p-?LD ACASE Of EXTENSIVE BRAIN DISEASE FROM EN"DARTERITIS -PROBABLY Of SYPHILITIC ORIGIN

,p (l S A'J\•[ U E,_L T. 0 R T 0 N, M.D. WORCES'.l'Bll, i\IASS.

R ep1'iute£l t•·om The Journal of t he American Medical Association October 5, 1912, V.ol. LIX, 1'1'· 1275-1277

C OPYRIGU'l', 1012 A~li~ RICAN MEDICAL ASSOCIATION l•'IYJ-: IlUNDRED AN D TIIIR '£Y·l~ IVE D EARBOHX AVEXUE CHICAGO ~\ OA 'E OF B.'\.'J'ENSl\'B BRAIN DISE_\ SB FRO?\ [ ENDAH'I'EIU'L'I S l'ROBABT_jY OF SYPH ILl'I'J 0 OHJGlN ':'

\VORCF:STEB. J\r AS S.

The .following u1::e off ers evidence o[ some interest in the OCClllTeme of: a diplopia explainable only On the hmctional a ct i vit~· of a Fmall remaining portion of on Yisual fi eld and of the retention of a Yery limited word­ hearing capacity, in spite of tbe destruction of practi­ cally all of the ldt cortical auditory anival-platform and tl1e sunounding and itopsychic skirt. From the history there can be li ttle ll onbt tl1at the concli ·on was luetic and yet the microscopi · examination failed to reveal eYidcnce of the specific nature .

.lfisl01·y.- Tlle patient, a man of :12. with n n egati,·c f am i\ ~ · l1i story RR far ns could be a scerta in ed. was born an1l r a i;;c:l in ]3rookline . :\lass. fli s childhood was llne ven tfnl. lk proYcd to be :t stnde nt of a\·eragc ability, bnt le ft sch ool at t he thi rd gradt> of t he high school. After one ~ ·ca r ''" an !'rrnnd ho_,. in a l:trgc d cpart111e nt .<;lore• he w ent into the lPnlh er llll s in<'ss and ro~c rap i d\~ · lo the pos ition of a ssistant 111>1nnger. _U t he onll1reak of t he i'pnn ish \\'ar he e nlisted " ·ith l he ::\aya\ HPSl'tTe a nd wa s a"igned to duty on the l-. Fi. f'::. ]'rai,.,ie and >c n :eh l:t sling· hYo \\"(•cks, ::mel was under· treatm ent for syphilis for o ne ye:tr. On di scl1a rge fro nt tl1e ""'-~ ' h e was in p oor phys ic:tl healtl1 a nd sho\\·e

• R cacl in the Rt•c li on on i\'r rvou R ancl M ental DiRcaRcs o f t h e Am eri ca n 1\frdical A ~~oc i ntion. nt the Sixty-Third Annual Session, belcl at .I tlnntic City.. Tu1 w . 1012 . • From the Lno ll':ltOI'_\" or Ill(' \\'QI'('CRtrr Rtate Jl ospilal, :uas3U­ chu sc• 1t~. 2

,,.,, , d iag nosed '" a r!t r ttm a t ic di ~l nrua n cr . T\\'o '"''ck,; hi ler lt P had a "shock" \\' ltil c a t hi s p laeP of uus ine,;,; , \\'lti clt was fo ll o\\'cd u.v SO II H' loss of ]10\\'Cr in hoth lei!'· At t lti ,; time it " ·as noted t ha t hi s mt·mo r ~ ' a nd j udg ment lacked t ltrir for mer a cc n nt c ~ · . The shock did not gi,·e ri;;c lo u nconscions­ li <'S ,;, l> nt left a d iplopia w hi ch necPssitatr(l the clos in g of one ,._,.<' to (' Il l out UtP second image. ·La ter he lost t lt e nst• of hi ,; r ig·lt t <'Y'' for a ." lt ort pe riod. Earl_,. in Srptemher he lt ad a g('ncra l Ppil <·plifo rm co nvul sion nml " ·a s sent to t he Ho ~to n 'Cil,v Jlos pita l \\'he re the dial!'nosi,; of ce rcbrospi 11 al sypl ti li ,; \\'as mttrlP and \\'here he rema i11 ed six " ·ecks ll ndcr actin• iod id t reatment. F rom t he middle of October, l !JO.+. to .Jn 11 . l , l!JO;:i. l11· rema in ed at home and durin g t hi s t ime he !tad a genera l con \'ltl s ion, which " ·as Yer.v se,·e rr. lasting th rcl' lt ot ll·,; , a nd wl ti d1 was foll owed b~ · aphasia. l[r det e· r ioratl' N·ame aplta ~ i c a nd fil t h~ · . . Ja n. l. l!JO.i. lt c \\' as co mmitted to the Boston State Hos- pita! wlterc lte rcmn in0d until tra nsfe rred to t his hosp itrtl , :\Iarl'h ~8 , 1!)0.>. , Hxamilla.lio'II.- On ad;n i,-sion he was q uiet aml showed n dement ia-l ik e reaction. 1-fe showed par t ial wonl -deafnes,;. paraphasia. and jargon aplta. ia, woul d not o u e ~ - req ue,;t s, wa,; unaull' t o name olJj <'rb. ex hi bited paralex ia. paragraphia 'and con t inun ll.v c l o~etl hi ,; ll'ft eye in attempt ing to Tead or obsrn ·e objects. ~ ' nolt• at· this t ime sugge,;ts t ha t a hem ianopsi;t was a lready present . On p h ~ · s i ca l exa min;ttion on the da~ - ;t.fter adtnission, t he patie nt " ·as qu iet w it lt expre. s ion less :feat. llr <'~ ; talked frcel.v " ·it lt out slur o r trC'ntor of words, hnt was mar ked ly paraphas ic and appeared demented. ) lan .l' of lltl' test s of spec ial srnses eould 11 0l be a ppl icd on a ccou nt of fa ilure of coopcra t ion. neaction to p in-pri ck " ·as pro 111 pt and equal on t!tr t11·o s ide• . T he knee-jerks wNc exaggerated, especia ll y on t he right . Bauin,;ki was p resent on ri g ht ; no an kl e-clonus. T lt r pupils reactcel'idcnre of a prax ize thP physicia 11 " rodnccJ " <·oherent sent ence in r espoJJ se t o t he imi t a t io 11 of ca b' m ew ­ in g·. !Je " ·a s nnal.J le to na c p id.1 n·c,; w illt one except ion (oog) or geomet ri cal fi g·nres and 11·o nld obe:'' onl y t he s im pl est co mmands. S pon tane0 11 S drawing produced s ~r mb o l.-; of no l ikeness to t he figure r e" ement t o1n trd t he eyes from t he sound side \\'Ot il d oftc 11 provoke a co l> c• rcnt a 11 d f air! ~ · lln c• 11 L )·low o [ p r o fa ni t ~ · . During t he six ~ ·e ; tr s of hi,; >Tsidcncc t il e n ·cord' ~ h ow a t otal of over :3 00 0p ileptifonn c o ll v u l~ i o ll iL . ~ ! a n y o f tilem lm1·c bee n w ell r ecorded and included t oni c spa,; 11 t,; o nl y , as w ell a s toni c spasms of one or more e x t r e m i t~ ' (not con ­ stant in origin ), spreading to ge 11 era I toni c i n1·oll·e ment foll o 11·ecl by clon ic spa sm, f rothi11 g of t he mo11 t h. ll ll con­ sciousness a nd inYoluntary e vacuations. :.Yecropsy.- T I>c a 11 topsy was performed six hours after dPnth . .In t he trun k orgnus t here 11 ·a s ll otecl nutrkcd sc l e r o,; i ~ of t he coron:ur a rter ies, fib rous ad il esions at t he a pex of t he left lung, m oder ca rt , splee n a nd kidneys. On r emoving t he ca lva riu m t he 's- hn tched) . T hi s a lteration " ·as most marked in t he lllidport ion, but i111·o h·ed t he gyrus f rom a point beneath t he a nteri or syll·ian trunk to t he posteri or end of t he main syll·ian fi ssure. inclnding a portion of the suprama rginal gyrn . T here was a su;,;-gcstion of rl'gression below t he general <:o rlica l level w ith some p ial thickening in two a reas of a bout :2 em. in diame t er, one o1·cr t he foo t of t he t hird left f rontal <:o nvolution (Broca's arr;t) a nd t he other at t he foot of the Ji rst left f rontal ( ill ustration, ligh t shading). ] n t he rio or of U1c left Ia tern I n·nt ri cle was a wedge-shaped I a rea of soften i11 g ]. ;) <· m. i11 s ize l ~ · in g in the head of t he caudate nucleus and extending down in to close relation with the a n terior lim b of the in ternal c ap ~ ul e . The ba sil ar a rtery slt o 11·cd nreas of opac i t ~ - and had a t e nd e n e ~ · towH rd irregulYa ri co~ it~ -. T he >1 11 teri or a nd mi dd le cc rebrals and the le ft posterior cerebral " ·<'n' >te m atter. T h('se in tact fibers of t he white co re were, I take it. the fib l' rs of ·commun ica t ion fro m other cortical a reas whose er ll s wc•re un in 1·0 ll·ctl. 5

The cyst of softening showed at its margin t he u sua l close glia mesh\l·ork associated with long standing softened [\ rea;; and a few adjacent vessels showed some perivascula r exudate w ith p igmented phagocytes and a Yery few plasma cells. Lcvaditi stains fo r the StJ'i?·ocltaetc£ pallidc£ in t he la rge ce re­ bral Yc ssels provcu negative. Sections f rom the r ight posterior cerebral a rtery sho\l·ed an interesting condit ion. The much contorted clastic lamina of the orig inal vessel had apparently withstood t he clcgenera­ tiYe changes which had been at work and remained as a loose, ill-fitting jacket ru ound a very small Ye ssel built up w it hin t he lumen of the or igin n. l. In other sections a mn.rked t hicken·

f\IGH T.

Ding·1.·a m ()f the l:llcr:~l aspect of cncb hemi sph crr. So l id bl:1ck . cyst of so ftening: c r os~-h:ltching-. co r tical atrophy with g lia l rcphl CL'­ mcnt; ligltt shading, pial thicken ing. ing o:f t he subintimnl connecti1·e t issue had occ urred g i1·in g· r ise to t hick masses of fibrous t issue rich in fibrob l a~b. nm rkecl ly n arro\\·ing the lumen but giving ll O\\·herc mi croscopil: evidence of a speci fie process. U nfortunatelr n o \Yassermann test was clo ne at an~- stage of the process. The occurrence. ho\\'eYer, of lesion s of t hi s t~ r pc in a nmn of 3~ with a defi ni te hi st01·~· of a recent acti1·c s~r p hil i t i c infection lea.Ye S little roo m fo r doubt that the process was,-., syph ilitic endarterit is. The similarity or the gross :findings and that part of: the microscopic picture pertaining to the imoh-ed areas to the organi c ckmcntiaR opens a qucf'tion of ho 11· great 6 tile et iologic role of S\'phili s ma :v he in tiLis group. Cc l'­ tain it is t ha t in this case in 1rhich s.r philis may be fairly ~ afeh accepted a ~ the caliSc, one co ul d not attempt a diagnosis h om the histologic picture, and it is entirelY concciYablc that many of the organic dcmcntias which arc similarlv negative as regards microscop ic evidence mii!·ht likC\ri!'c be luetic. The refl ex alterations and pares is of t.llC right side arc explained b:r the area of softening in tile left lenticular nucleus anfl internal capsul e, and tile l1emianopsia, on the basis of the loss of: t he greater part of: the right cal­ cai·inc cortex. The occurrence or <:l iplopia, ho 11·cyer, 1rbi ch ncccssitatccl tlJc closing oJ: i-il c left c.rc, indicates the occurrence in co n ~e i ou sncss o C hro 1mJmcd images. The cuneus 'ras the on !.1· remaining pa l't of t il e receptive cortical Yisual field on t he right, an cl thi s mliSt then have been the area of recept ion of the confusing image. The isolatcll obscnation recorded aboYc suggests that the ri ght eye, i. c., the lcCt hemisphere, approached more nearJ.,· a hnc image o [ the ob ject Yiewcd than did the othcl'. On e is tempted to explain the occurrence of the confusin g image by the colllparativcly intact group of n~wciation J-ibers leading from the cuneus to the adjacent posteri or association ficJcl, i. c., parietal cortex, and the diplopia to interference 'ritlt the intCJ·l1cmispheric asso­ eiation fibers crossing in the corpus cnllosmn, many of "·hich see m to haYe been lost. The aphasic disturbance was o C the sensory type and must be explained on the basis of the temporal lobe Jcsions. 'l'hc method of: seri al totnl sections in these cases yields tl1e only accmate localization o£ the lesions, but renders impossible careful histologic study of the nerve­ cells, and in cases studied by this mctl10cl one must dis­ count the possible occurrence of '"ide-spread fo cal or ~tratig rapbi c cell losses in fLelds, '"hich in the total sec­ t ions arc apparently normal, and yet which stand in close Junctional relation to the areas '"hich have been dcstroYccl. In choosin g between these t wo methods one must take into consideration tbc clinical findings, and I feel inclined to resenc the total section proceclm e for those cases sho11'ing the least complication, while apply­ ing the histologic studies to those whose clinical expres­ sion has been complicated, or in 'rhich record is incom­ plete. In the present instance the concunencc of apha- 7 sia. l1cmianops in, pe. i. e., trsnsYerse temporal gn.i and adjacent cortex with the exception of a small portion of the second transnn:e temporsl gnus ncar ·its union "·itlt the conY olntions o[ the insuls, wa~ al so imolvcc1. ::\[i croscopically, this cor­ tex belonged to the bord er-line bctlrcen audito~ensorY and insular trpcs .. This les ion had not r c~ nlt ec1 in complete softening but in an atroplt:· of t he co rtex with g·li al replacement. The white core of the g_ni still containewe must either grant a f unctionsl cspacih to the small practically isolated island of cortex of mixed auditoscnsory sncl insular type, or accept a Yer_,. limited word-hem·ing capacih reaching the cortical centers of the left l1emi sph cre bY way of: the right auditory ani­ Ya 1-platf:onn and co mmiss ursl tracts. The dementia 1ras of comparatiYely sudden derelop­ ment and not of tll c graduallY schsnci.n g tYpe nsualh· following loss o[ the 1rord-hearing cspscity. This cliE­ t nrbancc is better explained perhaps on the tremendous loss in the right hemisphere than on the smaller and probably more slo1dy progressiYc lesion of: the left te_m­ poral lobe. Spitzka bss put forward the suggestwn that the right hcmi3phcrc is the predominant sensory 8 hemisphere as the left is the predominant motor. The left hemisphere in right-handed persons has been accorded the centers for origination of motor-speech impulses and such localization seem s to be definitely proved, but this in no way reacts against the possibility that the right t emporal fi eld may be an important store­ hou ·e of the auditory memories, which probably form in most persons the basis for internal speech.