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[Case report of dentatorubral pallidoluysian atrophy in a patient on a ketogenic diet].
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We
report
a
16
-
year
-old
woman
with
dentatorubral
pallidoluysian
atrophy
(
DRPLA
)
on
a
ketogenic
diet
.
She
was
scheduled
for
dental
treatment
under
general
anesthesia
.
She
was
diagnosed
as
having
DRPLA
at
the
age
of
10
.
Medication
included
clonazepam
,
chlordiazepoxide
,
ethosuximide
,
sodium
valproate
and
piracetam
.
She
had
been
placed
on
a
ketogenic
diet
at
the
age
of
15
,
and
seizures
decreased
.
Preoperative
laboratory
data
were
normal
except
for
serum
cholesterol
level
(
228
mg
x
dl
(
-
1
)
)
and
blood
ketones
(
1
.
8
mmol
x
l
(
-
1
)
)
with
the
use
of
Medisense
Xtra
.
General
anesthesia
was
induced
using
thiamylal
,
vecuronium
,
sevoflurane
and
maintained
with
sevoflurane
and
nitrous
oxide-oxygen
.
Fluid
infusion
employed
Solita
T
1
(
glucose
content
of
2
.
6
%
:
total
150
ml
)
.
Operation
and
general
anesthesia
presented
no
problems
.
We
continued
to
infuse
Solita
T
1
(
total
350
ml
)
for
about
4
hours
postoperatively
.
Ketogenic
diet
and
additional
medicine
were
started
after
5
hours
postoperatively
.
We
measured
perioperative
and
postoperative
BS
and
blood
ketones
with
the
use
of
Medisense
Xtra
.
She
recovered
from
anesthesia
without
any
significant
complication
.
