amyotrophic lateral sclerosis presenting with

8/8/2019 Amyotrophic Lateral Sclerosis Presenting With

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DOI 10.1378/chest.71.5.6121977;71;612-614Chest

G B Fromm, P J Wisdom and A J Block

patients.dependence on mechanical ventilation in tworespiratory failure. Diaphragmatic paralysis andAmyotrophic lateral sclerosis presenting with

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College of Chest Physicians, 3300 Dundee Road, Northbrook, IL 60062. Allhas been published monthly since 1935. Copyright 1977 by the American

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1977 American College of Chest Physiciansby guest on September 1, 2010chestjournal.chestpubs.orgDownloaded from
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F IGURE 1 . An t e r i or h o r n o f c e r v i ca l c o r d (case 1 ). N eu ronald epopu lation associated w ith g lio sis is n oted . T w o deg ene r-ating anterio r h orn cells are seen at le f t (Hema to xy l i n an deosin , o rig inal m agn f f ication x 400) .

F IGURE 2 . A n te r io r horn o f ce rv ic al cord (case 2). Degener -ating an te rio r ho rn cell i s seen in upp er f ie ld . L ipo f u sc in -f illedapp earing ante rio r horn ce llHema t o x y l i n an d eosin , originalmagnif ica tion x 400) .

CHES T, 7 1 : 5 , MAY, 1 9 7 7 A M Y O T R O P H IC L A T E R A L S C LE R O SIS 613

o f th e co rticospin al trac ts w as no ted , and the cortex and th ebrain stem w ere norm al.CASE 2

Th i s patien t w as a 69 -y ear-o ld w h ite m anith a pastm ed ical h is to ry o f alcoh o l abu se , 50 pack -y ears o f c igare ttesm ok ing , and reported chro n ic ob stru ctiv e pu lm onary dis -ease . R esu lts o fpulmonary f unc tion te sts perf o rm ed at h islocal hospital six m onth s be fore adm ission w ere though t toshow airw ay ob stru ction and inc lu ded the f o l l ow in g va lues :fo rced vita l capac ity , 2 .25 L ; f o rced ex p irato ry v o lum e inone second , 1 .72 L ; PaO 2 , 72 m m H g; PaC O 2 , 38 m m H g;and pH , 7 .47 . O n N o v 1 1 , 1 975 , generalized wea k n e s s ,p leu ritic ches t p ain , andshortness o f b reath b egan and ledth e patien t to seek m edical atten tion on N ov 29 , 1 975 .

T he p atien t w as believ ed to be in acu te re sp irato ry d is-tre ss , and an endo tracheal tu be w as p laced . In itially , h ew astreated w ith a v o lum e-con tro lled v en tilato r (Benn e t t M A - i ),antib io t ics, an d w ith heparin fo r suspec ted pulmonary emb o l i .During th e en suing tw o w eek s t h e p a t i en t s c o n d i t i on r e -sponded we l l ; h owe v e r , severa l attempts at w ean ing , ev enw ith equipm ent f orI M V , wer e unsuccessfu l . T he patien t w astransf e rred to the Gainesvil le (Fla) V eterans A dm inistratio nHospita l on D ec 16 , 19 75 , to b e w eaned f rom mechanicalv en tilato ry supp ort.

O n adm iss ion the patien t w as ale rt, cooperativ e , in nodistress, and on a v en tilato r (Em erson ). R ale s w ere heard atboth bases o f the lu ngs , bu t f in d ing s f rom th e re st o f th eph y sical ex am ination w ere no rm al. T heeurolo gic ex asnina-tio n rev ealed m ild generaliz ed w eak ness an d m uscu lar w as t-ing , w i th norma l sen sation . D eep tendo n re f lex e s w ere b risk ,ex cep t f o r absen t ank le re f le x e s, and the f ind ing s f rom th erem ain der o f the examination w ere norm al.

Laboratory data revealed le v e ls o f elec tro ly te s, b lood urean itrogen , creatin ine , calcium , phospho rus , transarn inases ,v itam in B 12 , m agnes ium , and f o late , as w e ll as the resu ltso f urinalysis, to be no rm al. A rte rial b loo d g as lev els w ere asf o llow s : PaO 2 , 7 8 m m H g; PaCO ,, 36m H g; and pH ,7 . 4 5 . T h e r a t e o f I MV wa s 6 / mm and th e Flo , w as 0 .4 . Aches t x -ray f ilm d isc losed bilateral in f il trates o f the low erl o be s c omp a t i b l e w ith pn eum on ia.

T he p atien t w as at f irst be liev ed to hav e chron ic o bstru ctiv epulmonary disease with acu te hac tenal pn eum on ia and pos -s ib le pu lm onary em bo li. W ith in six hou rs o f adm iss ion , h isarte rial b lood gas le v e ls w ere s tab le, de sp ite a reduc tion o fthe re sp irato rs rate o f IM V to 2 /m m . A t th is po in t th e p atien th imse l f rem o v ed the endo tracheal tube . O v er the nex te n

hours, his cond ition p rogres siv e ly de te rio rated , w ith increas -ing dy spn ea and sev ere respiratory ac ido sis. T he patient wa srein tubated and again treated w ith aen tilato r (Em erson ).

Shortly th e reaf ter, m oderate distal m uscu lar w eak n ess,w i th prominen t fascicu la tions, w as n o ted . O n Jan 8 , 1976 ,f luo ro scop ic examination of the ch est rev ealed paradoxicalm ov em en t o f th e righ t d iap hragm and no m o v em en t o fth e le f t. T he re su lt o f a te s tw i th edroph on ium ch lo ride(T ensilo n) w as nega t ive . Electrodiagnostic s tu d ies w ere per-f o rme d and show ed normal neural condu c tion s and a norm alev ok ed -po ten tial e lec trom yogram . Need l e s tu dy o f striatedmu s c l e s sh ow ed fasc icu lation s, f ibrillations, andosit ivew av es . T he ce ll count, g lu co se lev e l, and pro te in o f thecerebrospinal f lu id w ere n orm al. W ith th e p reced ing f ind ing sth e d iagnos is o f am yo troph ic lateral scle ro s is w as m ad e .S hortly th e reaf te r, th e p atien t d ied .

Po stm ortem ex am inatio n o f th e lung s show ed sev ere andex ten siv e b ron chopneum on ia. M ild cen triacinar em phy sem aw as no ted in bo th lungs.

T he brain and spin al cord rev ealed no g ross ab norm alitie s .In the sp inal cord , there w as lo ss o f ante rio r h orn cells, w hichw as m os t m ark ed in th e cerv ical segm en ts (F ig 2). T helateral cortico spinal tracts in the sp inal co rd and th e p y ramid sin the m edu lla sh ow ed m ild d e my e l i n a t i o n . A m uscu lar sec -t ion tak en at au topsy rev ealed atro phy w i th f iber angula tion .

D I SCU S S I ON

S egm ental atrophy and w eak n ess in th ex tremi-tie s are the in itial m anif e statio ns o f am y o tro ph iclateral sc lerosis in m o st patien ts , altho ugh 25 per-cen t o f the patien ts w ill h av e bu lbar paraly sis .8 T hepatien ts w ith bu lbar paraly sis w ould be pred isposedto resp iratory com plicatio ns earlier in the cou rse o fth e ir illn e ss becau se o f re sp irato ry trac t in f ection sand repeated asp iratio n . In tho se patien ts w hosein itial sy m ptom is sp inal m uscu lar atro phy , resp ira-tory com plicatio ns u sually occur w ith th e on se t o fbu lbar paraly sis o r in tercostal m uscu lar paraly sis .O nly rare ly has dy spn ea been reported as the in i-tial sy m ptom in patien ts w ith sp in al m uscu lara trophy .5

W e are unaw are o f prev ious reports w ith patho-log ic docum en tatio n o f f ind ings in patien ts w ith

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614 FROMM , W ISDOM , BLOCK CHEST , 71 : 5 , M AY , 1 977

acu te re sp irato ry f ailu re as the f irst m an if e statio n o fam y otroph ic late ral scle rosis . T he tw o patien ts re -p orted here w ere in acu te resp iratory failure andd ep enden t o n m echan ical ventilatory support . A f t e rma n y u n s u c c e s s f u l a t t e mp t s at w ean in g had beenma d e , d i a p h r a g ma t i c p a r a l ys i s w as dem onstrated ,in add ition to c lin ical and e lec trod iagno stic ev i-dence o f sp in al m u scu lar atro ph y . T he c lin icald iagnosis o f am y otroph ic lateral sc le ros is w as con-f irm ed at au topsy by the loss o f an terior horn ce lls ,w h ich w as m ost sev ere in th e cerv ical reg io n .R esp irato ry paraly sis w as attrib u ted to inv o lv em ento f m o tor neuron s innerv ating thei a p h r a g m a n din te rco stal m u sc les.

T h ese patien ts w ere strik in g ly sim ilar in in itialsy m p tom s and f in al c lin ical and patho lo g ic d iag-n osis . W e agree th at b ac terial in f ec tio n , probab lyas a resu lt o f asp iration , acce lerated resp irato rycom prom ise ; h ow ev er, su bsequ en t chest x -ray f ilm sand de term inatio ns o f arte rial b lood gas lev els ineach patien t retu rn ed to nearly n orm al w h ile thepatien ts w ere b ein g v en tilated w ith low F1o2 . D ur-ing postm ortem ex am ination , m in im al ev idence o fchron ic o b s t r u c t i v e pu lm onary d isease w as f ound .B ecause o f the sev erity o f bo th patien ts illnesses,rou tine te stin g o f p u lm onary f unc tio n cou ld no t beperf orm ed . G o ldste in and assoc iates7 hav e sug-gested that m ax im um m id in sp irato ry f low and m ax -im um m idex p iratory f low m ay be used as tests toaid in the d iagno sis o f w eak ness o f resp iratorym usc les. T hese tests m ay be o f v alu e and cou ld b eperf orm ed on patien ts w ith endo tracheal tubes inp l a c e .

In patien t 2 , w e w ere f o rtunate to hav e the re -su lts o f pu lm onary fu nc tion tests p erf orm ed sixm on ths b ef ore adm ission . T hese lim ited stu d ies re -v ealed ev idence o f a restrictiv e v en tilato ry de f ec t.T h e pattern o f th e stu d ies is com patib le w ith m anye tio log ies b u t w as s im ilar to stu d ies reported b yG oldste in and assoc iates7 in a patien t w ith resp ira-tory insu f f ic ien cy as the f irst m anif es tatio n o f aperip heral n eu ropathy w ith inv o lv em ent o f th ephren ic nerve . T he v alues are also com parab le toth ose reported b y M ille r and co-w ork ers5 in threepatien ts w ith ex ertional dy spn ea as th e in itial com -p lain t in p rogress iv e m uscu lar atro phy and am y o -troph ic lateral sc le ro sis and also by M cC red ie andassoc iates8 in a report o f p u lm onary f unc tio n test-in g in patien ts w ith d iaph ragm atic paraly s is . T hepatien t o f G o ldste in e t al7 had rad io graph ic repo rtso f h igh d iaphragm s, probab ly rep resen ting d ia-p hragm atic paraly sis . Pon topp idan and assoc iates9hav e repo rted th at p atien ts receiv ing m echan icalv en tilation dev e lop d iaph ragm atic palsy . T he f lu -o roscop ic patte rns rev eal d iscoo rd ination and no t

the con sisten t parado x ical m ov em ent w e report.D iaphragm atic p araly sis can certain ly lead to a re -strictiv e patte rn on pu lm onary f un ctio n tests andto resp iratory com prom ise , andt w as th e m ajo rcon trib u ting f acto r in bo th o f o ur patien ts de -p endence on m echan ical v en tilatio n .

O u r inab ility to w ean these tw oatien ts f romm echanical v en tilatio n , in the f ace o f w hat ap-p eared to be lim ited in trinsic pu lm onary or card io -v ascu lar d isease , p rom pted our though t that theym igh t h av e a neu rom uscu lar prob lem . D if f icu lty inw ean ing patien ts has been a prob lem since the in -cep tion o f artif icial v en tilatio n and m ay be due toabnorm al pu lm onary m echan ics and b lo od gas ex -change , to low card iac ou tpu t , to a hy perm etabo lics tate , o r to m u scu lar w eak ness.1# { 176} R ecen tly , IM Vhas fac ilitated w eanin g o f p atien ts , bu t ev en th ism e thod w as un succes sfu l in our tw o patien ts .1 Ad isease o f m otor neu ron s, su ch as am y otro ph ic lat-e ral sc leros is , th at lead s to resp iratory m uscu larw eak n ess and d iaphragm atic paraly s is shou ld ,there f ore , b e consid ered w hen resp irato ry supportm us t be con tinu ed fo r un c lear reasons.A C K N OW L EDGM EN T : W e thank M rs . A lice C u llu , M rs.M arjo rie R id er, and M rs . M aryA nn C obb , w ho ed ited an dty p ed our repo rt. D r. W illiam B alling er and D r. R obe rtS ch im p if as s is ted inhe in te rpretation o f the pathologic sp eci-men s .

REFERENCES1 C husid EL , B ry an H : A pp lication o f v en tilato rs in acu te

respiratory fa i lure. M ed C lin North A m 5 7 : 15 5 1 , 1 9 7 32 S h a p i r o AG, W a l k e r C C : R esp iratory in ten siv e care.

M ed C lin N o rth A m 5 5 :1217 -1231 , 19713 Z w illich CW , Pie rson D J, C reag h CE , e tal: C om p licatio n s

of assis ted v entilat ion : A prosp ec tiv e study o f 354on -se cutiv e episod es. A m J M ed 57 :161-170 , 1 9744 O D onohu e W J Jr, B ak er JP, B e ll CM , e t al:espiratory

f ailu re in neurom u scu lar d isease : M anagem en t in arespiratory in ten s ive care u n it. JA M A 235 :733 -735 , 19 76

5 M iller R D , M u lde r DW , Fow ler W S , e t al:xertionald y spnea: A p rim ary comp la in t in unusual cases o f progres-s iv e m uscular atrophy and am y otrophic lateral sc lero sis .A nn In te rn M ed 46 :1 19 -1 25 , 19 57

6 M ulder DW : T he c lin ical sy ndrom e o f am y o troph ic lateralsc lero sis . Pro c S taf f M ee t M ay o C lin 32 :427 -438 , 1957

7 Go ld ste in R L , H y de R W , Lapham L W e t a l : Peripheraln eu ropathy presen ting w ith re spirato ry insuf f iciency as thep rim ary com p lain t: Prob lem o f recogn iz ing alv eo lar hy po -ven t i la t ion due to neurom u scu lar d iso rders . A m J M ed56 :443 -449 , 197 48 M cC red ie M , L ov e joy FW , K altre ide r N L : Pu lm onaryfunc t ion i n diaphragmatic paraly s is . T ho rax 1 7 :213 , 1 962

9 Po n topp idan H , L ay er M B , G e f f in B : A cu te re sp irato ryf ailu re in the su rg ical patien t. A d v S u rg 4 :163 -2 54 , 1970

10 Pon toppid an H , B ushnell L S : R espiratory th erapy forthe con v alesc ing surgical p atien ts w ith ch ron ic lu ng dis -eases, in Holaday D (ed): C lin ical A nesth esia and L ungD isease. Ph ilade lph ia, FA D av is C o , 196 7

1 1 D ow ns JB , K le in EF Jr, D esau te ls D , e t al: In term itten tm an dato ry v en tilation : A n ew approach to w ean in g pa-t i e n t s f r o m me c h a n i c a l v e n t i l a t o r s . Ch e s t 6 4 : 3 3 1 , 1 9 7 3

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DOI 10.1378/chest.71.5.6121977;71; 612-614Chest

G B Fromm, P J Wisdom and A J Blockpatients.

Diaphragmatic paralysis and dependence on mechanical ventilation in twoAmyotrophic lateral sclerosis presenting with respiratory failure.

September 1, 2010This information is current as of

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