features and response to corticosteroids. Chest. 1984;. 86:723-28. 8 Sterling. KM, ... Mindy. Aisen,. M.D.;t. Geoffrey. Arlt, M.D.; and. Steven. Foster, M.D.. A patient.
Diaphragmatic paralysis without bulbar or limb paralysis in multiple sclerosis. M Aisen, G Arlt and S Foster Chest 1990;98;499-501 DOI 10.1378/chest.98.2.499 The online version of this article, along with updated information and services can be found online on the World Wide Web at: http://chestjournal.chestpubs.org/content/98/2/499
Chest is the official journal of the American College of Chest Physicians. It has been published monthly since 1935. Copyright1990by the American College of Chest Physicians, 3300 Dundee Road, Northbrook, IL 60062. All rights reserved. No part of this article or PDF may be reproduced or distributed without the prior written permission of the copyright holder. (http://chestjournal.chestpubs.org/site/misc/reprints.xhtml) ISSN:0012-3692
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processes.
Bleomycin
as a model that
instilled
for pulmonary
glucocorticoids
following
can
bleomycin
cells
in the
wide
variety
foreign been
Steroids these
of
can
also
parenchyma.
lung both
cumulative
dose
several mg);
may
support, can
be
generation the
at
of
patient’s
(1) severe
a relatively
toxicity,
ie,
abnormalities aggressive
We
with
concur
from
is presented
compromise
those
who
screen
PFTs both before early pulmonary
respiratory respiratory
the
The
Department
pathologic
authors
of Surgical
findings;
Mr.
Hospital Respiratory the manuscript; and
Allen
thank the
Rochester
Methodist
Department for his helpful review Johnson for her aid in its preparation.
muscle muscle
espiratory
of
Carter
2 Samuels
ML,
bleomycin
prior 3
Johnson
RM,
K. A clinical
review
Cancer
1973;
31:903-14
Holoye
PY, Lanzotti
DE, and
pulmonary
JAMA
1976;
toxicity:
ofdrug,
of bleomycin-
stem
Large
VJ.
in patients
SB.
iron
treated
LaMantia patients.
6 Van
Click
KR,
not cause
does
and
Activated
JH,
degrades
failure
Anesthesiology
1984;
PWC,
Mulder
Bleomycin
and
pulmonary
DT.
BE.
Howland
morbidity Br Med J
Supplemental
and 1978;
oxygen
in bleomycin-treated
surgical
60:65-67
NH,
Van der toxicity
In
a DNA.
Mark
DA,
Stover
features
J Med
Neth
1985;
DE.
Severe
and
bleomycin-induced
response
to
had
February
1982,
KM,
Di PetrilloT, accumulation
intratracheal
bleomycin
Cutroneo by
expiratory
static
living
MEl
in MS”2
stem
and
that
high
revealed
patient
presented
paralysis
has
cervical
an unusual
MS who
spinal
Chest
with
without
extensive
the brain
abnormality
cord. REPORT
loss
in that
eye.
The
normal
fundi,
pupillary
evoked
potentials
analysis
revealed
99;
WBC,
the
vision.
months
KR,
Prestayko
glucocorticoids
instillation.
in
Cancer
Res
A. Inhibition rat
lung
1982;
after
defect.
The
were
normal
RBC,
with
later with
in her
the
symptoms
next
adrenal
woman progressive
and was
that
20/20
OS
normal
abnormal
and
OD.
protein,
The 67;
treated intravenously’ experienced gradual
developed
arm
experienced
and
she
and
an
visual CSF
glucose,
with return
leg weakness
incomplete
recovery
slowly
progressive
arm.
four
years
the
urgency,
loss
but was
the patient
left
scan
and
She
by revealed
OD
present;
She
ACTH.
of urinary
increasingly
sone;
OS bands
0.
healthy
followed
examination
CT brain
oligoclonal
treated
days,
of 20/409
(Solumedrol)
Three
previously
five
physical
acuity
12;
of normal was
for
visual
methylprednisolone
and
41-year-old
pain
Over 1984;
this
eye
proprioceptive
of collagen
or
98:499-501)
of daily
finding
brain
diaphragmatic
right
afferent
pneumonitis:
corticosteroids.
86:723-28 8 Sterling
is a rare
We describe
The cervical
of strength
clinical
of
upper
experienced
TW, Sleijfer
28:516-23 7 White
1990;
maximal activities
nondisabling
symptoms.
visual 0,
bleomycin.
Marshall
Barneveld
involve-
of
bleomycin:
that
oxygen
with
respiratory
cord
dysfunction
dose role
1:1664-67 5
Pmax ADL
symptoms.3’4
onset
of the
a possible
J Biol Chem 1981; 256:11636-43 Goldiner PL, Carlon GC, Cvitkovic E, Schweitzer WS, et al. Factors influencing postoperative mortality
symp-
235:1117-20
J, Horwitz
Peisach
complex
spinal
bulbar (Chest
CASE
radiotherapy
Burger
Agre agent.
therapy
transient 4
5K,
antineoplastic
from
with
longstanding
insidious RH,
pulmonary
to cervical
pressure; pressure;
associated
cord
with
REFERENCES
a new
due
compromise
been spinal
1 Blum
disabling
Dr. Thomas A. Gaffey for his review of the
Pathology
Kendallfrom
Therapy Ms. Lyn
in whom
Mill = magnetic resonance imaging; MS multiple sclerosis; OD = right eye; OS left eye; CTcomputed tomography; CSF cerebrospinal fluid; ACTH adrenocorticotropic hormone; ECG electrocardiogram; pH negative logarithm of hydrogen ion activity; Pco1 partial pressure ofcarbon dioxide; Po, partial pressure of oxygen; VC vital capacity; FEV forced expiratory volume; Pimax maximal inspiratory static
the
toxicity.
ACKNOWLEDGMENTS:
and
toms in the absence of other significant disability complicated a long-standing course of MS. Clinical presentation and fluoroscopy confirmed the diagnosis of bilateral diaphragmatic paralysis. Magnetic resonance imaging revealed atrophy and extensive white mailer changes within the cervical cord. This case is unique in that significant ment by MS was dissociated profound limb paresis.
high
to support
patients reularly with in an effort to detect
Foster, M.D.
M.D.;
long-term
management;3
temporarily
Steven
Arlt,
Geoffrey
markedly
with
as required
M.D.;t
respiratory
low
hypoxemia
and
reverse
Aisen,
A patient
release
points:
occur
partially
used.
bleomycin-treated and during treatment
has
chemotactic our
important
(2) severe
warranting
concentrations,
anion
Mindy
without in
only
Bleomycin
to inhibit
can
radiographic
Dco,
a
not
to glucocorticoids because they development of an actual fibrotic
the
(240
are key release
damage
Presumably
toxicity
extensive
diminished
shown
agents.
illustrates pulmonary
patient,
been
responded before
patient
accumulation They
superoxide
Diaphragmatic Paralysis Bulbar or Limb Paralysis Multiple Sclerosis*
used shown
macrophages
as well as neutrophil
have
bleomycin
Flo,
which
reactive
pneumonitis were instituted
steroid
products
macrophages,>
both
at rest,
lung.
but
activity.’0
process. Our
of the
be
been
collagen
defenses
to increase
by alveolar
lung
it has
Alveolar
of toxic
shown
inhibit
can
and
therapy.>
immune
invaders
intratracheally
fibrosis,
in her frequent
insufficiency
gait distal
patient
had
impairment, lower
courses
and
extremities. of orally
developed
and
paresthesia She
treated
she
needed
administered eventually
and
was
predni-
42:405-
08 9 Conley
NS,
increases
macrophages. 10
Wesselius
Yarbro
Ferrari
JW,
superoxide
ani(In
Mol Pharmacol U,
tactic
activity
mycin
injury.
Catanzaro Rev
1986; by
Respir
alveolar Dis
RB.
Bleomycin
by pig peripheral
Zeidler
alveolar
30:48-52
A, Wasserman
generation Am
HA,
generation
1984;
SI.
Neutrophil
macrophages 129:485-90
chemoafter
bleo-
*From
the
Cornell tAssistant Instructor, §Assistant
University Professor Department Professor requests: Dr.
Reprint
NY
Burke
Rehabilitation
Center,
White
Plains,
NY; and
Medical College, New York City. of Neurology. of Neurology. of Medicine and Department of Medicine. ALsen, 785 Mamaroneck Avenue, White Plains,
10605
CHEST
I 98 I 2 I AUGUST,
Downloaded from chestjournal.chestpubs.org by guest on July 10, 2011 © 1990 American College of Chest Physicians
1990
499
.
1 T2-weighted al)normalities.
F1:URE
matter maintenance logic
cortisone
symptoms,
pattern
treatment.
negative
>f netmrollOgic
firmed.
Adrenal
family
magnetic
Based
ous age
history,
CSF
deterioration,
insufficiency
resonance
at o>nset
ascribed
o>f MS
movement
(obtained
con-
36 mm
exogenous
the
In June
1987,
at tile
breath on >rthopnea.The
exertio>n.
ness
The
(If
was
previously
no
and
Burke
1 1: packs years
sounds motion
when
sitting,
but
recumbent
improlvefor
of cigarettes
per
at the
was
hmng bases She
in
short
4 +/5
the
h>wer iii
disclosed
strength
in
extremities
the
left
(with
extremity,
in a C2-4
right
circumducti>n
of
Disability ofrotmtine
revealed
the
no>rmal
Statums
Scale
lahoratory’
tests
l)ilateral
lower
elevation.
ankle
lower score
Fhmoroscopy’
and lobe
clonus
extremthc was
evidence
o>f atr>phy
mnagntmm
to> C5.
diaphrag-
c>rd
were
were
evident.
(If
breath
assuming
a
status,
right 4/5
throughotmt),
Treatment
at the
to eniplo>y
labor-saving
and
were
atelectasis revealed
t> inipro>ve
pursed Six
examninatio>n
regio>n
Bimrke
patterns,
rehabilitation, later
ss’ere
the
from
and
the
abnormalties the
perfurming endumrance
orthopnea
and
forearm
patient
all ADL
diaphragmatic
ADL
altho>umgh symptoms
the
o>f training when
were revealed
within
stem
emphasizing
exercise
magnet
30-100)
o>f brightness
consisted
techniques
o>f
defect.
Images
1). N> llrain
Cemiter
task
TE
extending
areas
(Fig
percent
condumctive
thickness.
cord
was
percent
of predicted). (.54
ventilato>ry
simper
(TR-2(XXI,
focal
(VC
L (53
percemlt of 1)redicte(l) of predicted). These
I)ercellt
5-mm
while
FEy,,
IJls1
(28
tesla
Pco2, valime o>f
Pulmonary
percent
55
ll()
a 1.5
spinal
breathing
after m>nths
84 (103
values
(Il)tained
of 1.34
restrictive
with
the
Her
he
n>t
VC and
images
plane
lip breathing.
improlved
of all impaired
weighted
in this
co>uld
reduced
24 cm II,() (29
In addition,
iu>ted
saturation
vo>lmmmiie was
with
(If
an o>xy’gen
amid FEV,, was
gas
of7.42;
p11
dyspnea.
a mn(>(lerate
sagittal
decreased
and
significantly’
persisted.
findings
of
of the
central
the
physical
tmnchanged.
amid extensor umnassisted The
with
DiscuSSION
Kurtzke Respiratory
4#{216}5
ECG
TR
a
to> excessive
rati>
I)erformed
in the
markedly
amhtmlated
was
MRI
obtained
perceptio>n
and
inolicated
hut
extremity,
tone
decreased
distrihtmtion
left
mental
FEV,NC
l)lood
showed
values
moderately
Pmmax was vas 45 cm
PF:mliax
20 years,
upon
tipper
Tile
The
f>r
gas dome
of predicted)
The
white
arterial
upright)
11g. with
mnaxinmummn vemitilatorv
(Philips).
no> sho>rtness
normal the
revealed percent
She
parado>xic
(If breath
proprioception in all fingers. Bilateral plantar responses were present. She
diaphragmatic
with
exhibited
acutely
examination
modalities
film
no>table
tests
irritants. for
84 mm
supine
and
Admuission
was
blood
was
allno)rmalities
to> admission.
recummhent.
pallor,
dysmetria
Expanded Results
day
P(>,,
atro>phy
test”.
patient
Arterial
predicted). and the
Center
indtmstrial
and
L, 47
The
positRln.
disc
sensory
500
umpon
hg;
revealing
“sniff
the
ofpredicted).
with intravenously and
to
o>n admission
became
The neurologic strength
prior
bilaterally
matic
x-ray
1.58
ohstrumctive
Rehahilitatio>n
>n the
while
ftmnctiom
for short-
chronic
was treated
o>f exposure
examinatioln
while
mild
shortness progressive
holspitalization
bronchodilators the
no>ted
cord
patient
A
history
five
Physical
mild
to
smoked
had stopped
optic
develo>ped
rehahilitation.
There
breath
first
she required
and
transferred
pulmonary
patient
ofpneumonia
The patient
antibiotics
was
the
eventually’
diagno>ses
were made.
administered
46,
(If
Subsequently
patient
ofbreath.
lung disease
age
spinal
95 percent.
suppression.
ment
cervical
and was
to chro>nic
>f the
of neumro-
al)nolrmalities
a diagnosis
was
image
no>rmal. with
A chest bilateral
mio diaphragmatic
system
occurs
colrd
lesions.
the
reticulum
failure
due
in the
setting
Inv(Iluntary of
the
to
disease ofbrain
stem
respiratory lower
brain
Diaphragmatic
Downloaded from chestjournal.chestpubs.org by guest on July 10, 2011 © 1990 American College of Chest Physicians
and
control stem
Paralysis
nervous
cervical
spinal
centers
l)etween
in MS (Aisen,
lie the
in
mid-
Ar/I, Foster)
patients
pons and the base of the medulla. Behavioral respiratory control resides in prepontine structures.7 Outflow pathways to the phrenic nerve are located in the upper (C2-C5) cervical
spinal
cord.
remain
study
ambulatory
of respiratory
are
suggests
unlikely
function.m0
that
to have
In 1952,
MS
patients
serious
Guthrie
tion
patient,
which
revealed
3 Guthrie
compromise
active
Boor
developed
positional
C2-C5
distribution
gastric
pressure
during
tidal
phragm
weakness.
increased respiratory This
and
urinary
days
limb paresis, compromise,
patient
features
indicated
with
an incomplete pathways
high
were
respiration
on
a background
patient
discrete
of acute
subsequently
died
bulbar
and
plaque
of the lower reported loss automatic
involving dorsomedial medulla. In contrast, Noda of voluntary
breathing
The
present
reports
of
exhibited normalities.
setting
differs
respiratory
of acute
dysfunction
in
MS.
a
regions
or
performance
of ADL.
The
of
SC,
Utell
dysfunction
L, Goldman
Wong,
Roy
Patterson,
We
report
disabling
F.C.C.?
a case man.
The
the diagnosis
to corroborate
of bilateral
in which
MRI
paralysis
was
The months weakness
states.
confirmation
patient’s before is
Careful
of the
pulmonary the etiology frequently
evaluation
diagnosis.
overlooked
of pulmonary
event
cramps, of IA.
gressed
urticaria,
to develop
interval.
This
threatening
!A=
in
present for Diaphragmatic
neurologic
dysfunction
ed.
in a case
21:563-66
CM.
Severe
sclerosis.
DJ. The
diaphragm
Arch
assessment
fatal
Neurol
of diaphragm
with
began
reaction
I diopathic systemic
six
disease
in MS
collapse
in a 76symp-
and vomiting subsequently pro-
patient
cardiovascular
and eventually infarction that the
cardiac
and
nausea,
flushing,
events
in
gastrointestinal
diarrhea, The
FCC.?;
cardiovascular
anaphylactic
myocardial emphasizes defined
M.D.,
Greenberger,
hypotension,
cardiovascular previously
idiopathic
*Fmm
symptoms were was diagnosed.
4th
breathing
1981;
from
symp-
cardiovas-
over a five-hour potential for lifeIA exists
in patients
risk factors.
(Chest
made clinically at the bedside. Fluoroscopy alone may be unreliable,” but the chest x-ray film findings, the patient’s spirometry result, inspiratory and expiratory muscle strength as well as the patient’s clear history with the absence of other conditions that can give similar symptoms facilitated
coma.
of
56:165-69
in vivo.
diaphragmatic
and
multiple
of nearly
toms of abdominal as manifestations
without
diagnosis
stupor
Wiles
in
to an idiopathic
year-old
patient
M.D.,
clinical The
J,
St
1977;
PaulA.
and case
is available
the diagnosis
17:79-83
sclerosis. Thorax 1985; 40:633-34 JF, Rudick BA, Herndon RM . Pulmonary
M.D.;
collapse
case
1983;
Nearly Fatal Idiopathic Anaphylactic Reaction Resulting Cardiovascular Collapse and Myocardial lnfarction*
cular
such
and
of voluntary
M, Newsom
Medicine
pain,
first
Neurol
45:1245-49
of chest
is the
P.
and
toms
This
sclero-
(EDSS).
1966;
Clin Neurol
Trend
symptom was dyspnea, which developed insidiously and has now been present for over one year without increase in other neurologic signs. The MEl revealed extensive involvement of the upper cervical spinal cord, confirming the impression.
of
Paralysis
Smeltzer function
aftributable
previous
The
only
H.
in multiple
Sansan
have
corticospinal dysfunction but no brain stem abLimb paresis did not significantly interfere with
ambulation
1977;
1982
sclerosis.
CB,
function.
quadriplegia. from
of
Neurol
cord
preservation
substantially
paralysis
Arch
in multiple
scare
Badiol
Diagnosis
weakness
Loh
11
JB.
S, Umezaki
1988;
revealed
central
with
in 1952;
consid-
and Umezaki>
breathing
in the case
and
Med
Reversible
movements Clin
FA Davis,
with multiple
10
dysfunction.
autopsy
failure
Intern
impairment status
Diaphragmatic
Posner
9 Cooper
erable corticospinal tract functions remained. The literature also contains two reports of central disturbances of respiration in MS referable to the brain stem. In 1977, Boor et al’ described reversible paralysis of automatic The
L.
sclerosis.
neurological
paralysis.
F,
8 Noda a
case.
but
sclerosis.
respiratory Ann
Canalese
disability
Philadelphia:
of dia-
cervical
affected,
F. Acute
in multiple
Rating
C.
7 Plum
and
present
of multiple 50:431-39
its management.
RJ,
expanded
diaphragmatic
developed
the
JF.
an
Alexander
6
gradient
diagnosis
the
1943;
history
33:1444-52
tongue fasciculations and greater necessitating ventilatory support.
some
Diaphragmatic
the
later
Prognosis
natural
21:135-67
34:686-89 sis:
in
Esophageal
no pressure
confirming
Fifteen
shared
hesitancy.
loss
1952;
AP Berlin
of the
Med
Psychiat JR.
and
respiration
5 Kurtzke
man with acutely
sensory
revealed
breathing,
signs
lesion.
marked
monitoring
patient
Initially,
dyspnea,
Krutzke
Johnson
JW,
automatic
posterior and lateral columns of lower cervical segments as well as the entire cross section of the upper thoracic cord. Severe diaphragm weakness producing respiratory failure MS
McIntyre
sclerosis
aspects
J
34:1197-1203 4
demyelina-
A 41-year-old progressive
Some Quart
Neurosurg
TC,
multiple
of the
in a MS patient has been reported.> a three-year history of relapsing
HD,
Arch Neurol
and intervention.
NCES
N.
sclerosis.
2 McIntyre
who
et al described
extensive
D, Comston
ofdisseminated
acute respiratory failure in four MS patients. Each developed acute quadriparesis with decreased thoracic excursions following the onset of dyspnea. Autopsy results were available in one
McAlpine
1
A recent
detection
REFERE
Respiratory failure in patients with MS is rare, but when reported has been associated with significant bulbar or limb paralysis.’’>’>
may lead to earlier
1990; 98:501-03)
anaphylaxis
anaphylaxis of unknown is definedetiologyas reaction
the Section
of Allergy-Immunology
a in life-threatening which there Department
is
of Medi-
cine, Northwestern University Medical School, Chicago. Supported by Public Health Service grant A! 1 1403 and the Ernest S. Barley Grant. Reprint requests: D Greenberger, 303 East Chicago Avenue, Chicago 60611
CHEST
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1990
501
Diaphragmatic paralysis without bulbar or limb paralysis in multiple sclerosis. M Aisen, G Arlt and S Foster Chest 1990;98; 499-501 DOI 10.1378/chest.98.2.499 This information is current as of July 10, 2011 Updated Information & Services Updated Information and services can be found at: http://chestjournal.chestpubs.org/content/98/2/499 Cited Bys This article has been cited by 1 HighWire-hosted articles: http://chestjournal.chestpubs.org/content/98/2/499#related-urls Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.chestpubs.org/site/misc/reprints.xhtml Reprints Information about ordering reprints can be found online: http://www.chestpubs.org/site/misc/reprints.xhtml Citation Alerts Receive free e-mail alerts when new articles cite this article. To sign up, select the "Services" link to the right of the online article. Images in PowerPoint format Figures that appear in CHEST articles can be downloaded for teaching purposes in PowerPoint slide format. See any online figure for directions.
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