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Blood-brain barrier, ion homeostatis and epilepsy: possible implications towards the understanding of ketogenic diet mechanisms.
[pyruvate dehydrogenase deficiency]
The
finding
that
epileptic
seizures
alter
blood
-
brain
barrier
(
BBB
)
properties
has
stimulated
interest
into
the
possibility
that
phenotypic
changes
in
brain
endothelium
may
constitute
a
pathological
initiator
leading
to
seizures
.
Recent
evidence
obtained
from
epileptic
patients
undergoing
cortical
resection
,
demonstrated
abnormal
expression
of
glucose
transporter
molecules
(
GLUT
1
)
,
while
[
18
F
]
deoxyglucose
PET
studies
demonstrated
regions
of
decreased
glucose
uptake
and
hypometabolism
in
seizure
foci
.
The
properties
of
other
'
nonexcitable
CNS
cells
'
are
also
altered
in
epileptic
tissue
,
and
glial
cells
from
epileptic
brain
displayed
diminished
capacity
for
ionic
homeostasis
;
voltage-dependent
mechanisms
were
primarily
affected
,
increasing
reliance
on
energy-dependent
mechanisms
.
Diminished
ion
homeostasis
together
with
increased
metabolic
demand
of
hyperactive
neurons
may
further
aggravate
the
neuropathological
consequences
of
BBB
loss
of
glucose
uptake
mechanisms
.
Since
ketone
bodies
can
provide
an
alternative
to
glucose
to
support
brain
energy
requirements
,
it
is
hypothesized
that
one
of
the
mechanisms
of
the
ketogenic
diet
in
epilepsy
may
relate
to
increased
availability
of
beta
-hydroxybutyrate
,
a
ketone
body
readily
transported
at
the
BBB
.
This
hypothesis
is
supported
by
the
fact
that
the
ketogenic
diet
is
the
treatment
of
choice
for
the
glucose
transporter
protein
syndrome
and
pyruvate
dehydrogenase
deficiency
,
both
associated
with
cerebral
energy
failure
and
seizures
.
Diseases
Validation
Diseases presenting
"increased availability of beta-hydroxybutyrate, a ketone body"
symptom
pyruvate dehydrogenase deficiency
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