Refractory status epilepticus arrested by vagus nerve stimulation.

A 54-year-old man developed altered mental state and generalised tonic-clonic seizures after 1 week of upper respiratory tract symptoms and diarrhoea, having been previously well. His MR scan of brain showed multifocal progressive T2 cortical signal changes. He was diagnosed with new-onset refractory status epilepticus (NORSE), initially treated as being secondary to autoimmune/paraneoplastic limbic encephalitis, although subsequent investigations were negative. His seizures and electrographic epileptiform activity continued despite escalating doses of antiseizure medications, immunosuppression with corticosteroids, immunoglobulins, plasma exchange and rituximab, and thereafter anaesthetic agents. A vagus nerve stimulator (VNS) was implanted 6 weeks after admission and its voltage rapidly increased over 4 days; his seizure activity resolved in the third week after VNS implantation. This case highlights the role of VNS in the early management of NORSE.

Monitoring the changing pattern of delivery of paediatric epilepsy surgery in England--an audit of a regional service and examination of national trends.

PURPOSE: The demand for paediatric epilepsy surgery in the UK greatly exceeds the number of operations performed. Hence, Children's Epilepsy Surgery Service (CESS) was commenced in 2012. This study is aimed to characterise the changes in service delivery in the North East of England Paediatric Neuroscience Network and nationally. METHODS: A retrospective cohort study of paediatric epilepsy surgery in Leeds between 2005 and 2012 is presented followed by analysis of British Paediatric Neurosurgical Group (BPNG) data before and after CESS commissioning. RESULTS: During the study period, 42 children underwent epilepsy surgery in Leeds. The commonest aetiologies were neoplasm (33%), focal cortical dysplasia (19%) and mesial temporal sclerosis (19%). Seizure outcome was 71 % EngelI and 83% EngelI+II. Complications included one infection (2%), two temporary (5%) and one permanent (2%) motor deficits, three new/worsened visual field deficits (7%). There were six re-craniotomies (14%). The BPNG data show a 48% increase in paediatric epilepsy surgery in England between 2009 (90 cases) and 2012 (133 cases), and a 20% fall in 2013 (106 cases)--the first calendar year for CESS. On average, 64% of all operations were performed in London. CONCLUSIONS: The number of children receiving surgery for epilepsy in England had increased annually up to, and declined after, the establishment of CESS centres. The yearly caseload in neurosurgical units outside of London is small. The outcomes from Leeds are comparable to those published elsewhere. Other UK units are encouraged to publish outcomes to facilitate patient, commissioner and provider decision making.

No association between bipolar disorder risk polymorphisms in ANK3 and CACNA1C and common migraine.

BACKGROUND: Migraine and bipolar disorder are characterized by a high level of co-morbidity, and a common familial-genetic basis has recently been hypothesized for the 2 disorders. Genome-wide association studies have reported strong evidence of association between the polymorphisms rs10994336[T] in the ANK3 gene and rs1006737[A] in the CACNA1C gene and risk of bipolar disorder. OBJECTIVE: The aim of this study was to evaluate the hypothesis of a genetic linkage between migraine and bipolar disorder by investigating the familial transmission of the 2 bipolar disorder risk polymorphisms, in a sample of family trios with probands with childhood migraine, and unrelated controls. METHODS: Our sample comprised 192 family trios, each with a proband with childhood migraine (137 migraine without aura, 44 migraine with aura) and 228 unrelated controls. The markers rs10994336 and rs1006737 were genotyped using a TaqMan single nucleotide polymorphism Genotyping Assay. The transmission disequilibrium test analysis for the family trios and the case-control analysis were performed using the program UNPHASED. RESULTS: The allelic and genotypic transmission disequilibrium test analysis did not show any evidence of transmission distortion of the 2 markers in both migraine overall (rs10994336: OR = 1.61, P = .11; rs1006737: OR = 1.12, P = .49) and in the migraine without aura and migraine with aura subgroups. Likewise, the case-control analysis of alleles and genotypes frequencies did not show any evidence of association. CONCLUSION: In the present study, we did not find evidence for association between the bipolar disorder risk polymorphisms rs10994336 in the ANK3 gene and rs1006737 in the CACNA1C gene in migraine. However, as these are variants that have a small effect on the risk of bipolar disorder (OR < 1.5), we cannot exclude a similar small effect on migraine susceptibility with the present sample size.

Correlation of autism with temporal tubers in tuberous sclerosis complex.

Tuberous sclerosis complex (TSC) is an inherited genetic disorder commonly associated with neuropsychiatric complications like epilepsy, mental retardation, autism and other behavioral problems and constitutes about 1-4% of the autistic population. Mental retardation and seizures, particularly infantile spasms are significant risk factors for the development of autism. Patients of TSC with autism are more likely to have temporal tubers than those cases without autism. We describe clinical and neuroimaging features of two such cases of tuberous sclerosis with autism.

Topiramate in the prophylaxis of pediatric migraine: a double-blind placebo-controlled trial.

Several large, randomized controlled trials have demonstrated the efficacy of topiramate in migraine prophylaxis in adults. However, there are limited data about the use of topiramate in migraine prophylaxis in children. We conducted this single-center, double-blind, placebo-controlled trial to evaluate the efficacy and safety of topiramate in the prophylaxis of migraine in children. A total of 44 children with migraine were randomized using random number tables to receive topiramate (n = 22) or placebo (n = 22). The total duration of treatment was 4 months, including a baseline period of 1 month during which topiramate was titrated weekly in 25-mg increments to 100 mg/d in 2 divided doses or to the maximum tolerated dose. The titration was followed by a 12-week maintenance phase during which topiramate was given in 2 divided doses. The primary outcome measures were the reduction in the mean migraine frequency and severity of headache. Secondary outcome measures included the number of times analgesics were required for a month for acute attacks and functional disability. Functional disability was measured by comparing school absenteeism and Pediatric Migraine Disability Assessment Scale (PedMIDAS). The decrease in mean (+/-SD) monthly migraine frequency from 16.14 (+/-9.35) at baseline to 4.27 (+/-1.95) at the end of the study in the topiramate group was significantly greater as compared with a decrease from 13.38 (+/-7.78) to 7.48 (+/-5.94) at the end of the study in the placebo group (P = .025). The difference in number of rescue medications used for topiramate and placebo was not statistically significant (P = .059). There was a statistically significant decrease in the PedMIDAS score from 50.66 (+/-32.1) to 10.42 (+/-6.39) at the end of the study in the topiramate group compared with a decrease from 42.66 (+/-27.5) to 23.7 (+/-19.1) at the end of 4 months in the placebo group (P = .003). The decrease in school absenteeism was significant with topiramate compared with placebo (P = .002). Weight loss, decreased concentration in school, sedation, and parasthesias were important side effects with topiramate. Most of these side effects were mild to moderate and were not significant enough to cause dropout from the study.

Acute disseminated encephalomyelitis in North Indian children: clinical profile and follow-up.

Acute disseminated encephalomyelitis in children is not uncommon in developing countries, yet there is little systematic documentation of its clinical profile and follow-up. We studied the clinical and neuroradiologic features of acute disseminated encephalomyelitis in 52 consecutive children. Clinical details, magnetic resonance imaging (MRI) findings, and the results of other investigations were recorded, and children were followed up from 6 to 48 months. A repeat MRI was done after 3 to 4 months, and in those with persistent lesions, another MRI was done after 6 to 7 months of discharge. The mean age at presentation was 6.14 +/- 3.17 years, 73.1% were male, and 17 children had a history of antecedent infectious illness or vaccination. Most children had a meningoencephalitic presentation, with sudden-onset motor weakness in 76.9% and seizures in 36.5%. Altered sensorium and pyramidal signs were seen in 55.8% and 80.7% of children, respectively. On MRI, scattered T(2)-weighted hyperintense lesions were seen, mainly in the subcortical white matter, especially in the parietal (53.8%) and frontal (30.17%) regions. Thalamic, basal ganglia, and callosal lesions were seen in 30.76%, 17.3%, and 13.46% of cases, respectively. Variable contrast enhancement was seen in 48% of those who had contrast MRIs. The response to methylprednisolone was good, with dramatic recovery in 26.9% and marked improvement in 51.9% at discharge. On follow-up, of 44 children, residual smaller MRI lesions were seen in 30. The MRI was repeated at 6 months in children with residual lesions, and it was found that the lesions either disappeared or were significantly reduced after 6 months in 75% of cases. Four children had relapse of acute disseminated encephalomyelitis with new lesions on MRI. All of them responded to methylprednisolone. None of the clinical or neuroradiologic factors at presentation had any significant correlation with relapse. Six months after discharge, no deficits could be found in 61.3% of cases; 15.9% and 4.5% had motor and cognitive deficits, and 9% had multiple deficits. The presentation of pediatric acute disseminated encephalomyelitis in developing countries is similar to that in developed countries. In spite of an aggressive presentation, most children respond well to corticosteroids. MRI lesions disappear or are significantly reduced at 6 months in the majority of cases.

Profile of West syndrome in North Indian children.

To study the profile of West syndrome (WS) in North Indian Children, 165 cases of WS were analyzed. Details of seizure semiology, prenatal and perinatal events, developmental milestones, treatment received, physical and neurological examination and investigations were recorded. The response of seizures to various therapeutic modalities and the final developmental status were taken as primary outcome variables. Analysis was done to find the factors influencing these outcome variables. The age of onset of infantile spasms ranged from 1 to 19 (mean 6.1 +/- 3.4) months. Age at presentation ranged from 1.5 months to 4.5 years (mean 14.7 +/- 11.4 months); 74% had flexor spasms. Other types of seizures were associated in 31 children. Antenatal problems and adverse perinatal events were reported in 26.7 and 59.4%, respectively. Developmental delay was recognized in 69.7% prior to and in 27.9% after onset of spasms. Microcephaly was seen in 72.7%. Interictal EEG showed hypsarrhythmia in 44; generalized spike and slow waves in 31% and burst suppression in 7%. Computed tomography scan done in 94 cases showed cerebral atrophy in 15%, infarcts in 8%, tubers in 7%, developmental malformations in 5%. Magnetic resonance imaging done in 77 cases showed periventricular T2WI white matter hyper intensities in 33.8% and cerebral atrophy in 21%. Prednisolone and ACTH were used in 57 and 35 cases, respectively. Complete control of seizures was seen in 49 and 46% cases. No significant difference in seizure control or developmental outcome was found in the two groups. Overall, 42.4, 30.9 and 16% children showed complete, partial and no control of seizures. After therapy, developmental improvement was seen in 55.8% and no change in 23.6% cases. The type of spasms had no correlation with the other parameters including etiology, seizure or developmental outcome. An early age of onset correlated with presence of antenatal problems (P < 0.05). Seizure control and developmental improvement correlated significantly (P < 0.005). Developmental outcome was better in cryptogenic as compared to symptomatic cases (P < 0.05). No other significant correlations were found. In India WS is often diagnosed late because of lack of awareness. Adverse perinatal events are important etiological factors. Non-affordability of ACTH and Vigabatrin prompts the use of prednisolone in most cases.

Chemotherapy related fatal neurotoxicity during induction in acute lymphoblastic leukemia.

Neurotoxicity is a common complication during cancer chemotherapy. It is estimated that 3-10% of children with acute lymphoblastic leukemia (ALL) experience acute, transient neurotoxicity during induction chemotherapy. Fatal acute neurotoxicity is rarely encountered. Neurological evaluation of children with ALL at diagnosis and during treatment is of value in order to diagnose neurological complications early so that appropriate intervention can be adopted. This communication describes the profile of two children with unexpected, acute fatal neurologic toxicity during induction chemotherapy for ALL.

Cerebral gigantism with West syndrome.

A case of cerebral gigantism (Sotos syndrome) with West syndrome in a one-year-old male child is reported. The case had a large stature, typical facies and neurodevelopmental delay along with infantile spasms, which were refractory to treatment with valproate and clonazepam.

Ohtahara syndrome with biotinidase deficiency.

Ohtahara syndrome is a rare epileptic encephalopathy in infants; the underlying etiology is generally thought to be structural brain malformations. The authors present a rare case of this type of epileptic encephalopathy in which a treatable metabolic condition such as biotinidase deficiency was suspected and diagnosed, and early institution of appropriate therapy led to a good clinical outcome.

Complex regional pain syndrome type 1 in a child with migraine.

Complex regional pain syndrome type 1 is a rare painful syndrome in children involving an extremity which consists of pain out of proportion to the cause, loss of function, and significant evidence of autonomic dysfunction. We report a child, a known case of migraine who presented with spontaneous onset pain and sudomotor changes in an extremity not preceded by any trauma. A good clinical eye is required for the identification and diagnosis of this underreported condition to prevent doing extensive investigations. The coexistence of migraine and complex regional pain syndrome type 1 in a patient is interesting and is probably due to the common underlying pathophysiological abnormalities involving central serotonin activity and neurogenic inflammatory mechanisms.

Effect of electronic media on children.

Radio, television (TV), movies, video games, cell phones, and computer networks have assumed central roles in our children's daily lives. The media has demonstrated potentially profound effects, both positive and negative, on children's cognitive, social, and behavioral development. Considering the increasing exposure of children to newer forms of media, we decided to review the current literature on the effects of media on child health both in the Western countries and India. It is widely accepted that media has profound influence on child health, including violence, obesity, tobacco and alcohol use, and risky sexual behaviors. Simultaneously, media may have some positive effects on child health. We need to find ways to optimize the role of media in our society, taking advantage of their positive attributes and minimizing their negative ones. We need to understand better how to reverse the negative impact of media and make it more positive.

Calcified neonatal renal vein and vena caval thrombosis.

We present an unusual case of an extensive venous thrombosis (involving the inferior vena cava, bilateral renal veins, gonadal vein and iliac veins) diagnosed in the neonatal period. The CT images revealed the typical diagnostic pattern.