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
- 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
- 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
Table 2. Results
*n/a= no available
Table 1. Patient data
- EEG characteristics (% discharges in NREM sleep) seem to
- fluctuate independently to KD
- No significant improvement in EEG or cognitive tests were found
- KD does not seem to be effective in the treatment
- of encephalopathy with CSWS
- Further studies with more included patients are needed
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.
Classical ketogenic diet with ratios 2.5-4.0: 1 were used with good
acceptance and tolerability