CYSTICERCOSIS OF THE NERVOUS SYSTEM: LESS FREQUENT
CLINICAL FORMS
I I I — S P I N A L C O R D F O R M S
H O R A C I O M . C A N E L A S * O S W A L D O R I C C I A R D I - C R U Z * * O V I D I O A. D. E S C A L A N T E * * *
T h e spinal forms of t h e cysticercosis of the nervous s y s t e m a r e r a t h e r
rare. I t must be emphasized, h o w e v e r , as H e n n e b e r g9
a l r e a d y did, t h a t
the incidence of these f o r m s can not be precisely e v a l u a t e d since t h e
intra-spinal structures a r e not s y s t e m a t i c a l l y e x a m i n e d in the necropsies; besides, t h e spinal s y m p t o m a t o l o g y f r e q u e n t l y is o v e r l o o k e d or e v e n masked by
the outstanding and s e v e r e c e r e b r a l manifestations.
R o c c a1 6
, w h o has the g r e a t e r series of cases in this field, r e m a r k s t h a t
the spinal cord f o r m s a r e four times r a r e r than the c e r e b r a l ones, this p r o -portion b e i n g much a l i k e t h a t of neoplasms. T h i s finding, w h i c h concerns
the cysticercosis of the nervous s y s t e m in a g e n e r a l w a y , could be e x p l a i n e d b y t h e d i f f e r e n c e of nervous mass inside the skull and the spine. H a r d e r t o explain is the g r e a t disparity b e t w e e n the p a r e n c h y m a t o u s cysticercosis
in the brain and the spinal c o r d ; actually, in a survey of the l i t e r a t u r e w e found only 7 cases of i n t r a m e d u l l a r y cysts, w h i l e t h e e x t r a m e d u l l a r y l o
-calization w a s r e p o r t e d in 35 cases ( t a b l e 1 ) .
T h e p a r a s i t e could f o l l o w t w o main routes in his w a y t o the intraspinal s p a c e : the subarachnoidal m i g r a t i o n , by passive t r a n s p o r t in the c e r e b r o -spinal fluid current, or the h e m a t o g e n i c r o u t e . T h e first p a t h w a y , w h i c h
w o u l d i m p l y in the secondary f e a t u r e of the spinal localization, could ac-count f o r t h e p e r i m e d u l l a r y m e n i n g e a l f o r m s , but only the b l o o d r o u t e could a p p a r e n t l y explain the p r i m a r y i n t r a m e d u l l a r y l o c a l i z a t i o n of the cysticercus.
T h e e x t r e m e rareness of such site l e d R o c c a1 6
to suggest t h a t the spinal cord is e n d o w e d w i t h a p a r t i c u l a r a b i l i t y t o f i g h t t h e c y s t i c e r c o t i c infestation.
W e suggest a third r o u t e f o r t h e approach of the p a r a s i t e to the spinal cord p a r e n c h y m a : the e p e n d y m a l r o u t e . T h e v i a b i l i t y of the c e n t r a l canal
is a n a t o m i c a l l y d e m o n s t r a t e d and one can accept that, in cases of intra-v e n t r i c u l a r hypertension, this canal w o u l d dilate, a l l o w i n g the caudal dis-p l a c e m e n t of c y s t i c e r c i a r r i v e d at the fourth v e n t r i c l e . T h i s hydis-pothesis could explain t h e finding of parasites l o d g e d in t h e upper c e r v i c a l cord tissue.
O n t h e other hand, the i n v o l v e m e n t of both the spinal cord and the m e n i n g e s could result f r o m an a l l e r g i c r e a c t i o n t o the toxins f r e e d b y
cys-ticerci l o d g e d in the intracranial structures. T h i s pathogenic mechanism w a s i n v o k e d b y V e r g a & D a z z i2 1
in an a t t e m p t t o explain, conversely, the c e r e b r a l m e n i n g i t i s found in a case of r a c e m o s e spinal cysticercus.
Spinal cord manifestations associated w i t h basilar cysticercosis had been a l r e a d y r e c o r d e d in 1905, by W o l l e n b e r g2 2
, w h o , in t w o autopsy cases, found parasites in the dorsal roots and c y s t i c e r c o t i c m e m b r a n e s in t h e filum
t e r m í n a l e . A s u r v e y of the l i t e r a t u r e ( t a b l e 1 ) shows, as one w o u l d expect, that the diffuse cerebrospinal i n v o l v e m e n t s p r e v a i l o v e r t h e e x c l u s i v e l y spinal f o r m s .
W a l t o n (apud G u c c i o n e7
) s e e m i n g l y w a s the first t o report, in 1881, a case of spinal cysticercosis in w h i c h t h e p a r a s i t e w a s localized in the a n t e r i o r horn of t h e c e r v i c a l cord. H i r t (apud G u c c i o n e7
) r e p o r t e d a case
w i t h tabes-like s y m p t o m a t o l o g y , the post m o r t e m e x a m i n a t i o n h a v i n g dis-closed s e v e r a l cysts in the cauda equina. M i n o r (apud G u c c i o n e7
) , in 1899, r e p o r t e d t h e first case of spinal cord compression by a cysticercus placed under the pia m a t e r of the thoracic cord.
T r é t i a k o f f & P a c h e c o e S i l v a 1 9
and B r i n c k3
described t h r e e syndromes
produced by spinal c y s t i c e r c o s i s : m e n i n g o m y e l i t i s , compression, and t a b e t i -f o r m pictures. T h e s y n d r o m e o-f p a r e n c h y m a t o u s lesion is an additional picture, displaying e i t h e r a p o l i o m y e l i c o r a funicular p a t t e r n .
T h e meningospinal v a r i e t i e s c o m m o n l y p r e v a i l in the posterior aspect and in the c e r v i c o t h o r a c i c s e g m e n t s ( S c h m i t e1 8
) , s o m e t i m e s g i v i n g w a y to p a c h y m e n i n g i t i d e s o r m e n i n g o r a d i c u l i t i d e s close to the t a b e t i c picture. T h e
e x t r a d u r a l l o c a l i z a t i o n is e x c e p t i o n a l ( S e h m a n s ' case, cited by G u c c i o n e7
, and R o c c a ' s 1 6
) .
T h e case r e p o r t e d b y M e y e r1 1
is w o r t h m e n t i o n i n g : his patient had cysticercosis w i t h c e r e b r a l s y m p t o m s associated w i t h a s y n d r o m e of l a t e r a l
a m y o t r o p h i c sclerosis, w h i c h c u r r e n t l y w o u l d be i n t e r p r e t e d as a s y n d r o m e of the a n t e r i o r spinal a r t e r y due t o the c y s t i c e r c o t i c p e r i a r t e r i t i s seen in the post m o r t e m e x a m i n a t i o n .
M o n t e i r o - S a l l e s 1 2
T h e v a r i e t y displayed by t h e p a r a s i t e is seldom r e f e r r e d . R a c e m o s e
f o r m s w e r e r e p o r t e d b y H i r t and R i c h t e r ( a p u d G u c c i o n e7
) , K n a p p1 0
,
H e n n e b e r g 9
, Busse 4
, V e r g a & D a z z i2 1
, R i c c i a r d i - C r u z 1 5
, and Cabieses et al.5
A m o n g 296 cases of neurocysticercosis o b s e r v e d in the D e p a r t m e n t of
N e u r o l o g y of the U n i v e r s i t y of S ã o P a u l o M e d i c a l School f r o m F e b r u a r y
1945 t o D e c e m b e r 1962 w e have found 8 patients w i t h spinal cord i n v o l v e
-ment, either isolated ( 7 cases) o r associated w i t h c e r e b r a l s y m p t o m s (case 3 ) ; t o these cases w e added one of p r i v a t e clinic ( c a s e 9 ) . T h e scarcity
of this clinical f o r m of neurocysticercosis, t h e possible diagnostic m i s t a k e
w i t h o t h e r i n f l a m m a t o r y or t u m o r a l conditions, and the good results some-t i m e s achieved w i some-t h c o n s e r v a some-t i v e o r surgical some-t h e r a p y seemed some-to us sufficiensome-t
reasons f o r t h e publication of t h e present paper.
T h e identification of our cases and l a b o r a t o r i a l data for t h e e t i o l o g i c
diagnosis are seen in t a b l e 2; the n e u r o l o g i c a l s y m p t o m a t o l o g y , t o p o g r a p h i c
diagnosis, and therapeutic results a r e seen in table 3.
C O M M E N T S
T h e e x c l u s i v e l y spinal cord f o r m s of neurocysticercosis a r e r a t h e r rare.
In a survey of the l i t e r a t u r e w e found r e f e r e n c e t o 42 cases only. A m o n g 296 cases of this parasitosis of t h e nervous s y s t e m e x a m i n e d in our S e r v i c e the incidence of such clinical f o r m w a s 2.7%.
I n spite of this r a r i t y , the c y s t i c e r c o t i c e t i o l o g y must a l w a y s be k e p t
in m i n d of the especialist in cases of subacute or chronic i n v o l v e m e n t of the
spinal cord, m o r e o v e r t a k i n g into account t h a t the s y m p t o m a t o l o g y of this
spinal parasitosis is polymorphous, simulating m e n i n g o m y e l i t i d e s , tabes
dor-salis o r radiculospinal compressions.
T h e e t i o l o g i c diagnosis w a s based, in 8 of our 9 cases, on the positive
result of t h e c o m p l e m e n t f i x a t i o n test f o r cysticercosis in t h e cerebrospinal fluid, s o m e t i m e s r a t i f i e d b y t h e finding of eosinophile cells in this fluid,
b y the p o s i t i v e result of the same r e a c t i o n in blood serum, a n d / o r the
d e m o n s t r a t i o n of intracranial m i c r o n o d u l a r calcifications. I n case 9, h o w
-ever, the c o m p l e m e n t f i x a t i o n test in t h e cerebrospinal fluid w a s n e g a t i v e and the diagnosis w a s not established but in the o p e r a t i n g r o o m , t h r o u g h
the r e m o t i o n of a subdural m a c r o c y s t i c cysticercus at t h e l e v e l of the first
l u m b a r v e r t e b r a . T h e p a r a s i t e w a s s u r g i c a l l y r e m o v e d in 2 additional cases.
I n cases 1, 2 and 8 simultaneous spinal and cisternal punctures w e r e
p e r f o r m e d . I n case 1 g r e a t e r pleocytosis w a s found in the spinal than in
t h e cisternal fluid; h o w e v e r , in this l a t t e r t h e eosinophile cell count g a v e
higher figures. I n cases 2 and 8 t h e eosinophile cell count and the total
c y t o m e t r y p r e v a i l e d in the sample w i t h d r a w n b y spinal p u n c t u r e ; these
facts suggest that, although g e n e r a l i z e d , t h e m e n i n g e a l r e a c t i o n to the
w a s found in all these cases but o n e ) . M y e l o g r a m , p e r f o r m e d in all these 5 cases, c o n f i r m e d the b l o c k ( f i g . 1 ) . T h e s e data supported t h e indication f o r l a m i n e c t o m y in cases 1*, 7* and 9, a l l o w i n g t h e r e m o t i o n of cysticerci l o d g e d in the subdural space ( f i g . 2 ) . T h e y assumed the racemosus t y p e
in t h e first 2 p a t i e n t s and the cellulosae v a r i e t y in case 9. S u r g i c a l results w e r e e x c e l l e n t in case 9, in case 1 the conditions r e m a i n e d unchanged, w h e r e a s in case 7 g o o d results w e r e a f f o r d e d b y subsequent intrathecal a d m i n i s t r a t i o n of h y d r o c o r t i s o n e .
T h e p r e v a i l i n g neurological picture w a s that of spinal cord a n d / o r
n e r v e r o o t compression ( 4 c a s e s ) . I n 2 patients a s y n d r o m e of the dorsal funiculi w a s present, closely s i m u l a t i n g tabes dorsalis; this impression w a s m a d e stronger, in case 5, b y the finding of A r g y l l - R o b e r t s o n ' s sign. I n o t h e r 2 cases the n e u r o l o g i c a l manifestations suggested m e n i n g e a l and spinal i n v o l v e m e n t w i t h no s y s t e m a t i z a t i o n .
I n t w o patients (cases 4 and 7 ) a l e v e l of touch, pain and t e m p e r a t u r e anesthesia could be d e t e r m i n e d , in this w a y r a t i f y i n g t h e spinal t o p o g r a p h y of t h e process. I n case 9 t h e r e w a s sensory i m p a i r m e n t in the area of the f i r s t sacral r o o t . I n case 7 the c y s t o m e t r i c e x a m i n a t i o n showed i m p a i r -m e n t of the b l a d d e r sensation.
I n case 3 t h e spinal s y m p t o m a t o l o g y pointed to a lesion in the l a t e r a l and dorsal funiculi; the association of achlorhydria could m a k e the c y s t i c e r -cotic e t i o l o g y questionable, if it w e r e not the f o l l o w i n g f a c t s : ( a ) this w a s
t h e o n l y case in w h i c h s y m p t o m s s u g g e s t i v e of c e r e b r a l cysticercosis ( h e a d -ache, v o m i t i n g , s e i z u r e s ) w e r e associated, the skull r o e n t g e n o g r a m s h o w i n g m i c r o n o d u l a r calcifications; ( b ) the d e g r e e and the p a t t e r n of the c e r e b r o -spinal fluid changes, pointing t o a chronic parasitic meningitis, as the c o m p l e m e n t f i x a t i o n test f o r cysticercosis did indicate ( s t r o n g l y positive
w i t h 0.1 m l . ) .
S U M M A R Y
T h e spinal f o r m s of cysticercosis a r e r a t h e r r a r e ( 2 . 7 % of 296 cases of neurocysticercosis r e c o r d e d in the D e p a r t m e n t of N e u r o l o g y of the U n i v e r s i t y
of S ã o P a u l o M e d i c a l S c h o o l ) . I n a s u r v e y of the l i t e r a t u r e only 42 cases w e r e found, m o s t of t h e m associated w i t h cerebral s y m p t o m s . T h e reasons f o r this l o w incidence, as w e l l as the possible routes f o l l o w e d b y the parasite in its approach t o the spinal cord, a r e discussed.
A f t e r a r e v i e w of the first cases r e p o r t e d in t h e l i t e r a t u r e , the authors
r e f e r the m a i n syndromes ( m e n i n g o m y e l i t i d e s , t a b e t i f o r m pictures and spinal c o r d compressions) and some of the clinico-pathologic features of spinal
cysticercosis.
N i n e cases of spinal cysticercosis a r e r e p o r t e d . T h e diagnosis w a s based on l a b o r a t o r i a l data ( m a i n l y the c o m p l e m e n t f i x a t i o n test f o r cysticercosis
* Cases a l r e a d y p u b l i s h e d b y o n e o f u s1 5
in the cerebrospinal f l u i d ) o r in t h e results of s u r g i c a l therapy. O t h e r cerebrospinal fluid findings ( p r e s e n c e of eosinophile cells, protein contents, and t h e results of t h e m a n o m e t r i c t e s t s ) a r e discussed. M y e l o g r a p h i c block w a s d e m o n s t r a t e d in 5 cases. T h r e e of these patients w e r e s u b m i t t e d to l a m i n e c t o m y , w i t h v a r i a b l e results.
T h e p r e v a i l i n g n e u r o l o g i c a l p i c t u r e w a s t h a t of spinal cord a n d / o r root compression ( 4 c a s e s ) . T w o patients s h o w e d a dorsal funiculi s y n d r o m e
closely simulating tabes dorsalis. T w o other patients presented a picture of m e n i n g o m y e l i t i s w i t h no s y s t e m a t i z a t i o n . O n e patient had a s y n d r o m e s u g g e s t i v e of subacute combined d e g e n e r a t i o n of t h e spinal cord, but the presence of cerebral s y m p t o m s and the l a b o r a t o r i a l data pointed t o cysti-cercosis as the m a i n disease process.
R E S U M O
A s f o r m a s medulares da neurocisticercose são r e l a t i v a m e n t e r a r a s (2,7% de 296 casos desta parasitose r e g i s t r a d o s na Clínica N e u r o l ó g i c a da F a c u l
-dade de M e d i c i n a da U S P ) . E m r e v i s ã o da l i t e r a t u r a só e n c o n t r a m o s 42 casos, na m a i o r i a dêles o c o r r e n d o associação c o m sintomas encefálicos. S ã o discutidas as razões desta baixa incidência, assim c o m o as possíveis vias seguidas p e l o p a r a s i t o a f i m de a l c a n ç a r a medula.
A p ó s r e v e r e m os p r i m e i r o s casos r e g i s t r a d o s na l i t e r a t u r a , os autores d e s t a c a m as principais síndromes ( m e n i n g o m i e l í t i c a , t a b e t i f o r m e e d e c o m -pressão m e d u l a r ) e alguns dos c a r a c t e r e s clínicos e a n á t o m o - p a t o l ó g i c o s da cisticercose medular.
Sã o relatados 9 casos de cisticercose medular. O diagnóstico baseou-se e m dados laboratoriais ( e s p e c i a l m e n t e a positividade da r e a ç ã o de f i x a ç ã o de c o m p l e m e n t o para cisticercose no líquido c e f a l o r r a q u e a n o ) ou nos resultados cirúrgicos. Outros aspectos do e x a m e do líquido c e f a l o r r a q u e a n o ( p r e -sença de eosinófilos, p r o t e i n o r r a q u i a e resultados das provas m a n o m é t r i c a s )
são comentados. A p e r i m i e l o g r a f i a demonstrou existência de b l o q u e i o do canal r a q u e a n o e m 5 casos. T r ê s dêstes pacientes f o r a m submetidos a la-m i n e c t o la-m i a , c o la-m resultados v a r i á v e i s .
O quadro n e u r o l ó g i c o p r e d o m i n a n t e foi o de c o m p r e s s ã o da medula e / o u das r a í z e s nervosas ( 4 c a s o s ) . D o i s pacientes a p r e s e n t a v a m uma
sín-d r o m e corsín-donal p o s t e r i o r q u e simulava a tabes sín-dorsal. D o i s outros pacien-tes a p r e s e n t a v a m um quadro de m e n i n g o m i e l i t e n ã o sistematizada. U m pa-ciente tinha uma s í n d r o m e sugestiva de m i e l o s e funicular, m a s a presença de sintomas cerebrais e os resultados dos e x a m e s de l a b o r a t ó r i o l e v a r a m a considerar a cisticercose c o m o a e t i o l o g i a mais p r o v á v e l .
R E F E R E N C E S
-V I T C H , G. — M é n i n g i t e à c y s t i c e r q u e s . R e v . n e u r o l . 42:514-518, 1924. 3. B R I N C K , G. — L a C i s t i c e r c o s i s C e r e b r a l : E s t u d i o a n á t o m o p a t o l ó g i c o y c l í n i c o . L e b l a n c S t a n -l e y & U r z ú a , S a n t i a g o de C h i -l e , 1940. 4. B U S S E , W . — B e i t r a g z u r k -l i n i s c h e n D i a g n o s t i k d e r p a r a s i t ä r e n E r k r a n k u n g e n des Z e n t r a l n e r v e n s y s t e m s . A r c h . P s y c h i a t . N e r v e n k r . 95:189-206, 1931. 5. C A B I E S E S , F . ; V A L L E N A S , M . and L A N D A , R . — C y s t i c e r c o s i s o f t h e spinal c o r d . J. N e u r o s u r g . 16:337-341, 1959. 6. D I X O N , H . B . F . and L I P S C O M B , F. M . — C y s t i c e r c o s i s : an a n a l y s i s and f o l l o w - u p o f 450 cases. M e d . R e s . C o u n c i l , S p e c i a l R e p . S e r v . N o . 299, H e r M a j e s t y S t a t i o n e r y O f f i c e , L o n -don, 1961. 7. G U C C I O N E , A . — L a C i s t i c e r c o s i del S i s t e m a N e r v o s o C e n t r a l e U m a n o . S o c i e t à E d i t r i c e L i b r a r i a , M i l a n o , 1919. 8. G U I L L A I N , G.; B E R T R A N D , I . and T H U -R E L , -R . — É t u d e a n a t o m i q u e et c l i n i q u e d ' u n e m é n i n g i t e b a s i l a i r e e t s p i n a l e à Cysticercus racemosus. R e v . n e u r o l . 60:114125, 1933. 9. H E N N E B E R G , R . — P l u r i -r a d i c u l ä -r e -r H i n t e -r s t -r a n g s d e g e n e -r a t i o n i n f o l g e v o n s p i n a l e -r C y s t i c e -r k e n m e n i n g i t i s . Z. g e s . N e u r o l . P s y c h i a t . 9:1-34, 1912. A p u d G u c c i o n e7
. 10. K N A P P , P . C. — Cysticercus racemosus (Taenia solium) o f t h e spinal c o r d . J. n e r v . m e n t . D i s . 50:62, 1919. 11. M E Y E R , E. — A m y o t r o p h i s c h e L a t e r a l s c k l e r o s i s c o m b i n i e r t m i t m u l t i p l e n H i r n c y s -t i c e r k e n . A r c h . P s y c h i a -t . N e r v e n k r . 41:640-652, 1906. A p u d G u c c i o n e7
. 12. M O N -T E I R O - S A L L E S , F . J. — C i s t i c e r c o s e c e r e b r a l . -T e s e . F a c u l d a d e de M e d i c i n a , S ã o P a u l o , 1934. 13. M O R A W I E K A , J. — M e n i n g i t e s p i n a l e à c y s t i c e r q u e s . R e v . n e u r o l . 48:762763, 1927. 14. P I C H L E R , R . — ü b e r e i n e m F a l l v o n C y s t i c e r k e n i m R ü c k e n -m a r k e des M e n s c h e n . P r a g . -m e d . W s c h r . 25:181-183, 1900. A p u d G u c c i o n e7
. 15. R I C C I A R D I - C R U Z , O . — C o m p r e s s ã o r a d i c u l o m e d u l a r p o r c i s t i c e r c o s : r e g i s t r o de dois casos c o m t r a t a m e n t o c i r ú r g i c o . A r q . N e u r o - p s i q u i a t . ( S . P a u l o ) 19:231-235, 1S81. 16. R O C C A , E. D . — C i s t i c e r c o s i s i n t r a m e d u l a r . R e v . N e u r o - p s i q u i a t . 22: 166173, 1959. 17. R O S E N B L A T H — E i n F a l l v o n C y s t i c e r k e n m e n i n g i t i s m i t v o r -w i e g e n d e r B e t e i l i g u n g des R ü c k e n m a r k s . D e u t s c h . Z. N e r v e n h e i l k . 46:113-126, 1913. A p u d G u c c i o n e7
. S C H M I T E , P . — L a c y s t i c e r c o s e c é r é b r a l e . A n n . M é d . 24:288-305, 1C28. 19. T R É T I A . K O F F , C ; P A C H E C O E S I L V A , A . C. — C o n t r i b u i ç ã o p a r a o estudo da c y s t i c e r c o s e c e r e b r a l e e m p a r t i c u l a r das lesões c e r e b r a e s t o x i c a s á dist a n c i a n e s dist a a f f e c ç ã o . M e m . H o s p . J u q u e r y 1:3766, 1924. 20. V A S I L I U , T . — M e -n i -n g i t e s p i -n a l e à c y s t i c e r q u e s . P r e s s e m é d . 29:522-523, 1921. 21. V E R G A , P . ; D A Z Z I , A . — D i un r a r o caso de c i s t i c e r c o r a c e m o s o a l o c a l i z z a z i o n e s p i n a l e : s t u d i o c l i n i c o ed a n a t o m o - i s t o l o g i c o . P o l i c l i n i c o , sez. med., 33:65-109, 1923. 22. W O L L E N B E R G , R . — ü b e r d i e C y s t i c e r k e n , i n s b e s o n d e r e den C y s t i c e r c u s r a c e m o s u s des G e h i r n s . A r c h . P s y c h i a t . N e r v e n k r . 40:98-150, 1905. A p u d G u c c i o n e7
