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Spectrum of epilepsy and electroencephalogram patterns in Wolf-Hirschhorn syndrome: experience with 87 patients.
[wolf-hirschhorn syndrome]
To
define
the
spectrum
of
epilepsy
in
Wolf-
Hirschhorn
syndrome
(
WHS
)
better
,
we
studied
87
patients
(
54
females
,
33
males
;
median
age
5
.
6
years
;
age
range
1
-
25
.
6
years
)
with
confirmed
4
p
16
.
3
deletion
.
On
the
basis
of
clinical
charts
,
we
retrospectively
analyzed
the
evolution
of
the
electroencephalogram
(
EEG
)
findings
and
seizures
.
Epilepsy
occurred
in
81
patients
(
93
%
)
within
the
first
3
years
of
life
.
Sixty
out
of
81
(
74
%
)
had
generalized
tonic-clonic
seizures
,
which
was
the
only
seizure
pattern
in
32
.
Tonic
spasms
occurred
in
15
out
of
81
(
18
%
)
,
complex
partial
seizures
in
10
out
of
81
(
12
%
)
,
and
clonic
seizures
in
6
out
of
81
(
7
%
)
.
Seizures
were
frequently
triggered
by
fever
(
59
out
of
81
;
73
%
)
,
and
occurred
in
clusters
in
36
out
of
72
(
50
%
)
.
In
the
same
36
(
50
%
)
,
unilateral
or
generalized
clonic
or
tonic-clonic
status
epilepticus
occurred
during
the
first
3
years
of
life
.
Twenty
-
seven
out
of
81
patients
(
33
%
)
developed
atypical
absences
between
1
and
6
years
,
accompanied
by
a
myoclonic
component
involving
the
eyelids
and
the
hands
.
Distinctive
EEG
abnormalities
were
observed
in
73
out
of
81
(
90
%
)
.
Epilepsy
was
well
controlled
in
65
out
of
81
(
81
%
)
,
mainly
with
valproate
and
phenobarbital
,
and
improved
with
age
in
all
.
Thirty
-
two
out
of
58
(
55
%
)
are
currently
seizure
-free
.
Seizures
stopped
at
a
median
age
of
4
years
6
months
.
Epilepsy
represents
a
major
clinical
challenge
in
WHS
;
however
,
it
has
a
good
prognosis
.
Early
diagnosis
and
treatment
of
atypical
absences
,
subtle
and
often
misdiagnosed
,
is
mandatory
.
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