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Meningomyelocele and Epilepsy

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Abstract

The prevalence of seizures and epilepsy and the occurrence of other brain malformations or structural abnormalities were examined in 81 children with meningomyelocele followed at the multidisciplinary Children’s Clinics for Rehabilitative Services, University of Arizona Health Sciences Center, Tucson, AZ.
ACUTE
LYMPHOBLASTIC
LEUKEMIA
AND
SEIZURES
The
incidence,
timing,
etiologies,
and
recurrence
rate
of
seizures
among
127
pediatric
patients
with
acute
lymphoblastic
leukemia
(ALL)
were
determined
at
the
Schneider
Children's
Hospital,
and the
Long
Island
Jewish
Medical
Center,
New
York.
Of
17
patients
(13%)
who
developed
one
or
more
seizures,
16
had seizures
during
antileukemic
treatment,
almost
always
related
to
intrathecal
methotrexate
or
subcutaneous
L-asparaginase.
The
long-term
recurrence
risk of
seizures
was
low,
occurring
only
in
2
patients
(12%)
who
had
static
encephalopathy
and
neurologic
deficits. Chronic
antiepileptic
drug
therapy
was
restricted
to
patients with
recurrent
seizures
and
structural
cerebral
lesions.
(Maytal
J
et
al.
Prognosis and
treatment
of
seizures
in
children
with
acute
lymphoblastic
leukemia.
Epilepsia
August
1995;36:831-836).
(Reprints:
Dr
J
Maytal,
Division
of
Pediatric
Neurology, Schneider
Children's
Hospital,
New
Hyde
Park,
NY
11042).
COMMENT.
Seizures
occurring
in
children
with
ALL
in
this
study
were
related
to
side-effects
of
chemotherapy.
None
had
seizures
secondary
to
CNS
leukemic
relapse.
Phenytoin
was
the
drug
of
choice
for
the
control
of
the
acute
seizures
because
of
its
relative
lack
of
behavioral
and
sedative
adverse
effects.
Carbamazepine
and
valproate
were
avoided
because
of
potential
bone
marrow
suppression
and
the lack
of
intravenous
preparations.
CEREBRAL
CORTICAL
DYSGENESIS
AND
EPILEPSY
IN
ADULTS
The
clinical,
EEG
and
neuroimaging
features
in
100
adult
patients
with
cerebral
cortical
dysgenesis
(CD)
were
reviewed
at
the
National
Hospital
for
Neurology
and
Neurosurgery,
St
Mary's
Hospital,
London,
and
the
National
Society
for
Epilepsy,
Chalfont
St
Peter,
Gerrards
Cross, UK.
Patients
had
medically
refractory
epilepsy
with
onset
at
a
median
age
of
10
years.
Only
15%
had
a
history
of
status
epilepticus.
Diagnosis
was
by
neuroimaging
in
70.
EEGs
were
abnormal
in
95%.
Of
35
patients
treated
by
surgery,
15
were
completely
seizure-free.
Demonstration
of
subtle forms
of
CD
by
MRI
lessen
the
incidence
of
cryptogenic
epilepsy
in
adult
patients.
(Raymond
AA
et
al.
Abnormalities
of
gyration,
heterotopias,
tuberous
sclerosis,
focal
cortical
dysplasia,
microdysgenesis,
dysembryoplastic
neuroepithelial
tumour
and
dysgenesis
of
the
archicortex
in
epilepsy.
Clinical,
EEG
and
neuroimaging
features
in
100
adult
patients.
Brain
Tune
1995;118:629-660).
(Respond:
Dr
DR
Fish,
Department
of
Clinical
Neurophysiology,
National
Hospital
for
Neurology
and
Neurosurgery,
Queen
Square,
London
WC1N
3BG,
UK).
COMMENT.
The
heterogeneity
of
abnormalities
associated
with
CD
in
adults
with
epilepsy,
as
listed
in
the
title
of
this
article,
was
associated
with
varied
clinical,
EEG
and
MRI
features.
Compared
to
children with
epilepsy
and
CD,
these
adult
patients
had
a
relatively
low
frequency
of
delayed milestones
(12%),
mental
retardation
(11%)
and
neurologic
deficits
(17%).
MENINGOMYELOCELE
AND
EPILEPSY
The
prevalence
of
seizures
and
epilepsy
and
the
occurrence
of
other
brain
malformations
or
structural
abnormalities
were
examined
in
81
children
with
meningomyelocele
followed
at
the
multidisciplinary
Children's
Clinics
60
for
Rehabilitative
Services,
University
of
Arizona
Health
Sciences
Center,
Tucson,
AZ.
Seventeen
(21%)
had
seizures
during
follow-up
ranging
from
1.3
to
16
years.
Fourteen
(17%)
had
epilepsy
and
5
had
seizures
controlled
by
anticonvulsant
drugs.
CNS
pathology
in
addition
to
the
shunted
hydrocephalus
included
encephalomalacia
in
7,
cerebral
malformations
in
2,
and
calcifications
in
1.
(Talwar
D
et
al. Epilepsy
in
children
with
meningomyelocele.
Pediatr
Neurol
July/August
1995;13:29-32).
(Respond:
Dr
Talwar,
Department
of
Pediatrics,
University
of
Arizona
Health
Sciences
Center,
1501
North
Campbell
Avenue,
Tucson
AZ
85724).
COMMENT.
Although
epilepsy
in
children
with
meningomyelocele
occurs
mainly
in
those
with
shunted hydrocephalus,
structural
cerebral
abnormalities
other
than
the
shunt
may
be
important
causes.
INFECTIOUS
DISORDERS
HERPESVIRUS-6
INFECTION
AND
FIRST
FEBRILE
SEIZURES
The
association
between
acute
human
herpesvirus-6
(HHV-6)
infection
and
first
febrile
convulsions
was
investigated prospectively
in
42
children
evaluated
by
virologic
and serologic
methods
at
the
North
Shore
University
Hospital-Cornell
University
Medical
College,
Manhasset,
New
York.
Primary
HHV-6
infection
was
documented
by
viral
culture
in
8
(19%),
and
fourfold
rises
in
HHV-6
titer
were
present
in
9
(26%)
of
34
children
whose
blood
was
analyzed
for
acute
and
convalescent
HHV-6
titers.
The
majority
(10
of
11)
HHV-
6
cases
were
less
than
24
months
of
age,
and
3/11
had
roseola.
Viral
isolation
in
CSF,
attempted
in
29,
including
7
with
evidence
of
HHV-6
illness,
was
negative.
(Barone
SR
et
al.
Human
herpesvirus-6
infection
in
children
with
first
febrile
seizures.
1
Pediatr
lulv
1995;127:95-97).
(Reprints:
Stephen
R
Barone
MD,
North
Shore
University
Hospital,
300
Community
Drive,
Manhasset,
NY
11030).
COMMENT.
Acute
HHV-6
infection
is
a
significant
factor
in
the
etiology
of
fever
and
convulsions
in
young
children.
Seizures associated
with
exanthem
subitum
and
HHV-6
infection
are
not
always
simple
in
type,
however.
They
are
occasionally
prolonged
and
complex
and
a
manifestation
of
encephalitis
or
encephalopathy.
See
Progress
in
Pediatric
Neurology
II.
1994,
Chicago,
PNB
Publishers,
for
a
report
and
comment
on
HHV-6
infection,
exanthem
subitum,
and
encephalitis/encephalopathy.
HHV-6
virus
DNA
was
detected
in
the
cerebrospinal
fluid
of
6
infants
with
exanthem
subitum,
3
having
a
pleocytosis and
elevated
protein
in
the
CSF.
(Suga
S
et
al.
Ann
Neurol
1993;33:597-603).
OPSOCLONUS-MYOCLONUS
OUTCOME
The
developmental
outcome
of
11
patients
with
opsoclonus-myoclonus,
8
having
occult
neuroblastoma,
is
reported from
the
Division
of
Pediatric
Neurology,
Children's
Memorial
Hospital,
Chicago.
Nine
were
treated
with
ACTH
and
3
received
prednisone.
Symptoms
recurred
in
9
when
ACTH
was
withdrawn.
The
response
to
predisone
was
minimal.
Symptoms
were
not
improved
by
removal
of
a
neuroblastoma.
The
median
age
at
presentation
was
17
months.
Follow-up ranged
from
12
to
115
months.
Delayed
development
with
motor
incoordination
and
speech
delay
occurred
in
8
children
and
3
had
behavioral
problems.
IQs
ranged
from
56
to
75
in
7
children
and
one
had
a
61
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