Beruflich Dokumente
Kultur Dokumente
21Z
Generat
The 57 patients had be~n a d m i t t ~ to UCLA Center for
the .Health Sciences From ~ to 14 times and foilo-~ced for
periods of 1 xvk to I I yr. "Neurolo~ Service consultations
were obtMned for 33 of the patients. ~ e r e were 47 females
and 10 radios: ages at time of onset of SLE ranged from I2
to 59 yr ,(6tean 31.3): and dt~ration of d~sease ranged from
onemo to l g y r (mean 5.7 yr).
The ARA ~creening liniea~ and laboratory findings for
these patients did not differ significantly from those
reported i~ other large series. :~-~-~'~-t~
P A T H O L O G Y tN SLE
From the
UniverM~y of
213
Vasc~foPathv
37 (65%)
25 (44%~
24142%)
16 (28%}
Infatctlon
Hemorrhage
(n~ection
Misc,|raucous
T~ansvetso myelopath
Pogress~vemult~f~-c~t
teukoencepfial~pathy
Hodgkin diseas~
Infarction in CA~5;S L E
As shown in Table 3, the incidence of large
and small multifocal infarctions within the
central nervous system was 30% and 53% in the
1955-1965~and 1966-1977, respeetiv~y. Both
micmlnfarcts and larger infarcts (measuring
more than I cm ~n dimension) increased in inc)denee (p = O:OI). Most microlnfarets (Figs.
7, 8) were ~n the superficial convexity of the
~ r t e x (miliary e~rtica! infaretL and their apparehl increase in incidence du~'ing the latter
period may be a reflection of a more critical
awareness of such pathology and, its identificat)on on gross eam~natmn or a manifestation of
increased microvaseular hypersen~h,;vity due to
changing therapeut~e moda[hies. ~vlicroinfarcts
were seen most commonly in the parietal and
temporal pole convexities, within the pons (Figs.
9, 10) and occasionally elsewhere in the brain
Vascular hyalinixalion
Pefivascular ~nflamr~tion
(without obvious infcc~io.~]
Endothefial proliferation
Thrombosis
Vasc~lit~S(w~thout irt{ection|
~otal I~ases)
1955-~ 965
~23Ca$~)
1966-.1976
i34 Cases)
Total
(57 Cases)
5 (22%}
:16 (76%)
31 (54%)
6 (2:6%)
3 (13%)
I (4%)
2 (9%)
10 (29%)
9 (26%)
3 (9%)
2 (6%1
16 (28%}
12 (21%)
4 (7%}
4 {7%)
14 (5t%1
26 (76%}
37 (65%}
0,01
Mic~oinfacl~
Large infarcts
Total (cases)
~955-T 965
(23 C~sesY
t966~tg76
(3 Cases)
t57 Ca~es)
6 {26%)
2 (9%)
14 (4t%)
S (I 5%}
20 (35%)
7 (12%~
7 {30%)
18 (53%)
25 (44%)
0.01
2 ~4
Fig. 1. Cortica| ~-erlatte~ot~ tymphocytos~s ~-~ 20~yr-old left, ate ~;th 1-y histo~-y of SUE ar~ ~ephfot~ syrct~ome.
H~E~ ~ 35.
F~. 2. En~t.~|ia| pro|~arat~o~ w/th 8ng~omato~ featut'e~ ~n deep su|cat coct~ of fr~ntat h a m ~ h e r e in 51-t-o|d femate
hospita|i2ed frJf g~)stroir)test~na! b~esd~ng and |eft oocip~ta|
hemorrhage. HistoW of SLE. P~ph~t~is.and hypertensior).
H&]E. >~ 60.
Fig. 3~ Acute caPii|ary endothelie| ewe|ling, exudz~ive vascuiopathy, psf~capi|lary hya][~ glot~t~ deposition, and pa~nchyma|
spo~giosis. H&E. 60.
Fig. 4. Necrot~ing vasculitis with thrombus format~n in 36-yr-oid female with SLE. hyr~-.ertenston,afiemia, and termlnai
sLtbaCu~ebee|aliBi e~ocarditis. Miliary mlcroabsc;esses ware four~ in heart a ~ rungs H&E. 60.
Fig. 5. Organizit~g reca~alize4 meningea| artery over temporal p01e iR 34~yr.old female with 7.yi. history of SLE. tupus 91o.
me~u|o,ephritis, arid c|~=icai|y dorumonted CNS vasculitis 3 yr prior to death. Elastic-van Gieson~ x 10.
Fig. 6. Thrornbosed. o~iteratad artery in walt of linear cystic ;nfar~ of external c~s~|e. H& E. 12.
215
Fig. 7. J~+mutecortical necrosis involving depth of sulcus in 64-t-o~d female w i t h 6-y history of SEE treated wPlh daily
predelsone, Teirminal course complicated by metastatic adenocarcinoma of lung. Wail stalo~ ~< 6,
Fig, 8. Chronic subcortical mtcroseople infarcts in sem~t patient aS Fig, 7, Wail stain. X 5.
Fig, 9, Ttansvexse sectioo of pone shgwing t w o cystic infarcts in baslspDntis in 22.yr-old black male w i t h 5-yr history o| SLE,
lupus nephritis, ceeehr|tis, and restrictive lung all)sense. Hyperplastie and filcrinold uascttlopathy was found in pancreas, liver, peripheral nerves, end adrenals. Weft strain, x 4.
Fig. 10. MicrOscopic detail of same patient~as F;g. 9 showing subacute damyetination, meGropheges, end gliosis at border of
microlmfatct+ H~E, x 35.
Su~rachnoid hemorThage"
M~ohemorrhages
~mrace, ebfal hemorrhage
$U~JU~a! P ~ o r r h a g e
Total (cases)
j
- -
=,
t955-~965
|23 Cases)
I966~1976
(34 Cases)
Tofal
157 Cases)
6 {26%)
5 (22%3
2 (9%)
~
11 {32%)
6 (I 8%~
4 (112~
2 (6~
| 7 ~30%J
11 (t9%)
6 (t 0%)
2 {4~
110(43%)
114(4 | %)
24 (42%)
~,
2 16
EI~)S=AND VERITY.
Fig. 11, H e r o , s o m a in patttcenlra) Iotmla el posterior hami~phele ~m'to'anded by Pe|at~hit~l h@motthagas corstaining Candida
sp. in 23-yr-oid lem~le v,~th ?.~r SLE. bdatatal glomerulot~tephtitis, bone marrow hypoolasia, a~d~pane/top~?ia. Other small
hemorrhages are noted in the splenium end 5uperio) parietal Iohule. T0rrninal ;ourse c0mplicated by pnaumon,ia, C a . ~ i a a sepsis.
and CNS involvement,
Fig, 12. Peteehial hemorrhage in ~ttll~orticat wh)te metier with c e n t r ~ "~tart~rst'" effect. H&E. 30.
Fig+ 13. Diffuse spreadingperivenular,hemorrhatlawith transmural taft|trOt+Ors e l inflammatory cells+ H~E+ x 25.
Fig, 14, Resolving sut3dural hemo.haga, H~E. I 0 .
without infection), the incidence of subarachaoid hemorrhage was high (71%) compared to
the incidence of subarachnoid hemorrhage in
tire absence or vascutitis (24%),
lnJi'ction i, C N S SL E
The incidence of CNS infection confirmed at
necropsy increased from 17% to 35% between
1955--1965 and 1966-.1977 (p = O.OY). As
~malyzed in Table 5. this is reflected in the incidence of meningitis, which has increased sixfold.
Aseptic meningitis is a rarely document~ feature o f C N S SLE but may precede and herald a
diagnosis of SLE. ):'- v, Johnson and Richardson ~
Ibund eight instances of |upus cerebritis with
pleocytosis" hut no evidence of bacterial infection. Keeffe et al, ~3 described a case of lupus
meningitis and indicated that, lupus cerebritis
should be included in the differential diagnosis
of meningitis when infectious causes have been
excluded. The dramatic increase in meningitis
seen in our series was not due to an increase in
a s e p i i e " ~ n i n g i t i s (Fig. 15) but' correlated
closely with the increased incidence of bacterial
(~ig. 16) and mycotic infections (Figs. |%=20):
TaMe 6 summarizes the infecting organisms
i,~3Iated from the C N S in patients presenting at
autopsy with meningitis. In n o case were septic
emt~Mi demonstrated within cerebral vessels as
in the case of lupus adocarditis r e ~ r t e d by At-
217
! 966~~976
(34 Coses)
~'ota|
(57 C;~$es)
2 (9%)
9 (26%)
~0 (I 8%)
0~0
2 (9%)
I (4 %,*
1 (4%)
0 (~)
6 (! 7%)
2 (6%)
2 (6%;
2 (6%)
8 (~4%)
3 (5%)
3 ~5%)
2 (3%)
----
12 (35%)
16 (28%)
0.03
~4~(~7%}
Clmicopathologic Correlation in C N S S L E
....Two out of every three patients with SLE
showed clinical evidence of C N S involvement.
Seizures were most common (24%), followed by
severe or persistent headache (23%), hemiparesis (21%), crania| nerve palsy (19%), and psychosis (14%). Multiple" ncurologic s~,mptoms
were seen in two-thirds of those with clinical
findings. Crafiial nerve palsies were most often
as~gcjated with other" findings (81%) and were
o f t e n m u l t i p l e ~, as had been p r e v i o u s l y
reportcd. ~ "~:~In comparing the cases coming to
autopsy from 1955-1965.. with those from
Fig. 15. Deep ~lcal~ eccentr~ ~ r ~ o n u l s r focus of |ymphoc~es consistent with isep1~ meni,gitis~ H& E. 15.
Fig~ 16. Embo;~ abscess in.molecu;at layer of c e r e ~ l | a r foltum with surrounding spongtos|s and g|tosts ir~ 21.yr.o;d female
with I~pus n ~ h r i t i s , chronic gtomeru|itis, u m m i l , s ~ S. aureus h~te~emia~ H & [ . x 25~
Fig, I 7 . N ~ o t l z t n g granu|omat~s mycotic abscess in ceret~tl|at dentate nucteus in e 27*yr-o|d female with lupus nephtltis
end ~ y l 4 ~ r o ~ S receding |ong-tefm Wednlsone I n d ~othiopt|ne thet~y~ R a t e g t l n t e||s are visua|ize(| 8t peripheW of
absces~ E~ colil~tpticemil andpfetermtna| CandidlaslS, H&E. x 10.
Fig~18. D e t s , cd same cese as Fig~ l T showing hyphsl focms of Cer~dlda ttoptcsdis in abscass. H& E. x 65.
218
~955-1965
1966-1976
"'AsepT~"
S. aul,eu~
Candlda sp.
CtVptocorcus
A:;pet g#t,ss
7oxr~plaSrno
2
I
Strep10~ecus sp.
Total cases
r~.~-
219
Maj~rN~u~pa~h~g~Fir~d~sinCNS~LEPatients~ithHemipa~esis*~eizu~es~andC~aniaINe~Pa|sies
Hem~pacesis (~ 2)
S~Jures (I ~)
Cranial nePve
pa~sies (1 I )
To~a! cases (57)
Mt~o ~
~|arl$
Lage
Infarcts
63
33
0
50
27
72
35
12
Subarachnoid
;ntracerebral
Microhemoffhage
Total
V~sc;ubt~s*
~7C a ~ )
No Vascu|~Its
(50 ~ses)
5 (7 | %)
3 (43%)
3 (43%)
~2 (24%)
3 (~
8 (~ 6%)
6 (86%)
18 (36%)
cerebca|
Hemor~hag~
Men~n~
Oi~s
Vascghl~s
50
54
25
18
25
27
18
10
30
18
13
Sg~aachno*d
Hemo~hage
Se;zures
Headache
Hero,paresis
Psychosis
Crania! n e ~ e
patsy
Cerebellsr
signs
Th~s
John~n
and
(57 Ca~s)
{24 Cases)
24
23
21
14
~
~
13
33
22 (7-57)
18 (8~31 )
9 (0-113)
38112-59)
19
42
15 (5-42)
13
5 (5-16)
I.~te~at~e
220
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221
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