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Zonisamide ameliorates symptoms of secondary paroxysmal dystonia.
[pyruvate dehydrogenase deficiency]
A
number
of
medications
have
been
used
with
varying
success
to
treat
the
symptoms
of
generalized
,
focal
,
and
paroxysmal
dyskinesias
;
these
agents
include
anticonvulsant
,
benzodiazepine
,
neuroleptic
,
dopaminergic
,
dopamine
antagonist
,
and
carbonic
anhydrase
inhibitor
types
.
The
carbonic
anhydrase
inhibitor
drug
group
is
best
represented
by
acetazolamide
,
which
has
been
widely
applied
in
the
treatment
of
paroxysmal
dyskinesias
.
Zonisamide
,
which
has
several
putative
pharmacologic
mechanisms
of
action
,
is
a
member
of
the
carbonic
anhydrase
inhibitor
drug
group
.
Zonisamide
was
chosen
for
treatment
of
secondary
paroxysmal
dystonia
in
a
patient
with
pyruvate
dehydrogenase
deficiency
(
case
1
)
and
in
two
patients
with
neonatal
hemochromatosis
and
family
history
of
neonatal
hemochromatosis
(
cases
2
and
3
)
.
Although
zonisamide
ameliorated
the
symptoms
of
secondary
paroxysmal
dystonia
in
these
three
patients
,
the
precise
biochemical
mechanism
remains
unclear
,
and
further
studies
are
needed
to
substantiate
and
explain
this
finding
.
Diseases
Validation
Diseases presenting
"secondary paroxysmal dystonia"
symptom
pyruvate dehydrogenase deficiency
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