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Multifocal motor neuropathy: update on clinical characteristics, pathophysiological concepts and therapeutic options.
[gm1 gangliosidosis]
Multifocal
motor
neuropathy
(
MMN
)
is
an
acquired
immune-mediated
neuropathy
characterized
by
chronic
or
stepwise
progressive
asymmetrical
limb
weakness
without
sensory
deficits
.
The
upper
extremities
are
more
often
affected
than
the
lower
extremities
with
distal
paresis
dominating
over
proximal
paresis
.
Important
diagnostic
features
are
persistent
multifocal
partial
conduction
blocks
(
CBs
)
and
the
presence
of
high
-titer
anti-
GM
1
serum
antibodies
.
Motor
neuron
disease
,
other
chronic
dysimmune
neuropathies
,
such
as
chronic
inflammatory
demyelinating
polyneuropathy
and
the
Lewis-
Sumner
syndrome
(
MADSAM
neuropathy
)
,
are
important
differential
diagnoses
.
While
corticosteroids
and
plasma
exchange
are
largely
ineffective
,
high
-dose
intravenous
immunoglobulins
are
regarded
as
first
-line
treatment
.
In
spite
of
significant
success
in
elucidating
the
underlying
disease
mechanisms
in
MMN
during
the
past
few
years
,
important
pathophysiological
issues
and
the
optimum
long
-term
therapy
remain
to
be
clarified
.
The
present
review
summarizes
the
clinical
picture
and
current
pathophysiological
concepts
of
MMN
with
a
special
focus
on
the
molecular
and
electrophysiological
basis
of
CBs
and
highlights
established
therapies
as
well
as
possible
novel
treatment
options
.