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394
Abstracts
FO-6
Seizure Disorders (2)
FO-6-1
Efficacy of ketogenic diet on intractable epilepsy in
children: a report of 215 cases
M. Ghofrani
Mofid Children Hospital, Tehran, Iran
The ketogenic diet is a high fat, low protein, low carbohydrate
diet. This study is conducted to determine the
effectiveness of ketogenic diet in epileptic children refractory
to medication. A total of 215 children, age 2–12 years,
who continued to have more than two seizure attacks
weekly despite adequate therapy with at least two anticonvulsant
medications, were enrolled in this study and
followed for 1 month to 3 years. Ketogenic diet’s effectiveness
was studied at the first, second and third follow
ups (1st, 6th and 12th month, respectively). Tolerance to
diet and adverse events secondary to ketogenic diet
consumption were recorded. Two hundred and fifteen children
with mean age of 5.1 years, averaged 235 seizures per
month before the diet, despite an exposure to a mean of 6.6
antiepileptic medications were enrolled. At 1 month follow
up after the initiation of diet, 68.8% were seizure-free and
11.7% had .50% decrease in seizure frequency. A total of
98.3% of the patients had adhered to the diet regimen and
reported at 1-month follow up. At 6 months, 44.5% were
seizure-free and 21.7% had .50% decrease in seizure
frequency. A total of 89.3% had observed the diet till the
6th month and reported for follow up. At 12 months, 14.8%
were seizure-free and 12.9% had .50% decrease in seizure
frequency. A total of 57.2% continued with the ketogenic
diet for 1 year and were observed. In conclusion, the ketogenic
diet should be considered as effective treatment for
epileptic children whose seizures remain refractory to treatment.
It seems that ketogenic diet is more effective and
cheaper than many new antiepileptic medications.
FO-6-2
Low-dose synthetic ACTH therapy for West syndrome:
initial effects and long-term outcome
M. Ito
Kyoto Multi-Institutional Study Group of Pediatric Neurology,
Shiga Medical Center for Children, Moriyama, Japan
Background: Most Japanese pediatric neurologists
attempt other treatments before using adrenocorticotropic
hormone (ACTH) therapy for West syndrome (WS), and
even then, they use only a low-dose synthetic ACTH to
avoid serious adverse effects. In this multi-institutional
study, we analyzed the initial effects, adverse effects and
long-term outcome in patients treated with low-dose
synthetic ACTH in Japan. Methods: We analyzed the medical
records of 138 patients with WS, who were treated with
low-dose synthetic ACTH therapy for the first time at our
institutions between 1989 and 1998. Results: At the end of
ACTH therapy, excellent effect on seizures was noted in
106 out of 138 (76%) patients, good effect in 23 (17%), and
poor effect in nine (7%). Initial effects on EEG were excellent
in 53 out of 138 (38%) patients, good in 76 (55%) and
poor in nine (7%). As for seizure prognosis at the time of
follow-up, 51 out of 99 (52%) patients were seizure-free,
while 48 (48%) patients had seizures. Mental outcome was
normal in six out of 98 (6%) patients, mild MR in 16
(16%), moderate MR in 26 (27%) and severe MR in 50
(51%). The initial effects of ACTH on seizures and longterm
outcome were not dose-dependent (daily dosage;
0.005–0.032 mg/kg, 0.2–1.28 IU/kg, total dosage; 0.1–
0.87 mg/kg, 4–34.8 IU/kg). The severity of adverse effects
correlated with total dosage of ACTH and the severity of
brain volume loss due to ACTH correlated well with the
daily dosage and total dosage of ACTH. Conclusion: Lowdose
synthetic ACTH therapy is as effective for the treatment
of WS as the higher doses used in previous studies.
The dosage of synthetic ACTH used in the treatment of
WS can be decreased as much as possible to avoid serious
adverse effects.
FO-6-3
Neurophysiological responses to novel stimuli in infants
with infantile spasms
K.G. Werner, T. Baldeweg, R.C. Scott, S. Boyd, B.G.R.
Neville
Neurosciences Unit, Institute of Child Health, London,
United Kingdom
Objectives: Infantile spasms (IS) are strongly associated
with cognitive and social impairment, possibly related to
abnormalities in frontal/temporal neuronal networks. In
adults and older children novel environmental sounds elicit
prominent event-related potentials (ERPs) in temporal and
frontal cortices. The aims of this study were to determine
whether similarly robust novelty ERPs can be recorded in
infants and if they are abnormal in infants with IS. Methods:
Twenty-four full term infants (age range 3–10 months)
and 13 infants with IS (range 3–10 months) were recruited.
EEG was recorded continuously from 19 electrodes (10–20
system). An oddball paradigm was used with frequent
tones (80%, 1 kHz), deviant tones (10%, 1.5 kHz) and
brief novel environmental sounds (10%, 200 ms long),
delivered binaurally (interstimulus interval 700 ms) via
speakers at a distance of 30 cm. Infants were either asleep
in stages I to II or awake and feeding. Three blocks were
recorded, with 180 deviants and 180 novels. Results:
Robust and reproducible ERPs to novels were detected in
all normal infants, with peak to peak amplitudes of up to 25
mV. They consisted of two components over the temporal
(latency 250 ms) and fronto-central scalp (500 ms), respec-