzed clinical data of 27 children and adolescents who had LGS and underwent resective epilepsy surgery despite abundant (>30% of preoperative interictal and/or ictal epileptiform discharges) generalized or generalized contralateral maximal and multiregional electroencephalogram abnormalities. RESULTS: On high-resolution MRI, cerebral lesions were noted in 23 (85.2%) patients but not in 4 (14.8%) patients. The age of patients at the time of surgery was between 1.7 and 17.3 years (mean: 7.8 years). Surgeries were lobar or multilobar resection in 21 (77.8%) patients and hemispherotomy in 6 (22.2%). At a mean of 33.1 months' postoperative follow-up, 16 (59.3%) patients had no seizures and 4 (14.8%) had infrequent seizures. Of 4 patients without brain abnormalities found on MRI, 2 patients became seizure-free after resective surgery was performed on the basis of electrophysiologic studies and concordant results in other multimodal neuroimages. Malformation of cortical development was the most common pathology and was seen in 20 (74.1%) patients, but 2 (7.4% patients) did not show any abnormal pathology. Sixteen (72.7%) patients, including 14 who had no seizures and 2 who had infrequent seizures after surgery, showed an increase in developmental quotient. No clinical profile was significantly associated with postoperative seizure-free rate. CONCLUSIONS: Resective epilepsy surgery should be considered for children with LGS, despite abundant generalized and multiregional electroencephalogram abnormalities.

PMID: 20008422 [PubMed - in process]

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Surgery for extratemporal nonlesional epilepsy in children: a meta-analysis.

Childs Nerv Syst. 2009 Dec 15;

Authors: Ansari SF, Maher CO, Tubbs RS, Terry CL, Cohen-Gadol AA

PURPOSE: Previous small studies have demonstrated that seizure outcomes following surgery for extratemporal lobe epilepsy (ETLE) in children are worse than those for temporal lobe epilepsy. We have conducted a meta-analysis of the available literature to better understand ETLE surgical outcomes in children. METHODS: We searched PubMed (1990-2009) for appropriate studies using the following terms: ETLE, ETLE surgery, ETLE surgery outcome, frontal lobe epilepsy, occipital lobe epilepsy, and parietal lobe epilepsy. Our collected data included patient age at seizure onset and surgery, the cerebral lobe involved with epileptogenesis, MRI findings, predominant seizure semiology, intracranial monitoring use (electrode implantation), epileptic region histopathology, and postoperative seizure outcome. Statistical analysis was performed to determine associations among these variables and postoperative outcome. RESULTS: Ninety-five patients from 17 studies satisfied the inclusion criteria. Pathological findings (p = 0.039) and seizure type (p = 0.025) were significantly associated with outcome: A larger proportion of patients with cortical dysplasia and complex partial seizures experienced better outcomes. Age at surgery (p = 0.073) and the cerebral resection site (p = 0.059) were marginally associated with seizure outcome. CONCLUSIONS: This study confirms previous reports: Surgical outcomes for ETLE epilepsy are significantly worse than those for temporal lobe epilepsy. The reasons for this difference may include the diffuse nature of the pathology involved in ETLE, difficulty in localizing the seizure focus in young children, and involvement of "eloquent" nonresectable cortex in epileptogenesis. Because of the reporting variability among different epilepsy centers, more uniform protocols are necessary for fair evaluation and comparison of outcomes among the different centers.

PMID: 20013124 [PubMed - as supplied by publisher]

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Cost-utility analysis of rufinamide versus topiramate and lamotrigine for the treatment of children with Lennox-Gastaut Syndrome in the United Kingdom.

Seizure. 2009 Nov 24;

Authors: Verdian L, Yi Y

PURPOSE: To estimate the cost-effectiveness of rufinamide relative to topiramate and lamotrigine as adjunctive treatment for children with Lennox-Gastaut Syndrome (LGS). METHODS: A Markov decision analytic model was developed to estimate the incremental cost-effectiveness ratio over a three-year time horizon in patients with LGS uncontrolled by up to three antiepileptic drugs. Utilities were assigned to health states, defined according to a patient's response to treatment (>/=75%, >/=50% and <75%, and <50% reduction in tonic-atonic [drop attack] seizure frequency and death). Efficacy and safety estimates were made using indirect/mixed-treatment comparisons of data obtained from published literature. Outcomes included costs and quality-adjusted life-years (QALYs), allowing the incremental cost-effectiveness ratio to be estimated as cost per QALY gained. RESULTS: Over three years, the total cumulative costs for rufinamide, topiramate, and lamotrigine were pound24,992, pound23,360, and pound21,783, respectively. Rufinamide resulted in an incremental QALY gain of 0.079 relative to topiramate and 0.021 relative to lamotrigine. The incremental costs of rufinamide were pound1632 and pound3209, relative to topiramate and lamotrigine, resulting in an incremental cost per QALY gained of pound20,538 and pound154,831, respectively. CONCLUSIONS: Considering the underlying assumptions, this current economic evaluation demonstrates that rufinamide is likely to be a cost-effective alternative to topiramate as adjunctive treatment for children with LGS in the UK. In addition, when compared to lamotrigine, which is an inexpensive treatment, rufinamide should be considered as a cost-effective alternative due to the importance of patient choice and equity of access in such a rare and devastating condition.

PMID: 19942457 [PubMed - as supplied by publisher]

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Prevalence of epileptiform discharges in healthy children-New data from a prospective study using digital EEG.

Epilepsia. 2009 Dec 1;

Authors: Borusiak P, Zilbauer M, Jenke AC

Summary Purpose: Data on epileptiform electroencephalography (EEG) discharges in healthy children are limited, with published studies dating back more than 20 years. Moreover, analyses have been performed exclusively using paper-recorded EEG, and reported prevalences differ significantly. With recent reports using these data as reference suggesting an increased prevalence of epileptiform EEG discharges in children with behavioral disturbances, acquisition of exact prevalence data has become even more critical. The aim of our study was to analyze the frequency of epileptiform EEG discharges in healthy children using digitally recorded EEG (DEEG) and to compare these data to those of previously published studies. Methods: Prospective analysis of DEEG was performed in 382 healthy children (226 male, 156 female) ages 6-13 years admitted to our hospital for minor head trauma. Recording was carried out for a minimum of 20 min including hyperventilation and photic stimulation. Analysis was carried out by two board-certified clinical neurophysiologists. Results: Epileptiform EEG discharges were detected in 25 of 382 children (11 of 226 male, 14 of 156 female) corresponding to an overall prevalence of 6.5%. Of these 25 children, 4 had either generalized or bifrontal spikes, 12 showed constant localized focal discharges, and 9 showed multifocal discharges. Compared to previous studies using non-DEEG recording, the prevalence of epileptiform EEG discharges in our population was significantly higher. No significant difference was found when comparing our data to prevalences recently reported in children with behavioral disturbances using DEEG. Conclusions: Our study further highlights the urgent need to reevaluate the prevalence of epileptiform EEG discharges in healthy children using DEEG recordings in a large cohort.

PMID: 20002145 [PubMed - as supplied by publisher]

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An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children.

Childs Nerv Syst. 2009 Dec 1;

Authors: Vanlandingham M, Hanigan W, Vedanarayanan V, Fratkin J

PURPOSE: This study determined the statewide incidence and prevalence of acute disseminated encephalomyelitis (ADEM) and examined the course of three pediatric patients treated for tumefactive demyelination (TD) at the Blair E. Batson Children's Hospital. METHODS: Analyses of ICD-9-CM code hospital records and clinical database were conducted. RESULTS: From 2001 through 2007 the incidence in pediatric patients under 20 years was 0.4/100,000/year, with a prevalence of 8.6/100,000 during 2008. Three patients presented with TD. Case 1 had a 3-week history of ataxia and diplopia; case 2 presented with a sudden onset of coma, while the third child had a 4-month history of increasing lethargy and clumsiness in all extremities. Cerebrospinal fluid examinations were nondiagnostic. MRI examinations revealed asymmetric T2/fluid-attenuated inversion recovery hyperintensity within the pons (case 1), a large heterogenously enhancing temporal lobe mass, with extensive edema (case 2), and multiple small brain lesions with occasional ring enhancement (case 3). In case 1, intralesional MR spectroscopy demonstrated changes consistent with ADEM. Case 2 required intracranial monitoring, and medical treatment to control elevated ICP. Cases 2 and 3 underwent cortical biopsies that revealed ADEM. All three patients improved with corticosteroid therapy. At a minimum of 15 months follow-up, cases 1 and 2 showed resolution of deficits and MRI lesions, while the third patient demonstrated additional MRI lesions and increasing paraparesis. CONCLUSIONS: These cases demonstrate that appropriate neuroradiological evaluation, treatment of acutely elevated ICP, and brain biopsy can play critical roles in the management of children with undiagnosed ADEM and TD.

PMID: 19949803 [PubMed - as supplied by publisher]

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Elizabeth H Tham,MBBS, Stacey KH Tay,MBBS, MRCP (Paed), MRCPCH, Poh Sim Low,MBBS, FRCPCH, FRACP

Introduction: While paediatric strokes are fairly uncommon, they are often associated with signifi cant long-term disability. Diagnosis is often delayed because of the need to exclude conditions that mimic stroke. Understanding the outcomes related to stroke in children is important in the development of secondary prevention strategies. The aim of this study was to evaluate the epidemiology of childhood stroke in a tertiary paediatric unit in Singapore and to assess factors infl uencing outcome in these children. Materials and Methods: A retrospective casenote
review of all childhood strokes presenting to the Children’s Medical Institute (CMI) at the National University Hospital (NUH), Singapore between October 1999 and May 2006. Data collected include demographic factors, clinical presentation, diagnosis, subsequent management and follow-up using specifi c outcome measures. Results: Twenty-six children with a median age of 8.0 years at presentation were identifi ed, comprising 15 ischaemic strokes (57.7%), 10 haemorrhagic strokes (38.5%) and 1 patient with both ischaemic and haemorrhagic lesions. The most common symptoms at presentation were seizures (15/26, 57.7%), lethargy (11/26, 42.3%), hemiparesis (10/26, 38.5%) and altered levels of consciousness (10/26, 38.5%). Vascular abnormalities accounted for 50% of strokes in our study population. The average length of follow-up was 33.2 months (range, 1 to 120) with only 11 children (11/26, 42.3%) achieving full recovery. Signifi cant prognostic factors include altered consciousness and seizures at presentation, lesions in both cortical and subcortical locations, systemic disease aetiology, neurological defi cits at discharge and seizures at the time of discharge. Conclusion: Long-term neurological, neuropsychological and functional impairment are common in survivors of paediatric strokes. Certain clinical features and lesion characteristics are useful indicators of prognosis in these children.[ Free Full Text ]

Delayed diagnosis of long QT syndrome is frequent. Symptoms are often attributed to alternative diagnoses, most commonly seizure disorder. Patients labeled as epileptic experience a particularly long diagnostic delay. ECGs were frequently requested, but interpretation errors were common. Given the potentially preventable mortality of long QT syndrome, emergency physicians investigating syncope and seizure should maintain a high index of suspicion.

In the July 30 , 2009 issue of the New England Journal of Medicine Tomohiro Yamashita ( Tokai University School of Medicine ) and Thomas C. Kwee, M.D. (University Medical Center Utrecht , the Netherlands) describes an MR-based approach that is capable of selectively visualizing the peripheral nervous system over long trajectories in a single examination: whole-body MR neurography.Although the brain and spinal cord are well visualized with magnetic resonance (MR) imaging, peripheral nerves cannot be selectively visualized by commonly used methods, such as T1-weighted and (fat-suppressed) T2-weighted imaging, because of the similarity in signal intensities between the peripheral nerves and surrounding structures on these images. In this report they demonstrate the use of a diffusion-weighted imaging sequence to perform a whole body Magnetic Resonance (MR) Neurography in a healthy volunteer and a patient with chronic inflammatory demyelinating polyneuropathy (CIDP).

The full text of this article can be accessed here [ Free Full Text ] : http://content.nejm.org/cgi/reprint/361/5/538.pdf

IV LEV was effective in terminating status epilepticus or acute repetitive seizures and well tolerated in critically ill children. Further study is needed to elucidate the role of IV LEV in critically ill children.