Transcript Dias nummer 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
EEG from patient 3
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
EEG
•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.
Table 2. Results
Patients
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
WPPSI-R/
WISCIII
IQ (verbal/
performance/ fullscale)
Before KD
1
2
•Neuropsychological test (WPPSI/ WISC) are available
before KD and at follow up
3
•24-hour EEG recording is available before start of KD
and at follow up, every 3-6 mo.
4
WPPSI/ WISCIII
IQ (verbal/
WPPSI/ WISC
IQ (verbal/
performance/ full-scale)
At follow-up with KD
(after 6-12 mo)
performance/ full-scale)
At follow-up with KD
(after 18-24 mo)
VIQ=72/
PIQ=50/
FSIQ=46
VIQ=91/
PIQ=64
FSIQ=76
VIQ=48/
PIQ=50/
FSIQ=40
na*
na*
VIQ=62/
PIQ=59/
FSIQ=52
VIQ=52/
PIQ=48/
FSIQ=40
VIQ= 44/
PIQ=42/
FSIQ=40
na*
IQ (Leiter)= 58
na*
IQ (Leiter)= 26
Table 1. Patient data
Patient
number/
gender
Aetiology
1/ male
idiopathic
2/ male
Symptomatic 6 (steroidsx3,
(neonatal
CLB,STM,LEV,
stroke)
VPA,ESM)
3/ male
4/ female
AEDs tried
before KD
CSWS
since
age
6 (CBL,ESM,
6y
VPA, LEV, STM,
steroids)
5y
Symptomatic 6
4y 8mo
(neonatal
(VPA,CLB,ESM,
stroke)
STM,LEV,
Steroidsx3)
Symptomatic 6 (VPA,CLB,
5y
(neonatal
STM,LEV,ESM,
stroke)
steroids)
Age at KD
start
10y1mo
EEG before KD/
& at follow up (%
interictal epileptiform
discharges during
NREM sleep every 3-6
mo)
Before KD: 90/ 67/ 95
During KD: 95/ 76/ 75
After KD: 75/ 87
Before diet: 90/ 95
During diet: 95/ 67
After diet: 93
Before diet: 80/ 85
During diet: 97/ 95
After diet: 95/ 96
Before diet: 68/ 86
During diet: 69/ 95/ 88/
95
After diet: n/a
*n/a= no available
Results
•EEG characteristics (% discharges in NREM sleep) seem to
fluctuate independently to KD
•No significant improvement in EEG or cognitive tests were found
6y4mo
Conclusions
7y6mo
7y
KD characteristics
Classical ketogenic diet with ratios 2.5-4.0: 1 were used with good
acceptance and tolerability
.
•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
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