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[Asymptomatic carnitine depletion on ketogenic diet in patients with pharmacoresistant epilepsies].
[pyruvate dehydrogenase deficiency]
The
ketogenic
diet
(
KD
)
is
a
nutritional
therapy
for
the
treatment
of
epilepsies
and
certain
metabolic
disorders
like
the
glucose-transporter-
deficiency
or
the
pyruvate-dehydrogenase-
deficiency
.
The
basis
of
the
ketogenic
diet
is
the
change
of
energy
metabolism
to
utilisation
of
fatty
acids
and
their
metabolites
,
ketonic
bodies
.
Carnitines
,
which
play
an
important
role
in
transport
and
elimination
of
fatty
acids
,
are
essential
for
effective
ketogenesis
.
Carnitine
deficiency
is
described
on
ketogenic
diet
.
The
aim
of
this
study
is
to
evaluate
when
to
expect
a
carnitine-
deficiency
during
KD
.
The
carnitine
levels
of
19
patients
aged
1
.
4
to
8
.
5
years
(
median
4
.
0
years
)
,
who
were
treated
with
ketogenic
diet
because
of
pharmacoresistent
epilepsy
,
were
evaluated
retrospectively
.
Carnitine
deficiency
during
KD
was
detected
in
26
%
of
the
patients
and
in
57
%
of
the
patients
without
carnitine
substitution
.
Decreased
carnitine
level
occurred
also
with
carnitine
substitution
independent
from
additional
valproat
therapy
.
The
time
of
appearance
of
carnitine
deficiency
on
KD
was
between
3
days
and
248
weeks
(
median
32
weeks
)
.
Regular
controls
of
carnitine
levels
should
be
performed
during
the
treatment
with
ketogenic
diet
,
both
at
the
beginning
and
during
longterm-therapy
.
