JAMA Neurology,
Journal Year:
2023,
Volume and Issue:
81(1), P. 50 - 50
Published: Nov. 27, 2023
Importance
Although
up
to
20%
of
patients
with
multiple
sclerosis
(MS)
experience
onset
before
18
years
age,
it
has
been
suggested
that
people
pediatric-onset
MS
(POMS)
are
protected
against
disability
because
greater
capacity
for
repair.
Objective
To
assess
the
incidence
and
factors
associated
progression
independent
relapse
activity
(PIRA)
relapse-associated
worsening
(RAW)
in
POMS
compared
typical
adult-onset
(AOMS)
late-onset
(LOMS).
Design,
Setting,
Participants
This
cohort
study
on
prospectively
acquired
data
from
Italian
Register
was
performed
June
1,
2000,
September
30,
2021.
At
time
extraction,
longitudinal
73
564
120
centers
were
available
register.
Main
Outcomes
Measures
The
main
outcomes
included
age-related
cumulative
adjusted
hazard
ratios
(HRs)
PIRA
RAW
factors.
Exposures
Clinical
magnetic
resonance
imaging
features,
receiving
disease-modifying
therapy
(DMT),
first
DMT.
Results
After
applying
inclusion
exclusion
criteria,
assessed
16
130
(median
[IQR]
age
at
onset,
28.7
[22.8-36.2
years];
68.3%
female).
Compared
AOMS
LOMS,
had
less
disability,
exhibited
more
active
disease,
exposed
DMT
a
longer
period.
A
48-week-confirmed
occurred
7176
(44.5%):
558
(40.4%),
6258
(44.3%),
360
LOMS
(56.8%)
(
P
<
.001).
Factors
older
(AOMS
vs
HR,
1.42;
95%
CI,
1.30-1.55;
2.98;
2.60-3.41;
.001),
disease
duration
(HR,
1.04;
1.04-1.05;
shorter
exposure
0.69;
0.64-0.74;
1.3%
20
but
rapidly
increased
approximately
7
times
between
21
30
(9.0%)
nearly
doubled
each
decade
40
70
(21.6%
years,
39.0%
50
61.0%
60
78.7%
years).
events
followed
similar
trend
(0.5%
3.5%
7.8%
14.4%
24.1%
years);
no
further
increase
found
(27.7%).
Delayed
initiation
higher
risk
1.16;
1.00-1.34;
=
.04)
1.75;
1.28-2.39;
Conclusions
Relevance
can
occur
any
although
pediatric
is
not
fully
protective
progression,
this
study’s
findings
suggest
likely
exhibit
over
follow-up.
However,
these
also
reinforce
benefit
POMS,
as
treatment
reduced
occurrence
both
regardless
onset.
Autoimmunity Reviews,
Journal Year:
2023,
Volume and Issue:
22(12), P. 103465 - 103465
Published: Oct. 16, 2023
Neuromyelitis
optica
spectrum
disorder
(NMOSD)
is
a
rare
relapsing
neuroinflammatory
autoimmune
astrocytopathy,
with
predilection
for
the
optic
nerves
and
spinal
cord.
Most
cases
are
characterised
by
aquaporin-4-antibody
positivity
have
disease
course,
which
associated
accrual
of
disability.
Although
prognosis
in
NMOSD
has
improved
markedly
over
past
few
years
owing
to
advances
diagnosis
therapeutics,
it
remains
severe
disease.
In
this
article,
we
review
evolution
our
understanding
NMOSD,
its
pathogenesis,
clinical
features,
treatment
options
symptoms.
We
also
address
gaps
knowledge
areas
future
research
focus.
JAMA Neurology,
Journal Year:
2023,
Volume and Issue:
81(1), P. 50 - 50
Published: Nov. 27, 2023
Importance
Although
up
to
20%
of
patients
with
multiple
sclerosis
(MS)
experience
onset
before
18
years
age,
it
has
been
suggested
that
people
pediatric-onset
MS
(POMS)
are
protected
against
disability
because
greater
capacity
for
repair.
Objective
To
assess
the
incidence
and
factors
associated
progression
independent
relapse
activity
(PIRA)
relapse-associated
worsening
(RAW)
in
POMS
compared
typical
adult-onset
(AOMS)
late-onset
(LOMS).
Design,
Setting,
Participants
This
cohort
study
on
prospectively
acquired
data
from
Italian
Register
was
performed
June
1,
2000,
September
30,
2021.
At
time
extraction,
longitudinal
73
564
120
centers
were
available
register.
Main
Outcomes
Measures
The
main
outcomes
included
age-related
cumulative
adjusted
hazard
ratios
(HRs)
PIRA
RAW
factors.
Exposures
Clinical
magnetic
resonance
imaging
features,
receiving
disease-modifying
therapy
(DMT),
first
DMT.
Results
After
applying
inclusion
exclusion
criteria,
assessed
16
130
(median
[IQR]
age
at
onset,
28.7
[22.8-36.2
years];
68.3%
female).
Compared
AOMS
LOMS,
had
less
disability,
exhibited
more
active
disease,
exposed
DMT
a
longer
period.
A
48-week-confirmed
occurred
7176
(44.5%):
558
(40.4%),
6258
(44.3%),
360
LOMS
(56.8%)
(
P
<
.001).
Factors
older
(AOMS
vs
HR,
1.42;
95%
CI,
1.30-1.55;
2.98;
2.60-3.41;
.001),
disease
duration
(HR,
1.04;
1.04-1.05;
shorter
exposure
0.69;
0.64-0.74;
1.3%
20
but
rapidly
increased
approximately
7
times
between
21
30
(9.0%)
nearly
doubled
each
decade
40
70
(21.6%
years,
39.0%
50
61.0%
60
78.7%
years).
events
followed
similar
trend
(0.5%
3.5%
7.8%
14.4%
24.1%
years);
no
further
increase
found
(27.7%).
Delayed
initiation
higher
risk
1.16;
1.00-1.34;
=
.04)
1.75;
1.28-2.39;
Conclusions
Relevance
can
occur
any
although
pediatric
is
not
fully
protective
progression,
this
study’s
findings
suggest
likely
exhibit
over
follow-up.
However,
these
also
reinforce
benefit
POMS,
as
treatment
reduced
occurrence
both
regardless
onset.