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Canomad: report of a case with a 40-year history and autopsy. Is this a sensory ganglionopathy with neuromuscular junction blockade?
[waldenström macroglobulinemia]
An
80
-
year
-old
man
had
a
40
-
year
history
of
chronic
sensory
ataxic
neuropathy
and
11
years
of
relapsing
/
remitting
episodes
of
rapid
deterioration
with
perioral
paresthesiae
and
weakness
of
bulbar
,
respiratory
,
and
limb
muscles
.
An
immunoglobulin
M
(
IgM
)
paraprotein
was
detected
12
years
before
death
,
and
Waldenstrom
macroglobulinemia
was
diagnosed
on
bone
marrow
biopsy
3
years
before
death
.
Chronic
ataxic
neuropathy
with
ophthalmoplegia
,
IgM
paraprotein
,
cold
agglutinins
,
and
anti-disialyl
antibodies
(
CANOMAD
)
was
diagnosed
.
Comprehensive
autopsy
showed
severe
dorsal
column
atrophy
and
dorsal
root
ganglionopathy
.
A
different
pathology
was
identified
in
cranial
and
peripheral
nerves
,
dorsal
roots
,
and
cauda
equina
,
comprising
infiltration
of
clonal
B-
lymphocytes
within
the
endoneurium
,
perineurium
,
and
leptomeninges
.
The
autopsy
provides
evidence
of
the
pathogenesis
of
the
relapsing
remitting
component
of
CANOMAD
,
and
we
postulate
that
this
may
relate
to
the
presence
of
clonal
IgM
anti-disiayl
gangliosides
secreting
B-
lymphocytes
within
nerves
.
Diseases
Validation
Diseases presenting
"episodes of rapid deterioration with perioral paresthesiae"
symptom
waldenström macroglobulinemia
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