Frontiers in Neurology,
Journal Year:
2024,
Volume and Issue:
15
Published: July 9, 2024
Objective
Dravet
syndrome
(DS)
is
a
refractory
developmental
and
epileptic
encephalopathy
characterized
by
seizures,
delay
cognitive
impairment
with
variety
of
comorbidities,
including
autism-like
behavior,
speech
dysfunction,
ataxia.
Vagus
nerve
stimulation
(VNS)
one
the
common
therapies
for
DS.
Here,
we
aim
to
perform
meta-analysis
systematic
review
efficacy
VNS
in
DS
patients.
Methods
We
systematically
searched
four
databases
(PubMed,
Embase,
Cochrane
CNKI)
identify
potentially
eligible
studies
from
their
inception
January
2024.
These
provided
effective
rate
treating
patients
The
proportions
achieving
≥50%
reduction
seizure
frequency
were
extracted
these
studies.
Meta-analyses
performed
respectively
evaluate
after
3,
6,
12,
18,
24
36
months.
Results
Sixteen
trials
total
173
included.
showed
that
pooled
efficiency
was
0.54
(95%
CI
0.43–0.65)
treated
(
p
<
0.05).
Meanwhile,
0.42
0.25–0.61),
0.39–0.69),
0.51
0.39–0.66),
0.49
0.36–0.63)
12
months
Conclusion
This
study
suggests
treatment
However,
few
have
focused
on
DS,
there
lack
high-quality
evidence.
Thus,
randomized
controlled
are
needed
confirm
Journal of Neurophysiology,
Journal Year:
2024,
Volume and Issue:
132(1), P. 34 - 44
Published: May 22, 2024
Many
neurons
package
and
release
a
peptide
along
with
conventional
neurotransmitter.
The
view
is
that
such
peptides
exert
late,
slow
effects
on
plasticity.
We
studied
form
of
cortical
plasticity
depends
the
activity
express
both
vasoactive
intestinal
(VIP)
inhibitory
neurotransmitter
GABA.
GABA
accounted
for
their
action
plasticity,
no
effect
deleting
this
phenomenon.
bioRxiv (Cold Spring Harbor Laboratory),
Journal Year:
2024,
Volume and Issue:
unknown
Published: June 6, 2024
ABSTRACT
Cholinergic
receptor
activation
enables
the
persistent
firing
of
cortical
pyramidal
neurons,
providing
a
key
cellular
basis
for
theories
spatial
navigation
involving
working
memory,
path
integration,
and
head
direction
encoding.
The
granular
retrosplenial
cortex
(RSG)
is
important
spatially-guided
behaviors,
but
how
acetylcholine
impacts
RSG
neurons
unknown.
Here,
we
show
that
transcriptomically,
morphologically,
biophysically
distinct
cell-type
–
low-rheobase
(LR)
neuron
has
very
expression
profile
cholinergic
muscarinic
receptors
compared
to
all
other
neighboring
excitatory
neuronal
subtypes.
LR
do
not
fire
persistently
in
response
agonists,
stark
contrast
principal
subtypes
examined
within
across
midline
cortex.
This
lack
persistence
allows
models
rapidly
compute
angular
velocity
(AHV),
independent
changes
seen
during
navigation.
Thus,
can
consistently
AHV
brain
states,
highlighting
specialized
neural
codes
supporting
bioRxiv (Cold Spring Harbor Laboratory),
Journal Year:
2024,
Volume and Issue:
unknown
Published: May 14, 2024
Despite
research
illustrating
the
cerebellum
may
be
a
critical
circuit
element
in
epilepsies,
remarkably
little
is
known
about
cerebellar
engagement
during
seizures.
We
therefore
implemented
novel
method
for
repeated
imaging
of
awake,
chronically
epileptic
animals.
found
widespread
changes
calcium
signals
behavioral
seizures
Rare Disease and Orphan Drugs Journal,
Journal Year:
2024,
Volume and Issue:
3(3)
Published: July 9, 2024
Dravet
syndrome
is
a
severe
epileptic
that
begins
during
the
first
year
of
life
otherwise
healthy
babies.
Over
years,
seizure
burden
changes,
and
pathology
evolves
in
strong
association
with
behavioral
alterations,
including
cognitive
delay
autistic
traits.
Initially,
this
aspect
was
considered
direct
consequence
epilepsy
severity,
DS
defined
as
an
encephalopathy.
Increasing
evidence
suggests
these
two
aspects
disease,
impairment,
might
not
be
so
strictly
connected.
mostly
caused
by
heterozygous
loss-of-function
mutations
SCN1A
gene,
which
encodes
for
alpha-subunit
voltage-gated
sodium
channel
Nav1.1,
responsible
GABAergic
interneuron
excitability.
Interneuron
dysfunction
evident
at
symptom
onset
murine
models,
but
their
activity
appears
to
recover
chronic
phase
when
series
secondary
modifications
arise
likely
drive
phenotype.
Given
genetic
basis
disease
clear,
innovative
therapies
based
on
restoration
sufficient
expression
levels
Nav1.1
re-establish
functional
neuronal
are
being
developed.
In
work,
we
review
such
therapeutic
approaches,
specific
focus
existing
ability
address
only
also
modifications.
Science Progress,
Journal Year:
2024,
Volume and Issue:
107(1)
Published: Jan. 1, 2024
Dravet
Syndrome
(DS)
is
a
severe
developmental
epileptic
encephalopathy
with
frequent
intractable
seizures
accompanied
by
cognitive
impairment,
often
caused
pathogenic
variants
in
SCN1A
encoding
sodium
channel
Na
V
1.1.
Recent
research
utilizing
vitro
patient-derived
neuronal
networks
and
accompanying
silico
models
uncovered
that
not
just
sodium—but
also
potassium—and
synaptic
currents
were
impaired
DS
networks.
Here,
we
explore
the
implications
of
these
findings
for
three
questions
remain
elusive
DS:
How
do
impairments
result
epilepsy?
can
identical
lead
to
varying
phenotypes?
What
mechanisms
underlie
delay
patients?
We
speculate
potassium
might
be
secondary
effect
1.1
mutations
could
hyperexcitable
neurons
Moreover,
reason
homeostatic
plasticity
actively
engaged
networks,
possibly
affecting
phenotype
impairing
learning
development
when
driven
extremes.
