paralysis in multiple sclerosis. Diaphragmatic ...

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,


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


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


I 98 I 2 I AUGUST,

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.
