R E G I S T R O D E C A S O S
PARASAGITTAL CORTICAL ATROPHY: A CAUSE OF "PRIMARY
LATERAL SCLEROSIS"
H E L I O L E M M I *
R I C H A R D D A L Y *
R O B E R T A N G * *
C u r r e n t l y t h e p r o b l e m of the e x i s t e n c e o f p r i m a r y l a t e r a l sclerosis as a distinct clinical e n t i t y is not a m a t t e r o f m u c h c o n t r o v e r s y . I t seems g e n e r a l l y a c c e p t e d t h a t w h a t w a s o n c e r e g a r d e d as a disease process char-a c t e r i z e d b y s e l e c t i v e d e g e n e r char-a t i o n of t h e p y r char-a m i d char-a l t r char-a c t s is usuchar-ally, if n o t a l w a y s , but one m a n i f e s t a t i o n o f a n u m b e r o f w e l l defined diseases, such as a m y o t r o p h i c l a t e r a l sclerosis, m u l t i p l e sclerosis, subacute c o m b i n e d d e -g e n e r a t i o n o f t h e cord, etc. H o w e v e r , it is not v e r y l o n -g a -g o t h a t such k e e n o b s e r v e r s as W e c h s l e r and B r o d y 1
, and S t a r k and M o e r s c h2
, h a v i n g f o l l o w e d a f a i r l y l a r g e n u m b e r of cases e x h i b i t i n g no a b n o r m a l i t i e s o t h e r than b i l a t e r a l p y r a m i d a l t r a c t signs o v e r periods o f s e v e r a l y e a r s , w e r e convinced t h a t p r i m a r y l a t e r a l sclerosis e x i s t e d as a s e p a r a t e disease state. N o c o n v i n c i n g p a t h o l o g i c a l m a t e r i a l has y e t been presented t o support their conviction. G r e e n f i e l d3
, and B r a i n4
b o t h i g n o r e t h e p r o b l e m . W a l s h e5 expressed t h e opinion t h a t " . . . m o s t cases so l a b e l e d u l t i m a t e l y p r o v e t o be a m y o t r o p h i c l a t e r a l sclerosis o r s o m e o t h e r k n o w n a f f e c t i o n o f t h e nervous system, and t h e t i t l e corresponds t o n o special r e c o g n i z e d p a t h o l o g i c process and should be abandoned." M e r r i t t6
has a v e r y s i m i l a r v i e w . T h e unusual features of t h e f o l l o w i n g case i l l u s t r a t e t h e h a z a r d i n v o l v e d in the l i b e r a l use o f this diagnosis.
C A S E R E P O R T
W . C . , a 54 y e a r o l d w h i t e m a l e , w a s a d m i t t e d t o t h e N e u r o l o g y S e r v i c e , J o h n G a s t o n H o s p i t a l , M e m p h i s , T e n n e s s e e , o n J a n u a r y 4, 1961. H i s o n l y c o m p l a i n t a t t h a t t i m e w a s m a r k e d d i f f i c u l t y i n w a l k i n g , d u e t o w h a t h e d e s c r i b e d as t i g h t n e s s a n d s h a k i n g o f b o t h l o w e r e x t r e m i t i e s . A t t h e a g e o f 7 y e a r s h e s u f f e r e d a s e v e r e i l l n e s s o f a b o u t 8 w e e k s d u r a t i o n c h a r a c t e r i z e d b y a h i g h f e v e r a n d c l o u d i n g o f c o n s c i o u n e s s . I m m e d i a t e l y f o l l o w i n g t h i s i t w a s n o t e d t h a t h e h a d d i f f i c u l t y in
e v e r t i n g t h e l e f t f o o t , a n d t h e r e w a s m o d e r a t e w e a k n e s s o f t h e l e f t l o w e r e x t r e m i t y . F u r t h e r d e t a i l s r e g a r d i n g t h e a c u t e i l l n e s s w e r e n o t a v a i l a b l e . A s l i g h t d i f f i c u l t y w i t h g a i t persisted, b u t t h e r e w e r e n o s i g n s o f p r o g r e s s i o n u n t i l , a t a b o u t t h e a g e o f 15, h e b e g a n t o e x p e r i e n c e f e e l i n g s o f t i g h t n e s s a n d s h a k i n g in t h e l e f t l o w e r e x t r e m i t y o n e x e r t i o n . A b o u t 20 y e a r s a g o t h e s a m e t y p e o f d i s a b i l i t y i n t h e r i g h t l o w e r e x t r e m i t y d e v e l o p e d g r a d u a l l y . S i n c e t h a t t i m e , sudden m a r k e d i n creases in s e v e r i t y h a v e p u n c t u a t e d l o n g i n t e r v a l s d u r i n g w h i c h t h e d i s o r d e r r e -m a i n e d s t a t i o n a r y , b u t t h e r e h a v e b e e n n o p e r i o d s o f r e -m i s s i o n . T h e r e w a s n o h i s t o r y s u g g e s t i v e o f b l a d d e r , b o w e l , o r s e x u a l d y s f u n c t i o n , a n d n o o t h e r c o m -p l a i n t s r e f e r a b l e t o t h e n e r v o u s s y s t e m . Examination — T h e g e n e r a l -p h y s i c a l e x a m i n a t i o n w a s w i t h i n n o r m a l l i m i t s . T h e r e w a s n o m e n t a l d e f i c i t . T h e g a i t w a s s l i g h t l y w i d e based, s l o w a n d m a r k e d l y spastic. A s s o c i a t e d m o v e m e n t s o f t h e u p p e r e x t r e m i t i e s w e r e n o r m a l . V e r y b r i s k d e e p t e n d o n r e f l e x e s in t h e l o w e r e x t r e m i t i e s w e r e a s s o c i a t e d w i t h s e v e r e b i l a t e r a l c l a s p k n i f e s p a s t i c i t y , s u s t a i n e d p a -t e l l a r a n d a n k l e c l o n u s , e x -t e n s o r -t o e responses, a n d c l o n i c m o v e m e n -t s o f -t h e l e g s on v o l u n t a r y e x t e n s i o n . T h e a b d o m i n a l r e f l e x e s w e r e a c t i v e . T h e r e w a s a s l i g h t v a r u s d e f o r m i t y o f t h e l e f t f o o t . T h e r e m a i n d e r o f t h e n e u r o l o g i c a l e x a m i n a t i o n r e v e a l e d n o a d d i t i o n a l a b n o r m a l i t i e s . Laboratory data — R o u t i n e a n a l y s i s o f t h e b l o o d , u r i n e a n d c e r e b r o s p i n a l f l u i d w e r e a l l w i t h i n n o r m a l l i m i t s . S k u l l , spine and chest X - r a y s w e r e n o r m a l . M y e l o g r a p h y o f t h e e n t i r e spinal s u b a r a c h n o i d s p a c e r e v e a l e d n o e v i d e n c e o f p a t h o l o g y . T h e p a t i e n t w a s d i s c h a r g e d o n J a n u a r y 22, 1961 a s " u n d i a g n o s e d d i s e a s e o f t h e n e r v o u s s y s t e m " , a l t h o u g h t h e p o s s i b i l i t y o f t h i s b e i n g a t r u e case o f p r i m a r y l a t e r a l s c l e r o s i s w a s c o n s i d e r e d .
2nd Admission — T h e p a t i e n t c o n t i n u e d t o c o m p l a i n o f i n c r e a s i n g d i s a b i l i t y , and a l t h o u g h t h e r e w e r e n o n e w f i n d i n g s , h e w a s a d m i t t e d f o r r e e v a l u a t i o n on M a r c h 4, 1962. E l e c t r o m y o g r a p h y o f t h e l e f t e x t e n s o r d i g i t o r u m b r e v i s , b o t h a n -t e r i o r -t i b i a l s , a n d -t h e l e f -t q u a d r i c e p s , a n d n e r v e c o n d u c -t i o n s-tudies o f -t h e l e f -t p e r o n e a l n e r v e r e v e a l e d n o e v i d e n c e o f l o w e r m o t o r n e u r o n e i n v o l v e m e n t . T w o e l e c t r o e n c e p h a l o g r a m s , t h e s e c o n d e m p l o y i n g s e v e r a l e x p l o r a t o r y e l e c t r o d e s i n t h e p a r a s a g i t t a l a r e a s , r e v e a l e d o n l y a f o c u s o f t h e t a a c t i v i t y in t h e r i g h t o c c i p i t a l r e g i o n , b u t n o o t h e r a b n o r m a l i t i e s . P n e u m o e n c e p h a l o g r a p h y d e m o n s t r a t e d r a t h e r m a r k e d d i l a t a t i o n o f t h e p o s t e r i o r r e g i o n o f t h e r i g h t l a t e r a l v e n t r i c l e , p a r t i c u l a r l y i n t h e a r e a o f t h e t r i g o n e , m u l t i p l e w i d e n e d sulci o v e r t h e r i g h t h e m i s p h e r e , a n d a p r o m i n e n t c r e s c e n t o f a i r o v e r t h e r i g h t i n s u l a . H o w e v e r , m o r e r e m a r k a b l y t h e r e w a s t h e a c c u m u l a t i o n o f b i l a t e r a l a n d f a i r l y s y m m e t r i c a l p o c k e t s o f s u b a r a c h n o i d a i r , a b o u t 2 c e n t i m e t e r s i n d i a m e t e r , l y i n g j u s t l a t e r a l t o e i t h e r side o f t h e f a l x , and s l i g h t l y a n t e r i o r t o t h e v e r t e x . B i l a t e r a l c a r o t i d a n g i o g r a p h y , i n c l u d i n g v e r y s a t i s f a c t o r y v e n o u s phases, s h o w e d n o a b n o r m a l i t y . T h e p a t i e n t w a s d i s c h a r g e d o n A p r i l 3, 1962 w i t h a p r e s u m p t i v e d i a g n o s i s o f t h r o m b o s i s o f t h e s u p e r i o r s a g i t t a l sinus, r e c a n a l i z e d .
D I S C U S S I O N
T h e b i l a t e r a l p a r a s a g i t t a l a c c u m u l a t i o n s of subarachnoid air, i n d i c a t e d b y t h e a r r o w s in F i g . 1, w e r e t h o u g h t t o b e a unique f i n d i n g . D a v i d o f f and E p s t e i n7
Consideration of the w o r k of Bailey and H a s s8
lends support to this conclusion. In one of their cases studied pathologically it was demonstrated that a thrombus in the superior longitudinal sinus can extend to adjacent veins, "even at some distance from the superior longitudinal sinus". These investigators also concluded that if a patient can survive for at least a few
weeks, there will be recanalization of the occluded sinus. The experimental
studies of Owens et aZ. 9
, Beck and R u s s e l1 0
, and D e n n y - B r o w n1 1
, using different techniques to occlude the flow of blood in the superior longitudinal sinus in a variety of animals, again showed rapid organization and recanal-ization of the thrombi in most instances.
Obstruction of the superior longitudinal sinus in its posterior two-thirds frequently results in infarction of the areas drained by the sinus, with
sub-sequent tissue necrosis, liquefaction and reabsorption. Secondary
hemor-rhage may occur into the wall of the sinus (Carrie and J a f f e1 2
) and into the necrotic cerebral tissue, as occurred in the cases of Bailey and Hass. They also described the lesions as extending as deep as the white matter, leaving a communication with the subarachnoid space. A portion or all of
a gyrus may b e involved in the atrophic process; necropsy examination reveals marked shrinkage at the affected sites.
In our opinion, the pneumoencephalographic demonstration of markedly
atrophic areas in the brain of the patient under consideration can not be
disputed. T h e location, distribution, and gross nature of the lesions
co-incides strikingly with the pathology occurring subsequent to obstruction
of the superior longitudinal sinus. W e would also like to stress that the
clinical picture has been one characterized by chronic progression over a
period of about thirty years. Assuming that the existence of "primary
lateral sclerosis" is a fiction, it appears extremely unlikely that any of
the other numerous causes of w h a t Courville
1 3has termed the "parasagittal
syndrome" could account for the prolonged course in this case.
Further-more, the exclusive involvement of the l o w e r extremities is well accounted
for by the bilateral, symmetrical, parasagittal lesions. The additional areas
of atrophy in the right hemisphere seen in the pneumoencephalogram
co-incide well with typical autopsy material ( F i g . 2 ) , in which asymmetrical
involvement of the hemispheres is very common. A n d considering w h a t is
known regarding recanalization of the thrombosed superior longitudinal sinus,
angiographic demonstration of a patent sinus in this case does not conflict
with our diagnosis.
It should be emphasized that except for the febrile illness at the age
illness. T h e e t i o l o g y of t h e e a r l i e r disease r e m a i n s a m a t t e r of speculation, but i t seems l i k e l y t h a t a f e b r i l e condition of 8 w e e k s duration m a y h a v e been associated w i t h a d e g r e e of inanition, a c i r c u m s t a n c e w e l l k n o w n to b e a c c o m p a n i e d b y t h e d e v e l o p m e n t of venous sinus thrombosis. B y e r s , c o m m e n t i n g on t h e paper b y R a y and D u n b a r1 4
, s t a t e d t h a t in a study of 50 cases of thrombosis of t h e superior longitudinal sinus a t t h e Children's H o s p i t a l in Boston, t h e r e w a s " e x t r e m e d i v e r s i t y of s y m p t o m a t o l o g y as c o m p a r e d t o p a t h o l o g y " . T h e r e is a corresponding w i d e v a r i e t y of late sequelae in those s u r v i v i n g t h e illness, and t h e absence of deficits other than s e v e r e m o t o r i m p a i r m e n t in o u r p a t i e n t suggests o n l y t h a t the original illness w a s not a s e v e r e , diffuse encephalitis, as t y p i f i e d b y numerous v i r a l diseases. T h a t t h e disturbance o f consciousness during t h e acute illness m a y h a v e been secondary t o occlusion of the superior longitudinal sinus seems p r o b a b l e 15, 16, 1 7
. I n f e c t i o n s about the head, o r t r a u m a t o t h e head as causes of venous sinus thrombosis a r e m e n t i o n e d b y s e v e r a l authors 18, 19, 20, 2 1
. B y history, n e i t h e r of these e t i o l o g i e s w e r e o p e r a t i v e in this case. O t h e r m o r e r e m o t e causes a r e included in an e x t e n s i v e list g i v e n b y F o r d 2 2
.
T h i s patient is considered as an unusual clinical case coupled w i t h a r e m a r k a b l e p n e u m o e n c e p h a l o g r a m . I t w o u l d be n a i v e to assume that the diagnosis has been d e f i n i t e l y established. H o w e v e r , w e hope t o h a v e pointed t o the need f o r careful and e x t e n s i v e studies in cases p r e s u m e d t o be " p r i m a r y l a t e r a l sclerosis", and t o h a v e d e m o n s t r a t e d t h e usefulness of p n e u m o e n c e p h a l o g r a p h y in s t u d y i n g o n e t y p e of c e r e b r o v a s c u l a r disease.
S U M M A R Y
A case of s e v e r e spastic paraparesis of the l o w e r e x t r e m i t i e s is presented as an e x a m p l e of t h e l a t e sequelae of thrombosis of t h e superior longitudinal sinus. E v i d e n c e in support of this p r e s u m p t i v e diagnosis is g i v e n b y analysis of t h e clinical history and the r e m a r k a b l e pneumoencephalographic find-ings. T h i s case affords t h e o p p o r t u n i t y t o stress t h e need f o r caution and r e s t r a i n t in the use of the diagnosis " p r i m a r y l a t e r a l sclerosis".
R E S U M O
R E F E R E N C E S
1. W E C H S L E R , I . W . ; B R O D Y , S. — T h e p r o b l e m o f p r i m a r y l a t e r a l sclerosis. J . A . M . A . , 130:1195, 1946. 2. S T A R K , F . M . ; M O E R S C H , F . P . — P r i m a r y l a t e r a l s c l e r o s i s ; a d i s t i n c t c l i n i c a l e n t i t y . J. N e r v . & M e n t . Dis., 102:332, 1945. 3. G R E E N -F I E L D , J. G. — N e u r o p a t h o l o g y , e d . 1. E d w a r d A r n o l d L t d . , L o n d o n , 1958. 4. B R A I N , R . — D i s e a s e s o f t h e N e r v o u s S y s t e m , ed. 5. O x f o r d U n i v e r s i t y P r e s s , L o n d o n , 1955. 5. W A L S H E , F . M . R . — D i s e a s e s o f t h e N e r v o u s S y s t e m , ed. 8. W i l l i a m s and W i l k i n s C o . , B a l t i m o r e , p. 240. 6. M E R R I T T , H . H . — A T e x t b o o k o f N e u r o l o g y , ed. 2. L e a & F e b i g e r , P h i l a d e l p h i a , 1959, p. 484. 7. D A V I D O F F , L . M . ; E P S T E I N , B . S. — T h e A b n o r m a l P n e u m o e n c e p h a l o g r a m , e d . 1. L e a & F e b i g e r , P h i l a d e l p h i a , 1950, pp. 356-363. 8. B A I L E Y , O . T . ; H A S S , G. M . — D u r a l sinus t h r o m b o s i s o f t h e s u p e r i o r l o n g i t u d i n a l sinus and its r e l a t i o n t o c e r t a i n a c q u i r e d c e r e b r a l lesions in c h i l d h o o d . B r a i n 60:293, 1937. 9. O W E N S , G.; S T A H L M A N , G . ; C A P P S , J.; M E I R O W S K Y , A . M . — E x p e r i m e n t a l o c c l u s i o n o f d u r a l sinuses. J. N e u r o s u r g . , 14:640, 1957. 10. B E C K , D . J. K ; R U S S E L L , D . S. — E x p e r i m e n t s on t h r o m b o s i s o f l o n g i t u d i n a l sinus. J. N e u r o s u r g . , 3:337, 1946. 11. D E N N Y - B R O W N , D . ; H O R E N S T E I N , S.; F A N G , H . C. H . — C e r e b r a l ' i n f a r c t i o n p r o d u c e d b y v e n o u s d i s t e n t i o n . J. N e u r o p a t h . , 15:146, 1956. 12. C A R R I E , A . W . ; J A F F E , F . A . — T h r o m b o s i s o f s u p e r i o r s a g i t t a l sinus ( c a u s e d by t r a u m a w i t h o u t p e n e t r a t i n g i n j u r y ) . J. N e u r o s u r g . , 11:173, 1954. 13. C O U R V I L L E , C. B . — T h e p a r a s a g i t t a l s y n d r o m e . B u l l . L o s A n g e l e s N e u r o l . S o c , 8:31, 1943. 14. R A Y , B . S.; D U N B A R , H . S. — T h r o m b o s i s o f t h e s u p e r i o r s a g i t t a l sinus as a c a u s e o f p s e u d o t u m o r c e r e b r i : m e t h o d s o f d i a g n o s i s and t r e a t m e n t . T r a n s . A m e r . N e u r o l . A s s . , 75:12, 1950. 15. S M I T H , J. C. — P r i m a r y c e r e b r a l t h r o m b o p h l e b i t i s . J . A . M . A . , 148:613, 1952. 16. P R I C K , J. J. — T h e s o c a l l e d s p o n t a n e o u s ( s i v e p r i m a r y ) t h r o m b o s i s o f t h e sinus l o n g i t u -d i n a l i s s u p e r i o r a n -d its t r i b u t a r y p i a l v e i n s . F o l i a P s y c h i a t . et N e u r o l . , 58:259, 1955. 17. M A R T I N , J. P . — L e s i o n s v e i n e u s e s c e r e b r a l e s sans i n f e c t i o n . R e v . N e u r o l . , 80:433, 1948. 18. S T U A R T , E. A . ; O ' B R I E N , F . H . ; M c N A L L Y , W . J. — C e r e b r a l v e n o u s t h r o m b o s i s : its o c c u r r e n c e ; its l o c a l i z a t i o n ; its s o u r c e a n d s e q u e l a e . A n n . O t o l . ( S t . L o u i s ) 60:406, 1951. 19. H O L M E S , G.; S A R G E N T , P . — I n j u r i e s of t h e s u p e r i o r l o n g i t u d i n a l sinus. B r i t . M e d . J., 2:493, 1915. 20. W H A R T O N , H . R . — W o u n d s o f t h e v e n o u s sinuses o f t h e b r a i n . A n a n a l y s i s o f s e v e n t y cases. A n n . Surg., 3 4 : 8 1 , 1901. 21. C A I R N S , H . ; C A L V E R T , C. A . ; D A N I E L , P . ; N O R T H -C R O F T , G. B . — -C o m p l i c a t i o n s o f h e a d w o u n d s , w i t h s p e c i a l r e f e r e n c e t o i n f e c t i o n . B r i t . J. Surg., W a r S u r g . Suppl., 1:198, 1947. 22. F O R D , F . R . — D i s e a s e s o f t h e N e r v o u s S y s t e m in I n f a n c y , C h i l d h o o d a n d A d o l e s c e n c e , ed. 4. C h a r l e s C. T h o m a s , S p r i n g f i e l d ( I l l i n o i s ) , 1960, p. 888.
