vascular myelopathy

13 Pages·2006·2.78 MB·English

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J. Neurol. Neurosurg. Psychiat., 1967, 30, 195 N
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Spinal cord arteriosclerosis and progressive e
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vascular myelopathy su
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KURT JELLINGER ch
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From theNeurologicalInstitute ofUniversityofVienna, Austria try
: firs
Spinal cord arteriosclerosis is considered to be Hughes and Brownell, 1966). Recently we reported t p
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infrequent compared with atheromatosis in other on more than 60 cases, all verified at necropsy, in b
parts ofthe body. Whereas Staemmler (1939) could which a complex neurological syndrome, often lis
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not observe any atheromatous changes in the spinal referabletoacombinedlesionoftheupperandlower e
d
cords of 700 unselected cadavers, Nunes Vicente motor neurone, was associated with generalized a
s
(1964) noted mild arteriosclerosis of major spinal arteriosclerosis and severe aortic atheroma without 1
vessels in 13 out of200 consecutive necropsy cases. thrombosis or occlusion of the spinal tributaries 0.1
Mannen (1963) evenreportedthe incidence of2-6% (JellingerandNeumayer, 1966). Thepathogenesis of 1
3
atheromatous plaques in the anterior spinal artery this rarely diagnosed condition is obscure as, till 6
of300unselected cases upon which necropsies were now, there have been neither sufficient observations /jn
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performedinageriatrichospital. Moderatetosevere regardingtheincidenceofspinalcordatherosclerosis p
atheroma of spinal cord vessels has been observed norrelevant data on thechanges ofspinalvesselsin .30
incidentally but only exceptionally has spinal cord old age, generalized arteriosclerosis, and systemic P.3
si1pn9if3na6ar;lctaiArontnteorbineeies,n(Tc1hai9lu4ls1,e;d19Gb2a3yr;sdtZoekciatu,lmien1n9at5ne3d;dLoHiccochlgtueasnnisotinaennod,f hscylpIeenrrottteihncisscihocnao.nmgmeusniacnadtivaosncutlhaerifnibcriodseinsceinotfhearstpeirniaol- rotecte.195 on
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variant of atheromatosis is also extremely rare Vienna University is reported and correlated with oe
(bAertewnedetn atnhdickWeuninnsgcheorf,th1e954s)m.alTlheintrrealmaetdiuolnlsahriyp taohretaa,gethaencdorthoenafrryeqaunednccyeroefbraatlhearrtoesrcileesr,osaisswoeflltahse pyrigh 1967
vessels in advanced age, known as 'perisclerosis', theincidenceofcerebrovascularlesionsandsystemic t.. D
'hyalinosis', or 'fibrosis' and arteriosclerosis, how- hypertension. Inflammatory vascular processes o
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ever,hasbeenuniformlydenied(LiithyandZollinger, (syphilis,panarteritisnodosa)andtheirconsequences n
1946; Stochdorph and Meessen, 1957). are not included. Table I sets out the age distribu- loa
On the other hand, spinal cord lesions due to tion of the examined cases. In addition, corrobor- de
anritneertieoesnctlehrosciesnthurayve(bDeeemnangken,own188s4i;nceCatmhpebellalt,e vataisvoecisrtcuudliaetsorwyermeyelmoapdaethoyfi7n6andevcarnocpesdy acgaes,esanodf d fro
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1894) but have subsequently received only an in the remaining series of necropsied cases of the h
occasional mention in the literature. Reviewing same ages without vascular disorders of the spinal ttp
mDyaevliospoanth(y193d3u)eftoouvnadscounllary dtiwsoeasceass,esKersecfhernaebrleantdo cord. ://jn
MATERIALANDMETHODS n
arteriosclerosis in 200 instances ofcerebral athero- p
sclerosis. Recent reports, however, suggest that the Fseocrtiotnhseoefxaatmilneaasttiosnixotfo 3th0eblsopcinkaslofcovradrivoeussseslesgmseernitasl .bm
condition may be far more common than was ofthe spinal cord stained with haematoxylin and eosin, j.c
formerly supposed (Jellinger and Neumayer, 1962; cresyl violet or Nissl's method, Van Gieson's elastica o
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TABLE I n
M
AGEDISTRIBUTION OFTHEEXAMINED NECROPSY CASES a
AgeGroup Total rc
h
0-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80 81-90 1
7
200 80 103 103 116 170 180 77 8 1,037 , 2
0
Vascularmyelopathy - - - 3 9 33 28 3 76 23
195 b
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technique, andHeidenhain's or Kluver-Barrera's myelin thickening of the vessel wall with obstruction causing ro
method were available. Selected sections were stained almost complete stenosis ofthe lumen). Similar criteria l N
with Azan or Masson's trichrome technique, Weigert's were used to grade adventitial fibrosis ofthe vessels of e
method forfibrin, P.A.S., toluidin blue, Gomori's silver thespinalleptomeninges. u
impregnation, Bodian's stain for neurofibrils, and the Complete necropsy records were available for 407 ros
cstoauipnleeddwittehtSrauzdoanniuImII,mSeutdhaond-.blaFcrkoBz,enandseSctpiioenlsmeyweerr'es cTahseeysawgeerdeoveexram21inferdomtothaes7s5es7scatsheesiinnctihdeesnecaegeofgraoourptsi.c urg P
myelinstain. atheroma, including aortic thrombosis, and ofcoronary s
The classification ofthe vascular lesions ofthe spinal and cerebral atheromatosis, grading the conditions yc
cord followed the usual pathological criteria recently according to the four degrees ofintensity ofthe coding hia
summarized for the cerebral vessels by Arendt and guideoftheWorldFederationofNeurology(1959),and try
Bhyapcehrmtaronpnhy(1o9f66t)h:e iinntteirmnaall efliabsrtoiscisla(mcionlal,ageandivzeanttiiotni)a,l alelssioonisnanrdeltahteionnectroopstyhefinfdrienqguseonfcysysotfemciecrheybpreorvtaesncsuiloanr : firs
fibrosis, and combined atheromatous changes. The (TableII). t p
intensity of arteriosclerosis of the spinal arteries was u
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(gsrlaidghetd caisrcufloalrlowosr: 0secntoiornmaall,in1t+imalmilfidbroisnivsolvaenmde/notr RESULTS lish
duplication and splitting of the internal elastic fibres Arteriosclerotic changes in the major spinal arteries ed
with or without adventitial fibrosis), 2+ moderate a
were seen in 12-7% of our total material, i.e., in s
sclerosis (same lesions of greater intensity without 22-2% ofthe age group over41 years and in 27-1% 1
much narrowing of the lumen), 3+ severe changes 0
(plaque-like intimal cushions and hypertrophy of the from those over 61 years respectively. Of these, .1
1
internal elastic layer with narrowing of the lumen), however, 10-5% showed only mild intimal fibrosis 3
6
4+ typical atheroma (containing cholesterol crystals and/or hypertrophy of the internal elastic lamina /jn
with stenosis or thrombosis of the vessel). Regarding without much narrowing of the lumen (Fig. la). n
fibrotic thickening of the sulcal and intramedullary Theselesions had always to be clearly distinguished p.3
vessels, five degrees of intensity were considered: from the 'physiological' intimal cushions in spinal 0
0tviessnssoueerlmwwaaillt,lhow1ui+tthnmcaiorlnrddoewncishnaagtnigooefnstoh(feslpilegurhmitevants)hc,iuclk2aer+nicnomgnondoeefcrtaittvheee aarttheerrioesscle(rHaosssilser(,Fig1.96l1b)). wMaosdenroatteed,innon1--8st%e.noOsnilnygProte.3.195
lesions (thickening of the vessel wall up to twice the two cases showed proliferative lesions with obstruc-cte on
na3bo+lremcnaoalnrsricodaewlriibanrbgeleowf(iththiheghlesurlmigedhnet)g,rne4ear+orfoswleeivsneirgoensloefwsiittohnhsec(olenuxsmtierdnee)mr,-e tpoilfoansqeu(veFesirge.(Falitcgh).eralondsd)c,laeinrnoodstiihcseartiintnwmgoaajtnoyrpiinsccpaiildnaaeltnchveeersoosfemlas0t.4oAu%ssd by co 1 June
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FIG. 1. Arteriosclerosis of major h7
spinalarteries. t.. D
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(a)Mildhypertrophyofinternal w
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elasticlamellaandslightthickeningof lo
theintimainanteriorspinalarteryat a
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the T 11 level. NI 131/63. Van e
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Gie(bs)onMoedleasrtaitcaesitnatiinmaxlf4i4b.rosisand from
thickeningofinternalelasticlamella h
withslightnarrowingoflumen ttp
cinerpvoisctaelrolelvaetle.rNalIs4p2i/na6l1aVrtaenryGiaetstohne ://jn
stain x 140. np
(c)Proliferationofsubintimal .b
m
ocbolninteecrtaitvieontiosfspueoswtietrhioprarstpiianlalartery j.co
atmidthoracic kvel. NI154/59. m
Haematoxylinandeosinstain x 140. o/
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(d)Atheromaofanteriorspinal M
arteryatthe T11 levelinprogressive a
myelopathyina66-year-old rc
sclerotic woman. NI49/66. h 1
Haematoxylinandeosinstain x 200. 7
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Spinalcordarteriosclerosis andprogressive vascular myelopathy 197 N
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TABLE II ro
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CORRELATIVE DATAON SPINALAND GENERALIZED ATHEROSCLEROSIS e
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Author Material AnalysisofMaterial s
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KeschnerandDavison(1933) Cer2e0b0raclassecslerosis Spinalsclerosisandmyelopathy............................ 1% rg P
s
Staemmler(1939) Unselectednecropsies Spinalatherosclerosis ......................................0 y
700cases Pialarteryatheroma .............. ......................1-4/ ch
(502agedover41yr.or Intermedullaryarteriolosclerosis, rare ia
Mannen(1963) 2G8e3r3i0aa0tgrceiadcseonsveecrrop6s1ieyrs. VCSSceaelrsveecerburorletoaigvrceanpmselycraeuaqllluoaieprzsaetld(ehasianiettohsenersr.ioo..mr..as..tp..oi..snai..ss..,..a..sr..yt..se..rtye..)m...i..c......h...y...p.e..r...t...e...n...s...i...o...n...........................2.81-9006%%%% try: firs
NunesViscente(1964) Con2s0e0ccuatsievsenecropsies CSeprienbalraslclsecrloesriossi(s11.cas.e.s..+..2..c.a.s.e.s..+..+.)..........................376--55%% t pu
(122agedover41 yr. or Coronarysclerosis.......................................36-0% b
50agedover61 yr.) Aorticatheroma........................................700% lis
Cerebrovascular lesions..................................80% h
Jellinger(1966) Neurologicalnecropsies SSpyisntaelmiscclheryopseirst(me1ni3sl2idoc(na1s.0e.s9.).c..a..s.e..s.)...............................................................................1.21-807515%%% ed as
1,037cases moderate(19cases)...... .................1-83% 1
Cerebralsclerosisse.v.er.e..(.9.c.a.s.es.).......................................................03-9338%% 0.1
Coronarysclerosis.......................................37-2% 1
Aorticatheroma........................................45-2y% 36
CSyesrteebmriocvahsycpuelratrenlseisioonn.s......................................................................78--62%% /jnn
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tthoisthceorargeespgornoduspstoov1e8ra4n1da2n0d%61miyleda,rs3r4esapencdti5ve3ly%, gMearnianternic'scas(e1s9,63,how1e9v6e6r), stehreiesincoifdennceecroopfsiseesverine Prote.3.195
a(mssteoevhdeeeerrrTeoaamtbaaeltt,eohseIaiIrns)o.,dmat0h7ebetiaontngadl2i21n-%5ci%deinnsceetvheeorfeprmseopsdieennartlatsceeroirtedos tanathothatheneerdroisoancmltaeohruioorcsfkiemssnaptiienanrgraielaolf.aatrvthNaeeiroliaaerbstsleteraw,itaailsstawilcastalolhlmodieunawgthhmaaotsoMtnahcniamgnsiheelesndr, cted by c on 1 Jun
corTdhuwsatsheafprperqouxeinmcayteoflyarttewriicoesclaesroshiisghofatsheisnpitnhale osfomettheimeisntearsnsaolciaetlaesdtiwcitfhibdruesp.licSaotmioen acnodmpsaprlaitttiivneg opyrige 196
series ofNunesVicente (1964). As the percentage of figures for the spinal cord and generalized athero- h7
generalized and cerebral atherosclerosis was very sclerosis are given in Table II. t.. D
similar in both series, this difference in part may be Although the presented results are derived from ow
duetodeviationscausedbymethodsofexamination, necropsy materialmainlyfrom cases ofneurological n
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e.g., number of spinal cord sections examined. In disease, good agreement ofour data on generalized a
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Severity of lesions %25 - fro
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15 ttp
FIG. 2. Incidenceofarteriosclerotic ://jn
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changesin majorspinalarteriesin p
10 differentagegroupsof1,037 .bm
unselectednecropsycases. S
subintimalfibrosis. Eelastic j.c
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hypertrophy. A adventitialfibrosis m
5 Ccombinedsclerosis. o/
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S S EA S EAC SEAC SEAC S EAC SEAC 17
3rd 4th 5th Decad6tehs of life7th 8th 9th , 2
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4 S Subintimal fibrosis ro
0/ Severity of lesions E Elastic hypertrophy l N
so O+ El++ *tE4 A Adventitial fibrosis e
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3- C Combined sclerosis s
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S E A C S E A C S E A C e
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Anterior spinal artery Ant. & Post. lateral chains Posterior spinal artery a
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FIG. 3. Localization ofatherosclerotic changesinmajorspinalarteries. 1
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FIG(.a)4.ArtLeerisoisocnlseroofsissmaolflssupkicnoal-cvoemsmseilsss.ural artery in systemic hypertension. NI 4S22-38. Haematoxylin on/
andeosin stain x 280. M
(b) Severe adventitialfibrosis ofsmalldorsalpial vein in sckerotic myelopathy. AH25/64. Van Gieson a
elasticastain x 250. rc
h
(c) Severefibrosisofdorsalfissuralvesselinthecervicalregionwithextremenarrowing oflumen. NI 159/ 1
59. Van Gieson elasticastain x 250. 7
(d)Fibrinoiddegenerationofsmallpialvesselinhypertonicmanaged86withseveremyelopathy. Haema- , 2
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toxylin andeosin stain x 360. 2
3
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Spinalcordarteriosclerosis andprogressive vascular myelopathy 199 N
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and cerebral atherosclerosis with large statistical The comparatively rare atheromatous changes of ro
seriesallowofacorrelationbetweenourfindingsand spinalarteriesareopposedbydiffusemuralthicken- l Ne
tfhroesqeuenicny uonfse3l9e3ctYe%dfonreccreorpesbryalseartihees.rosTclheerosmiseainn ibnrgancohfes,thtehossemailnltheinptorsatmeerdiuolrlaarnyd aanntedriorradsiuclucluasr, uros
our total series, e.g., corresponds well with recent and the pial veins and capillaries, the frequent urg
dataofZschoch(1966)derivedfrommorethan13,000 occurrence and severity of which in advanced age P
consecutive necropsies, indicating an incidence of are well known (Campbell, 1894; Stern, 1936; sy
39.44% for cerebral atheromatosis. Bailey, 1953;Morrison, 1959).These lesions,known ch
sclNerooticreclheavnagnetsicnorsrpeilnaatliaornterwieassanfdoautnhderobmeattwoeseins ulongdiecraltahnedsyhniostnoycmhsemmiecnatlicornietderiaabo(cv.ef., bAyremnodrtphaon-d iatry
osbcfyletrrhaoesnigsrienissgtntoohtfedtvehapereinbodouedsnyt.leoTsnihoaantsgesohpfainstahbleeecmnoarjcdoornaftsihpreimrneoad-l Btthhaiecsehcxmotanradn-inta,inod1n9i6ins6t)ursamumaaelydluyblmleaucrchyahvreamscsoterleesr.ipIzrenodtnhaoesusfnpiicbnreaodlsitcshoarondf : first pu
vesselsaccordingtodecades(Fig. 2). Astothesiteof are identical changes of the small pial and intra- blis
atherosclerotic changes, in agreementwith Mannen cerebral vessels in the senile brain (Stochdorph and h
e
p(1r9e6d6o)m,intahentalnytearfifoerctesdp,inaanldatrhteeriyncwiadsenfceouonfdletsoiobnes sMiedeesrseedna,ss1e9q5u7e;laBeaokfecrhraonnidclrealnanposnien,go1e9d59e)m.aCoorno-f d as
remarkably decreased via the anterior and posterior hypertension secondary to haemodynamic disturb- 1
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lateral chains to the posterior spinal artery (Fig. 3). ances (Zollinger, 1959; Hughes andBrownell, 1966), .1
The clear correlation between the frequency of or occurring as symptomatic vascular changes in 13
vscelsesreoltsiclencdhsansgupepsoratntdotthheescuaglgiebsrteioonfthtahtemeacffheacntie-d doergdeenresraotfivteh,eciennftlraamlmanteorrvyo,usansdystdeemm,yetlhiensaetifnigbrodtiis-c 6/jnn
calfactors ofcirculation are ofsomeimportancefor lesions are not specific for any particular disease. p.3
the pathogenesis of arteriosclerosis (Staemmler, In vessels of the leptomeninges, fibrosis mainly 0
a1n9t3e9r).iorAslptihnaolugahrteraythweerroemaftoouunsd atpltahqeuelsevelofoftthhee ccoonnsdiesntssaotfiovnarioafbltehethsicukrernoiunngdionfgthceonandevecnttiivteiatiwsistuhe Prote.3.195
tsnrlahuootepwuipenosorccrleottefdheroodtitrnhiabecctyihlcveeaMsasacicnenourdnnlvseaiuncropa'fplwseatrrlh(eli1gsl9iu6toa6omnr)btt(aedFhrraieytgc.adwoier3o)adrn.m,eeTttimhhneeiorsrgaeevofneafecrprtaratgolhi-e,es 4c(ioanobfdl)vslpeeriannaagtilreilenetvloieyusassplls,ieenalfatsdilriboanrccognoscsirtafsodso)sirsotmn(oeaaLnrtloaislnociyzsnoa.dm,oepFfsli1ceb9trtr3hiie8ebn;eovdaiGecsdreasalsdeluelu'xglh,weayarna,lGelluirnaaoo(ztfsFzitiiioesg,n'.n cted by copy on 1 June 1
lumen, indicating a higher degree of obstruction in and Gesquier, 1962) was noted in two cases of rig96
the cervical region than elsewhere. hypertension aged over 80 in combination with h7
Atheroma ofthe radicular tributaries seems to be severe generalized atherosclerosis (Fig. 4d). In t.. D
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extremelyraieandwasonlynoted onceinourwhole sulcalbranches andintramedullaryvessels, complete w
series. Occlusive and proliferative changes described acellular and structureless thickening of the walls nlo
insmallpialvesselsoverinfarctedbrainintheborder withoutevidence offibrinoid or hyalin degeneration ad
zonesofarterialsupply (Romanul and Abramovicz, or angionecrosis often does not permit of clear ed
t1i9o6n4)ofwetrheenmeevdeirafionundodrsionltahteersaplinsaplincaolrda.rtCearliceisfiwcaas- aditsitoinnctoifontbheetwveeesnsealrsteriinotloeshaonmdovgeeinnso.usT,ransslfioghrtml-y from
detected in two patients, one ofwhom had suffered eosinophilic and strongly P.A.S.-positive tubes ofa h
from paraplegia with sensory loss below T8 due to bright red when stained by Gieson's method and a ttp
mcayleclaorneeocursosairsachpnroopbaatbhlyy. attributable to severe balcuceomtipnatnwiietdhbAyzacnonadnednsMaatliloonryo'fslmoeostehopderiisvausscuuallalry ://jnn
Arteriosclerotic changes of small intramedullary connective tissue in the perivascular spaces. Severe p.b
vessels are much more infrequent than in the major fibrotic thickening may result in extreme narrowing m
spinal arteries. In our material spinal arteriolo- ofthe lumen, the intima remaining intact (Fig. 4c). j.c
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sclerosis wasjustanexceptional finding inthesulco- Fibrosis ofthe extramedullary vessels favours the m
commissuralarteries (Fig. 4a)andsmallbranches of dorsalpialveinswithincreasingseverityfromcervical o/
tgehneesrpailniazledgreaythmeartotsecrleursousailslyaansdsocisaytsetdemwiicthhsyepveerr-e itontrlaummebdouslalcarraylarnedgiosnulc(aFlig.bra5nac).hesF,ibruossuiasllyofptrhe-e n M
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tension. Contrary to the findings of Arendt and dominating in the dorsal fissure and in the posterior rc
Wunscher(1954) weneverwereable to detect senile andposterolateral columns, revealed a clearcervical h 1
'kongophilic' or'drusige' angiopathy (Schlote, 1965) predilection with an inverse caudal decrease of 7
andgenuinehyalinosis, i.e., hypertensive angiopathy intensity (Fig. 5b). These latterfindingsare opposed , 2
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(AndersandEicke,1940)inthespinalcordsexamined. to theobservationsofLanza(1938)whoemphasized 2
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Severity of lesions Onil 5+ E++ M+,++ *+ ro
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Decades of lifELI nlo
FIG. 5(a). Incidence offibrosisofthepialveinsin 1,000unselectedspinalcords. ad
(b)Incidenceoffibrosisofintramedullary vesselsin1,037unselectednecropsycases. ed
that intramedullary vessels in the lumbosacral cord tension was established, but thickening of intra- from
were favoured, probably related to aorticatheroma. medullary vessels was much more pronounced in h
Though there is a large local and individual varia- arteriosclerotic myelopathies than in other cases of ttp
nbioltietydisnevienrterastmeendouslilnagrylevsaisocnuslianrtfhiebrcoasiusd,alweparrtarseolyf theInstaermeestaigneg.results ofsome pathogenic value were ://jnn
the spinal cord. High degrees of fibrosis of intra- brought about by correlating investigations in 76 p
medullaryarterioles, veins, andcapillaries, however, necropsy cases of 'progressive vasocirculatory .bm
were often encountered inthecervicalintumescence myelopathy of advanced age' (Jellinger and Neu- j.c
and theuppermost thoracic segments. mayer, 1962, 1966)withtherestoftheserieswithout om
VicIenntaecc(o1r9d6a4n)cewewitshawtheanobsaeprpvraotxiiomnasteolfy Nluinneeasr pvaastchuylagrroduips,ordceornssiodfertehde asspinaalclcionridc.o-pTahteholmoygeilcoa-l on/
relationship betweenfibrosis ofthe intra- andextra- entity, comprises aboveall observations ofsubacute M
medullary spinal vessels and advancing age (Fig. or chronic spinal cord ischaemia caused by insuffi- arc
5aandb)althoughthesechanges arenotconsidered ciency ofarterial supply attributable to atheroscler- h
as the normal accompaniment ofaging. A negative osis. Thisgroupofchroniclesionsofthespinalcord 17
association between spinal vascular fibrosis and was formerly known as 'senile paraplegia' (Leyden, , 2
generalized atherosclerosis and systemic hyper- 1892) or 'spastic paraparesis ofthe arteriosclerotic'. 02
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Spinalcordarteriosclerosis andprogressive vascular myelopathyv 201 N
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FIG(.a)6.CysPtriocgrneescsriovseisviansciunltaerrmmeydeilaorpyatghryeydumeatttoerarbteertiwoescelnerCosi6s.and C 7 level. Spongy degeneration of marginal white rotecte.195 on
smtaat(itbn)erxAanr75ed.acptailvleorshoafrppo-sltienreidorcycsotlduumnes.toMoaldninafgaerdct6i6nwiintthersmeevdeiraeryatghrereoyscmlaetrtoesrisaannddpossutbearciuotrecmolyuellmoipnaatthyt.heHeCid5etnohaCin6 d by co 1 June
levelin managed77 withprogressive myatrophies. NI89/66. Kluver-Barrerastain x 8. py 1
(c) Centralspongy necrosisofgreymatterandadjacentpartsofposteriorandposterolateral white columnsatthe T2 rig96
levelinhypertonic managed77withprogressive incomplete transversesyndrome. All4/60. Heidenhain stain x 7. h7
(d) Smallfocalinfarction in centralgreymatterofanteriorhornandintermediaryregionat the T5levelinman aged t.. D
66 withparaplegia offiveyears'durationassociatedwith malignanthypertonia. NI35/57. Heidenhain stain x 20. ow
n
The often ill-defined clinical picture of'arterioscler- gliosis, spongy areas of disintegration or the form- loa
otic myelopathy' is accompanied by a picture ation of cysts with a minor glial reaction (Fig. 6b) de
spismeiuldaor-tmoylaattreofpohrimcsloaftenruacllsecalrermoysaistroofpvhaysc(uslo-acralolreid- -criablepaotrchpyerliavcausncaurlasrtaltaecsuinmaielarintothtehebacsearlebgraanlgleitaa.t d fro
m
gin) or atypical para- or quadriparesis with signs Like these well-known lesions in the arteriosclerotic h
referable to lesions of the upper and lower motor brain, we consider them to be secondary to a rare- ttp
tnreaunrsovneerseosrynodcrcaosmieosna(lKleyscbhynersuabnadcuDtaeviisnocno,mp1l9e3t3e; fbayctcihornonaincdrceolnasteivceutairvteerriealsoripstcihoanemoiaf.tiPsrsueesucmaaubsleyd ://jn
n
JellingerandNeumayer, 1966;HughesandBrownell, lesser degreesofarterial insufficiencythanoperatein p
1966). The chief alterations in the spinal cord are spinal infarction or softenings are responsible, and .bm
found in the grey matter. Their morphological an additional factor of relative tissue sensitivity to j.c
features are often focal, 'rarefaction necrosis', hypoxia becomes important. The dominating factor o
m
waintdhgolinallyoargasnliigzhattitoenndleonccaytedtoiwnartdhse dsepgirnaaldatgiroeny fisicatthieonunnseycrsotseemsa't.izTehdesdeisitrrriebguutliaornlyofplatcheedsele'sriaornes- on/
matter. They prefer the intermediary regions of the grey matter are often small and may even M
corresponding to Rexed's (1964) laminae VII affect one half to one third of one spinal segment. arc
and VIII and extend to the anterior and posterior They may be accompanied by moderate damage to h
horns (Fig. 6a and b). This damage may result in the marginal white matter or by degeneration ofthe 17
simple atrophy of the nerve cell parenchyma posterior columns (Hughes and Brownell, 1966). , 2
accompanied by moderate cellular and fibrillary Occasionallyapencil-likenecrosisofthecentralgrey 02
3
b
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t.
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202 KurtJellinger N
e
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P<001 ro
l N
30 e
u
0Se+ve0ri+tyt+omf+leasi:c[)n+s+ ...."P 0D SIe]v+eriEt2y+o+f 1lesi+onsE++ rosurg
'<0-0005 P
P<00005 P<0.001 P>0-025 FIG. 8. Correla- s
.:. 100_ tion ofincidence yc
h
andseverityof ia
20 80- fivienbstrsroeaslmissedionufl9l6a1ry try: firs
j..j unselectednecropsy t p
60- cases without u
b
ovafstchuelasrpidniaslorders lish
40- cord(A) and76 ed
10p P>0.3 casesofvascular a
myelopathydue s 1
P<0-3 20- to arteriosclerosis 0
(B). .1
1
3
6
0 A B A B A B /jnn
0 Cervical Thoracic Lumnbar p.3
xyz xyz xyz xyz 0
FIG. 7. CorrelLatiSon- ofincEidenceLa-nAd-.severiCty-ofathero- Prote.3.195
scuvcanalssseeecrsluoeltoacirtfceddvciahsnsaoecnrcudgrleeaorsrpssmyooyffcealttshoheeepsastm(phxai)ynj,aold9ru6ce1osprtnidonea(caylr)ro,tapersartyineodrscicae7lss6eersnoinsewicisr1to,h(p0ozs3u)yt7. cted by c on 1 Jun
fSibrsousbiisn.tiCmalcofimbbroisnies.dEscleelraosstiisc.hypertrophy. A adventitial opyrige 196
h7
t.. D
o
w
n
lo
a
d
100 P< 0-2 P<005 ed
P<0.05 SOe1vneirlityEolf+lesio+ns from
80 St+ *++ FIG. 9. Correlationsofincidenceand http
eAortic thrombosis sceevreerbirtoyvaosfcguelnaerralelsiizoends,atahnedrossyclsetreomsiics, ://jn
70j n
hypertension innecropsyseries without p
vascularlesionsofthespinalcord(a-c,) and .b
m
60 P<0.005 airnte7r6iocsacsleesroosfisva(sd)c.ulaaragmeysel0-o8p0atyheyardsueinctlousive j.co
(1,037cases). bages0-40years inclusive m
40 P 0-2_ ((343017ccaasseess)).. cdwwiitthhouvtasvcauslcaurlamryemlyoeplaotphaythy on/
(76 cases). M
a
o a bcd a bcd a bc d a bc d a b c d rch 1
Aortic- Coronary Cerebral Cerebro- Hyper- 7
sclerosis sclerosis sclerosis vascular tension , 2
lesions 0
2
3
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Spinalcordarteriosclerosis andprogressive vascular myelopathy 203 e
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rmeagtitoenrisinfotuhnedl(oFwieg.r 6cce),rviwchaelreaansdinuprpareeritnhsotraancceisc rtieopnosrtoedf avnariinocuisdeonrciegionfs2a%ndfoorfsp9i%nalfcoorrdceirnefbarroc-- rol N
e
small focal infarctions ofvariable localization were vascular accidents in 200 unselected necropsies. uro
seen (Fig. 6d). Mannen (1966) noted similar lesions On the other hand, Blackwood (1958) found no s
u
in six of25 cases ofarteriosclerotic myelopathy, the examples of arterial softening of the cord in 3,737 rg
clinical manifestations ofwhich, however, were not post-mortem examinations from 1909 to 1958 at the P
always definite. Myelopathies attributable with NationalHospital,QueenSquare,London.According sy
c
certaintytoseverebonyandcartilaginousdeformities to our personal experience in a series ofabout 200 h
iinncSltcauetdrievsditciiacnallthseipvsoanlsdeuryailteoiss.oinsof(Hbuogthhesg,rou1p9s66)shoawreednnoot ibdniessitonargndceerrssefeiornfabalsepwiniatdloercvosaersndcsuel,daaromnalgayend,t/wocrooncscaiisdreecsruel(ad1to%ar)ys iatry: firs
relevant differences in the frequency and severity of were due to infarctions resulting from local ather- t p
intimalfibrosisandhypertrophyoftheelasticaofthe oma in major spinal arteries. For comparison, u
b
major spinal arteries. In the myelopathy group, 3.5%ofmyelonecroseswerecausedbyaorticlesions, lis
however, there was a significant preponderance of whereas 2-5% of cases of spinal cord damag_ h
e
adventitial fibrosis, which is not regarded as a occurred in combination with panarteritis nodosa. d
a
sclerotic change, and a highlysignificant occurrence Considering the percentage of moderate to severe s
of atherosclerosis in the spinal arteries (Fig. 7). A spinal cord atherosclerosis in our total material it 10
highly significant positive association between can be suggested that only about 5% to 10% of .1
1
myelopathy and fibrosis ofthe small intramedullary atheromas in major spinal arteries may result in 3
6
vessels was established, its significance decreasing typicalspinal cord infarcts. This confirms theminor /jn
from cervical to caudal cord levels (Fig. 8). importance of local atherosclerosis for the patho- n
p
A statistical comparison of generalized athero- genesisofacutevasculardisordersofthespinalcord. .3
sclerosis revealed a significant preponderance of Ourcorrelative statistical investigations, however, P0.3
pcoartohnyagrryouapnadncdeirtesbhriaglhlaythsiegrnoimfaictaonstisassioncitahteiomnyewliot-h airntdeirciaotseclearoscisertaasin asicgnoinftirciabnucteoryforfacstpoirnalincotrhde rote.195
necropsy findings of systemic hypertension. There comparatively frequent manifestation ofcirculatory cte on
awvatashsceuorlnoalmryaleasaisnoldnisghiatnorittrhrieeclmeytvehalrnotopmpabrtoeshpiyosgndraeonrudapnacosefcoocfmepraeaobrrrteoid-c mvdaaitssoocsruidlsea,rrsdsyoysfsftutenhmceitcisopnihsny.apleTrhctioesrndssieoiennm,sgeontrehreaomltiohzreered iacmtaphroedrritoo--- d by c 1 Jun
oe
with the large series without vascular disorders of ant because chronic spinal cord ischaemia due to py 1
the spinal cord (Fig. 9). atherosclerosis seems to be far more common than rig96
DISCUSSION rweafserafbolremertloy asrutgegreisotsecdl.eroPsriosgrersespirveesemnyteedlopaaltmhioesst ht.7. Do
one-third ofourtotalnecropsymaterial ofprobable w
n
Thereportedfindingsprovetheinfrequent incidence vasocirculatory disorder of the spinal cord, and lo
of arteriosclerosis in major spinal arteries with the about one-fifth of Hughes' relevant cases (1966). ad
extremely rare occurrence of moderate to severe Contrary to rare spinal cord infarctions resulting ed
atheroma, the incidence of which in the examined from documented interference with spinal blood fro
series represents approximately 5% of its mean flow dueto occlusion ofspinaltributaries, including m
frequencyincerebralarteries. Uptonow,nostatisti- the aorta and its segmental branches (Gruner and h
cal observations exist regarding the incidence of Lapresle, 1962; Hughes, 1966; Jellinger, 1966), the ttp
vascular disorders of the spinal cord except some siteofthenon-systemicischaemiclesionsinvascular ://jn
correlative data with cerebrovascular lesions. In the myelopathy shows neither a relevant relationship n
p
material of KeschnerandDavison (1933)therewere to the segmentary pattern ofspinal vascular supply .b
twoscleroticmyelopathiesoutof200casesofcerebral nor to the organic impairment of the major spinal m
arteriosclerosis, indicating an incidence of 1% for arteries (Jellinger and Neumayer, 1962, 1966; j.c
o
spinal cord damage due to arteriosclerosis. In Mannen, 1933). This indicates that the underlying m
Mannen's (1966) geriatric material the incidence of disorders are predominantly located in the extra- o/
cerebrovascular syndromes was 80%comparedwith medullary feedingsystem ofthe spinalcord. n M
one of 9% for vascular myelopathies with multiple The obvious cause of the spinal cord damage a
small ischaemic softenings comparable with the thought to be due to chronic diminution in its total rc
'rarefaction necroses' mentioned above. Mannen, bloodsupply is the severearteriosclerosis present in h 1
however, observed no spinal cord infarction caused every case, and particularly evident as severe 7
byocclusion ofmajorspinal tributaries ordissecting atheromaoreventhrombosisoftheabdominalaorta , 2
0
aneurysm of the aorta. Nunes Vicente (1964) from which the blood supply of the caudal part of 2
3
b
y
g
u
e
s
t.
J
N
204 KurtJellinger e
u
ro
the spinal cord arises (Gruner and Lapresle, 1962; comparatively rare in our series. In some cases this l N
HughesandBrownell, 1966). Dissectionoftheinter- might be due to a special pattern ofvascularization e
u
costal arteries and the lumbar spinal branches of the caudal parts of the spinal cord providing ro
usually failed to demonstrate any thrombosis or collateralsupplyviaaccessorylowerlumbarbranches s
u
occlusion but frequently themouths ofthesevessels (Lazorthes, Gouaze, Bastide, Sontoul, Zadeh, and rg
were buried in plaques of calcified or ulcerating Santini, 1966). Recently Garland, Breenberg, and P
s
atheroma. As the segmentary arteries do not form Harriman (1966), in a case of spinal cord malacia y
c
ananglewiththeaortabutanelbow-likearchwhich withischaemiclesionsatvariouslevelsofthelumbo- h
ia
is almost 900, partial blocking oftheir orifices may sacral cord, found stenosis of the anterior spinal try
larotweerrialcobnesdi.deTrhaubslyatnheinbtelroroudptpiroenssourreseivnertehedismpiinnua-l aornteeryaetaitoloongeicallumfbaacrtorlevoefl iwnhsiucffhicwiaenscydiosfcuasrsteedriaasl : firs
tion oftheblood supply tothespinalcordmightbe supply. t p
caused without any occlusion of spinal tributaries The predilection forchronic ischaemic damage to ub
(Gonzalo-Sanz,1962).Nothing,however,isknownso beinthecervical cordindicates thatthemostsevere lis
h
far of the 'critical' minimum blood pressure in the decrease in blood flow occurred in the spinal e
d
capillarybedofthehumanspinalcord, whereblood territory where the vertebral arteries are the source a
flow reaches a critical level, that is to say, when the of supply. This appears the more surprising as the s
1
average 02 uptake begins to decrease and severe anatomical pattern ofarterial supply to thecervical 0
neurological disturbances occur. Whereasadecrease cord is thought toprovidealmostperfect protection .1
1
ofarterial blood pressure in the canine aorta below of this part from the complications of athero- 3
6
15 mm.Hg for one hour causes ischaemic lesions in sclerosis (Turnbull, Breig, and Hassler, 1966). On /jn
the spinal cord (Blaisdell and Cooley, 1962), exten- the other hand, it is well known that occlusion, n
p
sive mobilization of the canine aorta from the stenosis, and other impairments of the vertebral .3
0
posterior parietes was not usually associated with arteries are accompanied by cervical infarction onP.3
pbleoromdanpernetssnuerueroilnotghiecailntseyrcmopsttaolmasrtwehrieenstdhiedpnaortieftaalll maryteelrioepsathbyei(nHgughefrse,q1u9e6n6t)l.yInasfpfietceteodfthbeyversteevberralerote.195
below 30 mm.Hg (Killen and Adkins, 1965). These atherosclerosis (Hutchinson and Yates, 1956;cte on
epsaxirdpaeetrrieivdmeelntytoarlabpeicdornefdlalielvtaiinontnsblfooarosdstohcpeiraetsceshdurroenwiiactrheannaodtcscolomon--w cFvriaisrthiieocrau,lspGrooesrdieut,icotniOsoknaobfeit,nheantnheedcikrW(hCbihltroeao,sdt1a9fn6ld5o)wKoardnbudiecktaht,oed by co 1 June
loweringofthespinalcordbloodflowinmandueto 1962), the causes ofthe preferential damage to thepy 1
arteriosclerosis and general circulatory disorders. spinal territory of supply of the vertebral arteriesrig96
h7
AlthoughtheexperimentaldataofOtomo,Wolbarsht, in chronic myelopathy associated with arterio-t.. D
Van Buskirk, and Davidson (1960), Bartsch and sclerosis is not yet elucidated since we lack detailed o
Swank(1967),andCzanaky(1967)lendsupporttothe angiographicalandmorphologicaldataregardingthe wn
suggestion thatspinalcordbloodflowsimilartothe extracranial part of this artery. Whereas cases with lo
a
regulation of brain circulation chiefly depends on severe cervical spondylosis as one possible cause of d
e
thesystemicbloodpressure,verylittleisknownofthe myelopathywerenotincludedintheseriesexamined, d
autoregulation ofspinal cord circulation. Therefore moderatebonyandcartilaginous deformation ofthe fro
anypathogenicinterpretationofspinalcorddamage cervicalspinewerenotedin 12%ofthis myelopathy m
in association with atherosclerosis up to the present group. They should therefore be taken into account h
restsonthepathologicalandmorphologicalfindings asacontributoryfactortospinalcorddamagedueto ttp
in the spinal cordand its feeding system. direct mechanical impairment or vasocirculatory ://jn
Athoroughevaluationofthesiteanddistribution disorders (c.f. Taylor, 1964; Hughes, 1966; Breig, n
p
of chronic ischaemic spinal cord lesions in 60 nec- Turnbull, and Hassler, 1966). Whether there is a .b
m
ropsy cases of progressive myelopathy referable to local correlation of spinal cord lesions with the
arteriosclerosis revealed a most striking preference cervicalpreference ofatherosclerosis ofthe anterior j.co
for damage to the cervical intumescence and the spinal arteryandfibrosisofthesmallintramedullary m
upper thoracic region between C 5-6 and T 2 vessels needs further discussion. o/
n
(Jellinger, 1966, 1967). This corresponds well with Fibroticthickening andstenosis ofintramedullary M
thefindingsofMannen(1966)whonotedafavoured vessels,which is regarded as secondaryto a diminu- a
site for small softenings in sclerotic myelopathy at tionofthetotalbloodsupply,isprobablycomparable rc
h
thelevelofthecervicalcord, especiallyinC5toC8. to'adaptationsclerosis'(Staemmler, 1939;Zollinger, 1
Ianthsepriotmeaofansdevseoremetaoirmteisc lsoclweerrosiasortwiicththurlocmebroastiisn,g v1a9s6c7u)laarndreistisseltfanccaeusienstgheenesrpailnaalndcorlodc.alThienrcerefaosreeoift 7, 20
ischaemic damage to the thoraco-lumbar cord was results in considerable reduction of the regional 23
b
y
g
u
e
s
t.