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Electroclinical features of epilepsy in patients with juvenile type dentatorubral-pallidoluysian atrophy.
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To
clarify
the
electroclinical
characteristics
of
epileptic
seizures
in
patients
with
juvenile
type
dentatorubral-pallidoluysian
atrophy
(
DRPLA
)
.
Seventeen
patients
with
juvenile
type
DRPLA
confirmed
by
genetic
analysis
were
studied
retrospectively
.
The
clinical
records
of
all
17
patients
and
the
ictal
video
electroencephalography
(
EEG
)
recordings
from
12
of
the
17
patients
were
reviewed
.
Electroclinical
studies
in
12
patients
identified
11
habitual
seizures
in
6
patients
as
partial
seizures
on
ictal
video
EEG
recordings
.
Clinical
manifestations
composed
mainly
of
versions
of
the
head
and
loss
of
consciousness
.
These
partial
seizures
were
persistently
recorded
throughout
the
clinical
course
.
Brief
generalized
seizures
(
atypical
absence
and
myoclonic
seizure
)
were
observed
in
6
of
12
patients
at
the
early
stage
.
In
contrast
,
generalized
tonic-clonic
seizures
(
GTCS
)
were
recorded
in
four
advanced
stage
patients
who
were
almost
bedridden
.
Semiological
studies
in
17
patients
showed
that
the
prevalence
of
partial
seizures
was
significantly
higher
in
patients
with
younger
epilepsy
onset
(
below
10
years
of
age
;
chi
(
2
)
test
,
p
<
0
.
05
)
and
that
the
age
of
epilepsy
onset
was
significantly
lower
in
patients
with
partial
seizures
than
in
those
without
partial
seizures
(
Mann-
Whitney
U
test
,
p
=
0
.
02
)
.
However
,
the
number
of
CAG
repeats
and
age
at
clinical
onset
were
not
significantly
different
between
two
groups
.
Partial
seizure
is
one
of
the
common
epileptic
features
in
juvenile
type
DRPLA
,
especially
in
patients
with
younger
epilepsy
onset
.
Seizure
types
may
be
affected
in
an
age-dependent
manner
and
change
evolutionally
during
progression
of
the
clinical
stage
.
