Dias nummer 1

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) sleep¹
• 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.

EEG from patient 3

EEG

Table 2. Results n/a
no available
Patients WPPSI-R/ WISCIII IQ (verbal/ performance/ full-scale) Before KD WPPSI/ WISCIII IQ (verbal/ performance/ full-scale) At follow-up with KD (after 6-12 mo) WPPSI/ WISC IQ (verbal/ performance/ full-scale) At follow-up with KD (after 18-24 mo) EEG before KD/ at follow up ( interictal epileptiform discharges during NREM sleep every 3-6 mo)
1 VIQ72/ PIQ50/ FSIQ46 VIQ48/ PIQ50/ FSIQ40 na Before KD 90/ 67/ 95 During KD 95/ 76/ 75 After KD 75/ 87
2 VIQ91/ PIQ64 FSIQ76 na VIQ62/ PIQ59/ FSIQ52 Before diet 90/ 95 During diet 95/ 67 After diet 93
3 VIQ52/ PIQ48/ FSIQ40 VIQ 44/ PIQ42/ FSIQ40 na Before diet 80/ 85 During diet 97/ 95 After diet 95/ 96
4 IQ (Leiter) 58 na IQ (Leiter) 26 Before diet 68/ 86 During diet 69/ 95/ 88/ 95 After diet n/a
Table 1. Patient data
Patient number/ gender Aetiology AEDs tried before KD CSWS since age Age at KD start
1/ male idiopathic 6 (CBL,ESM, VPA, LEV, STM, steroids) 6 y 10y1mo
2/ male Symptomatic (neonatal stroke) 6 (steroidsx3, CLB,STM,LEV, VPA,ESM) 5 y 6y4mo
3/ male Symptomatic (neonatal stroke) 6 (VPA,CLB,ESM,STM,LEV, Steroidsx3) 4y 8mo 7y6mo
4/ female Symptomatic (neonatal stroke) 6 (VPA,CLB, STM,LEV,ESM,steroids) 5y 7y

• 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_at_vestamt.dk marnik_at_vestamt.dk