Browsing by Author "Menon, Ramshekhar N."

Now showing 1 - 3 of 3
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    Atonic variant of benign childhood epilepsy with centrotemporal spikes (atonic-BECTS): A distinct electro-clinical syndrome
    (BRAIN & DEVELOPMENT, 2012)
    Purpose: To describe the clinical and electroencephalographic features, treatment strategies and outcome in a series of children with the atonic variant of benign childhood epilepsy with centrotemporal spikes (atonic-BECTS).Material and methods: Out of the 148 patients with BECTS reviewed from January 2005 to June 2010 in our Institute, there were seven (5%) with atonic-BECTS. All underwent video EEG, high-resolution magnetic resonance imaging (MRI), neuropsychological evaluation and language assessment. Their progress was followed. In addition to sodium valproate, three were treated with steroids, followed by intravenous immunoglobulin (IVIG) when the seizures relapsed while tapering or after stopping the steroids.Results: All of the children had earlier onset (mean = 2.4 years), increased frequency and increased duration of focal seizures compared to typical BECTS. Head drop and truncal sway due to axial or axiorhizomelic atonia occurring several times per day or week was the key manifestation. The atonic seizures worsened with carbamazepine in three, clonazepam in two and clobazam in one. When the atypical seizures commenced, some children developed one or more of the following problems: hyperactivity, attention deficit, clumsy gait, and mild cognitive or language dysfunction. Three children became seizure free, one on steroids and the other two on IVIG.Conclusions: BECTS in children with an early age of onset and frequent and prolonged seizures is more likely to evolve into atonic-BECTS. Carbamazepine and some benzodiazepines may worsen these seizures. Three children became seizure free with immunomodulatory therapy, one on steroids and the other two on IVIG, and had complete resolution of the transient motor and cognitive impairment. Atonic-BECTS needs to be differentiated from Lennox-Gastaut syndrome since it is potentially treatable and children recover with no sequel. Although all the children in this series continued to be on treatment with sodium valproate it is currently undetermined whether they would have required to do so if followed up for an extended period of time. (c) 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
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    Post-irradiation "acquired cavernous angiomas" with drug resistant seizures
    (EPILEPSY RESEARCH, 2011)
    Cavernomas are well-known congenital vascular lesions with presumably high epileptogenicity. We report two patients who developed cavernomas; both were in remission from childhood acute lymphoblastic leukemia following standard chemo-radiotherapy. They developed drug-resistant focal epilepsy secondary to cavernomas and were subjected to surgical/medical management. This report highlights the ictogenesis of radiation-induced "acquired" cavernous angiomatosis in the brain. Appropriate treatment, including resection of these lesions in selected cases, improves the quality of life in such patients. (C) 2011 Elsevier B.V. All rights reserved.
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    The evolving electroclinical syndrome of "epilepsy with ring chromosome 20"
    (SEIZURE-EUROPEAN JOURNAL OF EPILEPSY, 2012)
    Purpose: Ring chromosome 20 {r(20)} - manifests as a refractory epilepsy syndrome with complex partial seizures (CPS), nocturnal frontal lobe seizures and non-convulsive status epilepticus (NCSE) in the majority of cases. r(20) lacks a specific phenotypic expression or dysmorphic features. Psychomotor development may be normal, making the diagnosis difficult unless there is a high index of suspicion. This description of further cases is intended to improve recognition of this syndrome.Methods: Karyotyping for r(20) was done in patients presenting with cryptogenic refractory epilepsy suggestive of r(20) from August 2008 to September 2010. We identified three patients with r(20) associated with refractory epilepsy. All were investigated with long-term video EEG. Their unique electro clinical features; epilepsy syndrome and outcome are discussed and compared to the available literature. Results: Karyotyping revealed ring 20 in 6-40% of lymphocytes. All manifested with seizures beginning at 2-10 years of age. All had CPS; two had NCSE and one a seizure disorder resembling Lennox-Gastaut syndrome. The interictal EEG showed epileptiform abnormalities predominantly over the frontotemporal regions. Two patients had NCSE with generalized, frontally dominant theta and spike and wave discharges. None had facial dysmorphism or imaging abnormalities. They remained resistant to antiepileptic drugs.Conclusions: The use of routine karyotyping can easily pick up r(20); this information is especially useful in resource-poor countries. We have evolved an algorithm stating the indications to attempt r(20) karyotyping in a given patient in the light of the results of the present study and the existing literature. (C) 2011 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.