Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Reassessment of phenytoin for treatment of late stage progressive myoclonus epilepsy complicated with status epilepticus.
[]
In
order
to
find
an
effective
treatment
option
for
status
epilepticus
in
progressive
myoclonus
epilepsy
(
PME
)
,
we
reviewed
the
clinical
course
of
9
patients
with
PME
.
Initially
,
epilepsy
was
successfully
treated
with
antiepileptics
.
However
,
it
gradually
became
refractory
to
medication
,
and
status
epilepticus
emerged
3
-
19
years
after
the
onset
of
epilepsy
.
In
these
patients
,
status
epilepticus
in
PME
was
classified
into
(
1
)
myoclonic
status
epilepticus
(
MSE
)
,
(
2
)
myoclonic
-generalized
status
epilepticus
(
MGSE
)
,
and
(
3
)
generalized
status
epilepticus
(
GSE
)
.
MSE
was
common
in
patients
with
neuronal
ceroid
lipofuscinosis
,
and
GSE
was
common
in
those
with
dentatorubral-pallidoluysian
atrophy
.
MGSE
was
characterized
by
the
mixture
of
escalating
myoclonus
and
generalized
seizures
,
and
was
observed
in
patients
with
Gaucher
disease
or
unspecified
PME
.
All
patients
were
often
refractory
to
infusion
of
benzodiazepines
and
barbiturates
but
phenytoin
was
able
to
terminate
status
epilepticus
in
7
patients
.
Oral
phenytoin
administration
as
preventive
therapy
was
effective
in
6
patients
.
Aggravation
of
myoclonus
was
not
provoked
by
these
treatments
.
We
propose
that
phenytoin
should
be
considered
as
a
treatment
choice
for
PME
patients
at
late
stages
to
prevent
the
detrimental
effects
of
prolonged
or
repeated
status
epilepticus
on
the
brain
tissues
.
