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Amelioration of disabling myoclonus in a case of DRPLA by levetiracetam.
[]
We
report
on
an
18
-
year
-old
male
patient
with
dentatorubral-pallidoluysian
atrophy
(
DRPLA
)
(
number
of
CAG
repeats
:
68
)
with
progressive
myoclonus
epilepsy
(
PME
)
,
who
showed
a
dramatic
response
to
levetiracetam
in
terms
of
the
intensity
of
myoclonus
.
He
began
to
have
convulsive
seizures
and
myoclonus
at
7
and
10
years
of
age
,
respectively
,
and
his
intelligence
deteriorated
from
12
years
of
age
.
EEG
showed
multifocal
and
diffuse
spike-and-wave
complexes
.
His
convulsive
seizures
were
suppressed
from
13
years
of
age
.
At
17
years
of
age
,
the
patient
showed
gradual
intensification
of
erratic
segmental
positive
myoclonus
as
well
as
frequent
atonic
falls
that
were
probably
attributable
to
negative
myoclonus
.
Back
averaging
of
EEG
data
revealed
cortical
discharges
associated
with
positive
myoclonus
.
Photosensitive
myoclonic
seizures
were
also
observed
.
The
administration
of
levetiracetam
alleviated
positive
myoclonus
and
suppressed
atonic
falls
,
resulting
in
a
remarkable
improvement
in
the
patient
's
quality
of
daily
life
.
Reports
on
the
efficacy
of
levetiracetam
for
myoclonus
in
DRPLA
are
still
rare
,
though
its
effect
on
PME
is
known
in
the
context
of
other
neurological
disorders
.
Thus
levetiracetam
should
be
subjected
to
clinical
trials
as
a
means
of
disabling
myoclonus
in
DRPLA
.
