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COMMENT:
This
syndrome
was
first
described
by
Segawa
M
et
al
in
Japan
(Therapy
1971;24:667)
and
should
correctly
be
referred
to
as
"Segawa
Syndrome".
Diurnal
fluctuation
of
the
dystonia
is
not
invariably
present
and
a
trial
of
levodopa
is
worthwhile
in
possible
variants
of
this
dystonic
syndrome.
Emotional
disturbance
is
a
feature
in
some
cases
and
may
lead
to
a
diagnosis
of
psychogenic
etiology.
In
fact,
in
all
cases
of
dystonia
musculorum deformans
(torsion
dystonia)
that
I
have
treated,
a
diagnosis
of
conversion
hysteria
had
previously
been
entertained
and
pyschotherapy
prescribed.
CONGENITAL
CMS
DEFECTS
HYDROCEPHALUS
AND
SHOOT
INFECTIONS
In
the
10-year
period,
1973-82,
431
children
underwent
cerebrospinal
fluid
shunt
insertion
for
hydrocephalus
at
Children's
Memorial
Hospital,
Chicago.
The
authors,
now
in
Verona,
Italy
(Casella
Postale
401.1-37100),
have
studied
the
relationship
between
the
etiology of
hydrocephalus,
age
at
the
time
of
shunt
placement,
and
infection
ate.
Meningomyelocele
was
present
in
40%,
congenital
communicating
or
obstructive
hydrocephalus
in
34%,
and
tumors
in
18%.
Intraventicular
hemorrhage
and
meningitis
were
the
causes
in
5%
and
3%,
respectively.
The
age
at
surgery
was
less
than
1
year
in
83%
and
1
week
or
younger
in
18%.
Each
patient
had
an
average
of
3
procedures.
Infections
occurred
as
a
complication
of
the
shunt
in
96
patients
at
rates
of
22%
per
patient
and
6%
per
procedure.
Younger
patients
and
those
with
meningomyelocele
were
most
susceptible
to
infection.
In
the
meningomyelocele
group,
infection
occurred
less
often
when
shunted
at
2
weeks
of
age
or
later,
compared
to
1
week
or
earlier,
when
the
rate
was
48%.
(Ammirati
M,
Raimondi
AJ.
Cerebrospinal
fluid
shunt
infections
in
children.
Child's
Nerv
Syst
1987;4:106-109).
COMMENT:
The
rate
of
operative
shunt
infection
reported
in
this
study
is
high,
and
the
authors
are
able
to
cite
similar
statistics
from
two
other
centers.
Attempts
to
reduce
the
incidence
of
infection
by
perioperative
antibiotics
or
a
surgical
isolator
had
not
been
successful.
If
a
rate
of
infection
of
20%
or
more
per
patient
is
the
rule
with
the
operative
treatment
of
hydrocephalus,
a
reappraisal
of
techniques
and
indications
for
surgery
would
seem
to
be
a
necessity.
Recent
experience
at
Children's
Memorial
Hospital
indicates
a
rate
of
infection
lower
than
that
reported
here,
and
Dr.
Luis
Yarsagaray
at
Loyola
Stritch
Medical
Center,
Chicago,
recalls
only
3
cases
of
shunt
infection in
a
total
of
2000
patients
of
all
ages,
both
children
and
adults,
that
he
has
himself
treated
by
surgery
over
a
17
year
period
(personal communication).
ARNDID-CHIARI
WITH
MYELOMENINGOCELE
The
outcome
of
19
infants
with
complications
of
Arnold-Chiari
malformation
and
meningomyelocele
was
reviewed
at
the
Depts.
of
Pediatrics,
Pathology,
and
Neurosurgery,
University
of
Pennsylvania
School
of
Medicine
and
the
Children's
Hospital
of
Philadelphia.
Vocal
cord
paralysis
and
inspiratory
stridor
alone
occurred
in
10
(grade
I),
apnea
was
an
additional
symptom
in
4
(grade
II),
and
cyanotic
spells
and
dysphagia
were
associated
in
5
(grade
III).
28
