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M.J. Miranda , M. Atkins, L. Sahlholdt, T. Hoffmann, H Nielsen, M Mortensen, M.Nikanorova

Ketogenic diet (KD) in the treatment of encephalopathy with continuous spike and waves during slow sleep (CSWS): a pilot study. M.J. Miranda , M. Atkins, L. Sahlholdt, T. Hoffmann, H Nielsen, M Mortensen, M.Nikanorova ¹ Danish Epilepsy Centre, Dianalund, Denmark. Introduction

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M.J. Miranda , M. Atkins, L. Sahlholdt, T. Hoffmann, H Nielsen, M Mortensen, M.Nikanorova

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  1. Ketogenic diet (KD) in the treatment of encephalopathy with continuous spike and waves during slow sleep (CSWS): a pilot study M.J. Miranda, M. Atkins, L. Sahlholdt, T. Hoffmann, H Nielsen, M Mortensen, M.Nikanorova ¹Danish Epilepsy Centre, Dianalund, Denmark • Introduction • Epilepsy syndromes with continuous spikes and waves during slow sleep (CSWS) are defined as “a cognitive or behavioural impairment acquired during childhood, associated with a strong activation of the interictal epileptiform discharges during non-REM (NREM) sleep”¹ • Treatment with Antiepileptic drugs (AEDs) as Valproate, Benzodiazepines, Sultiam, Ethosuximide and more recently Levetiracetam² as well as Steroids might be helpful but the effect is often intermittent • In a proportion of children, CSWS is resistant to all AEDs with high risk of cognitive deterioration³ • Aim • To evaluate the effectiveness of KD for treating medically refractory CSWS, regarding tolerability, electro-clinical features and cognitive functions. • Patients and methods • The first 4 patients, fulfilling clinical and • electrophysiological criteria4 (at least 85% interictal discharges in NREM sleep) of CSWS are presented. • CSWS was refractory to all tried medications • Neuropsychological test (WPPSI/ WISC) are available • before KD and at follow up • 24-hour EEG recording is available before start of KD • and at follow up, every 3-6 mo. EEG from patient 3 EEG Table 2. Results *n/a= no available Table 1. Patient data • Results • EEG characteristics (% discharges in NREM sleep) seem to • fluctuate independently to KD • No significant improvement in EEG or cognitive tests were found • Conclusions • KD does not seem to be effective in the treatment • of encephalopathy with CSWS • Further studies with more included patients are needed . References ¹Van Bogaert P et al,2006 Acta Neurol Belg ²Aeby A et al, 2005 Epilepsia ³ Veggiotti P ,2002 Epileptic Disord. 4 ”Epileptic syndromes in infancy, childhood and adolescence”, 2005, Ed. Roger M, Wolf P et al. r KD characteristics Classical ketogenic diet with ratios 2.5-4.0: 1 were used with good acceptance and tolerability marimn@vestamt.dk marnik@vestamt.dk

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