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Epilepsy in conjoined Siamese twins
CLINICAL A N D L A B O R A T O R Y N O T E S Edited by R. S. SCHWAB, M.B. and H. FISCHGOLD, M.D. EPILEPSY IN C O N J O I N E D
SIAMESE T W I N S
IAN H a y BaOWN, M . D .
Miami Valley Hospital, Dayton, Ohio (Received f o r p u b l i c a t i o n : J u n e 26~ 1958) INTRODUCTION C o n j o i n e d t w i n s were f i r s t described in t h e y e a r 375 A.D. T h e r e were m a n y n o t a b l e cases f r o m t h a t time oil, b u t t h e t e r m ~ S i a m e s e t w i n s " became popularized following t h e a p p e a r a n c e s of C h a n g a n d E n g (1811-1874) in t h e B a r n u m Circus in this c o u n t r y ( L u c k h a r d t 1941). The incidence of Siamese t w i n s is d i f f i c u l t to estimate~ b u t in l a r g e r cities t h e r e have been r e p o r t s of these b i r t h s o c c u r r i n g iu approxi m a t e l y every 60,000 to 80,000 deliveries ( M o r t i m e r et al. 1951, 1952; P o t t e r 1952), while it h a s been e s t i m a t e d t h a t world wide t h e r e is p r o b a b l y a n incidence of s u r v i v a l of a b o u t one in every 2 million b i r t h s ( A i r d 1954, 1955). The i n t e r e s t in S i a m e s e t w i n s in t h e p a s t h a s been m a i n l y o b s t e t r i c a l a n d a n a t o m i c a l , b u t m o r e r e c e n t l y s u r g e o n s have been a t t e m p t i n g to s e p a r a t e t h e m with v a r y i n g success (e.g. A i r d 1955; G r o s s m a n et al. 1953; H o l g a t e et al. ] 9 5 6 ; Spencer 1956; W i l s o n et al. 1957). I n spite of all m o d e r n advances, tile t o t a l n u m b e r of s u r v i v a l s is small a n d it is believed t h a t at t h i s t i m e t h e r e are only f o u r sets of conjoined t w i n s alive in the U. S. A. I n tile h i s t o r y of D a y t o n , Ohio, t h e r e have been f o u r sets of S i a m e s e t w i n s delivered b u t t h e f i r s t s u r v i v o r s were R o n n i e a n d Donnie G. who were born on October 28, 1951. A l t h o u g h t w i n s h a d been anticipated, the p r ~ c t i t i o n e r h a d considerable d i f f i c u l t y with the delivery. T h e h e a d a n d shoulders of the f i r s t twin were easily delivered b u t w h e n t h e t r u n k failed to exit, the doctor s u s p e c t e d t h a t t h e u m b i l i c a l cord h a d t w i s t e d r o u n d the b o d y a n d in a t t e m p t i n g to correct this he discovered t h a t t h e reason f o r the de]'ly w a s the f u s i o n of the two t r u n k s . E v e n t u a l l y the doctor was able to o b t a i n a s u c c e s s f u l b u t belated delivery. The i n f a n t s were in no a p p a r e n t distress a n d weighed a t o t a l of 1 1 ~ ]bs. I n their early d e v e l o p m e n t t h e r e were no m a j o r difficulties. As cast be seen f r o m the p h o t o g r a p h s (fig. 1), the t w i n s are t h o r a e o p a g u s , b e i n g j o i n e d below tile xiphis t e r n m n . T h e y have i n d e p e n d e n t sets of all u p p e r o r g a n s , i n c l u d i n g h e a r t , liver, etc., b u t t h e y s h a r e a c o m m o n lower bowel a n d have a r u d i m e n t a r y urog e n i t a l a p p a r a t u s a n d a n u s w h i c h is e s s e n t i a l l y a cloaca (fig. 2, 3). A s is so o f t e n t h e case w i t h S i a m e s e twins, R o n n i e a n d D o n n i e were n o t quite s y m n l e t r i c a l or identical a n d in earlier y e a r s Donnie was t h e l a r g e r t w i n a n d t h e i n i t i a t o r of m o s t of t h e i r activities, m e n t a l l y a n d physically. However, in the p a s t few y e a r s the roles have been reversed a n d
Ronnie is now the l a r g e r a n d the leader, in w a l k i n g the t w i n s can move in t h e conventional w:,y, b u t are more p r o f i c i e n t when m o v i n g laterally. F ( r i n c r e a s e d speed t h e y have developed a t e c h n i q u e , , h e r e b y , in
Fig. 1 Early photograph of the twins illustrating the ar~:a of fusion.
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566 effect, Ronme
IAN HAY BROWN
carries Don~ie and only one set of legs
is u s e d . The twins were well until February, 1957, when Ronnie developed a high fever which lasted a few days. Two months later a similar febrile episode
o c c u r r e d , b u t a s t h e t w i l l s wer(~ i n c i r c u i t w i t h a c a r l d v a l , n o d i a g n o s i s w a s m ' l d e i,, e i t h e r i n s t a n c e a n d t r e a t m e n t w a s n o n - s p e c i f i c . Thes,. i l l n e s s e s d i d r e v e a l , however, the physiologic indepe~dence of the twins for Donnie was completely una~:'ected while Ronnie
l~tg. 2 A catheter inserted a short distance into the cloaca enables dye to fill the cotillion u r e t h r a a n d separate bladders.
Fig. 3 The same catheter as in figure 2 inserted further into the cloaca allows the c( ~mlon rectum to be filled. The separation into 2 transverse colons call be seen.
EPILEPSY
IN CONJOINED SIAMESE TWINS
was febrile a n d sick. I n J u n e , 1957, R o n n i e b e g a n to have w h a t were f i r s t believed to be "cramps" of t h e l e f t leg a t w h i c h t i m e t h e leg w a s s l i g h t l y ra!sed from the ground and appeared "stiff". The "cramps" l a s t e d only a f e w seconds a t a t i m e b u t even so were e m b a r r a s s i n g as t h e y u s u a l l y occurred
567
the t w i n s were r e c o m m e n d e d to r e t u r n h o m e f o r a n e v a l u a t i o n of t h i s possibility. T h e p h y s i c i a n who h a d b r o u g h t the t w i n s into the world some six y e a r s previously r e f e r r e d t h e m to t h e E E G D ( . p a r t m e n t of M i a m i Valley H o s p i t a l in N o v e m b e r 1957, f o r study. On closer q u e s t i o n i n g of t h e p a r e n t s , it w a s
Fig. 4 Electrode placements. in periods of e x c i t e m e n t w h e n R o n n i e w a s r u n n i n g a n d as a consequence he would f a l l on t o p of Donnie. As weeks w e n t by, it w a s n o t e d t h a t R o n n i e ' s l e f t a r m was b e c o m i n g involved w i t h these c r a m p s , a n d l a t e r the p i c t u r e included h e a d - t u r n i n g to t h e left, u s u a l l y a f i x e d stare, w h i c h r a r e l y w a s also to t h e left. I n the course of t h e t r a v e l l i n g a r o u n d , no d e f i n i t i v e diagnosis was made until the twins reached Arkansas where t h e d i a g n o s i s of epilepsy w a s considered a n d
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revealed t h a t ~ ' s h a k i n g " h a d occurred very rarely d u r i n g the n i g h t a n d b a d been h e a r d b u t m i s t a k e n f o r nightmares. TECHNIQUE W i t h the u n u s u a l a s s i g n m e n t g i v e n ~ , some caref u l p l a n n i n g w a s obviously necessary. Iv was decided t h a t in so f a r as t h e h i s t o r y w a s n o t t,,o clear, a n d because of the u n i q u e n e s s of t h e ~ituation, a
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Simultaneous recordings on the twins showing different phases of sleep. Note also the prominent EKG artefact in R'. Cent. lead of Donnio.
IAN HAY BROWN
568
s i m u l t a n e o u s r e c o r d i n g f r o m t h e two b r a i n s w a s m o s t desirable• Silver cup electrodes (of t h e t y p e supplied by t h e G r a s s I n s t r u m e n t C o m p a n y ) were a f f i x e d w i t h b e n t o n i t e over t h e f r o n t a l , central, occipital a n d a n t e r i o r - t e m p o r a l areas. A n electrode was placed on the v e r t e x a n d also one on each e a r ( f i g . 4). A n 8-channel G r a s s console w a s employed f o r m a k i n g s i m u l t a n e o u s r e c o r d i n g s f r o m f o u r c o m p a r a b l e derivations on each twin. B o t h m o n o p o l a r a n d b i p o l a r rec o r d i n g s were m a d e . I n a d d i t i o n a " c a r d i a c " lead
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RESULTS B e f o r e t h e r e c o r d i n g actually b e g a n t h e physiologic i n d e p e n d e n c e of t h e twins w a s i l l u s t r a t e d by t h e f a c t t h a t one became asleep s,,me 10 min. before, a n d in spite of, t h e other. The f i r s t p a g e s of r e c o r d i n g revealed t o t a l l y differ, at p h a s e s of sleep p a t t e r n for each t w i n a n d the t i f f e r e n c e r e m a i n e d t h r o u g h o u t ( f i g . 5). I n t h e ear!y p o r t i o n s of t h e r e c o r d i n g no a b n o r m a l i t i e s were observed in either twin, b u t t h e r e developed a hig.~ a m p l i t u d e slow 4
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l~ig. 6 The f i r s t 30 sec. of s e i z u r e a c t i v i t y b e g i n n i n g in th e r i g h t c e n t r a l r e g i o n of the a f f e c : e d twin. The m u s c l e t e n s i o n a n d movement a r t e f a c t coincide w i t h the c l i n i c a l onset c ' a r i g h t - s i d e d clonic s e i z u r e . ( S a m e sc a le as fig. 7.)
was a d d e d to each i n t r a s c a p u l a r region, the more desirable a n t e r i o r position b e i n g impossible on account of t h e j u x t a p o s i t i o n of the two chests. E v e n so, because of t h e positions t a k e n u p b y the twins, one l y i n g on his back a n d the o t h e r over h i m on his chest, the t a s k w a s n o t easy. I t w a s h o p e d t h a t b o t h w a k i n g a n d sleep records could be obtained, b u t the f o r m e r b e c a m e impossible as t h e boys c o n s t a n t l y f o u g h t a n d rolled over each o t h e r on t h e bed so t h a t Seconal ® h a d to be a d m i n i s t e r e d .
per sec. wave in t h e r i g h t c e n t r a l lead of R o n n i e which w a s followed a f e w seconds later b y a n increase in f r e q u e n c y of t h e s e w a v e s w i t h a s p r e a d essentially a n t e r i o r l y (fig. 6). These were followed by a f u r t h e r increase in f r e q u e n c y an,[ spread, a n d when s u p e r i m p o s e d muscle f a s t a c t i v i t y a p p e a r e d it w a s observed t h a t a r i g h t - s i d e d clonic seizure w a s in prog r e s s i n v o l v i n g the leg predomimLtely, b u t the a r m as well, l a s t i n g a b o u t 50 sec. I n :~ m u c h later portion of the record spike d i s c h a r g e s ~t,re observed in t h e
EPILEPSY
IN CONJOINED SIAMESE TWINS
r i g h t c e n t r a l r e g i o n w i t h a s l i g h t reflection to t h e l e f t c e n t r a l r e g i o n b u t w i t h no s p r e a d to t h e rem a i n i n g r i g h t - s i d e d leads. T h r o u g h o u t no abnorm a l i t i e s were n o t e d in t h e record of D o n n i e ( f i g . 7). T h e E K G riddle seemed to be solved r a t h e r early in the r e c o r d i n g f o r E K G a r t e f a c t could be detected in t h e m o n o p o l a r r e c o r d i n g s of b o t h l~onnie a n d Donnie a n d it w a s observed t h a t t h e s e a r t e f a c t s were
DISCUSSION W i t h t h e exception of t h e case of the c r a n i o p a g u s Brodie t w i n s ( D u r r 1952; G r o s s m a n (~ al. 1953; S u g a r 1953), t h e d a t a here p r e s e n t e d :Ire believed to be the f i r s t e l e c t r o e n c e p h a l o g r a p h i c r~cordings of epilepsy in S i a m e s e twins. I t is also of a t e r e s t f r o m the p u r e l y epileptic viewpoint t h a t t h e soizure h i s t o r y i n d i c a t e d the earliest m a n i f e s t a t i o n s as tonic move-
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Fig. 7 Simultaneous record of the unaffected twin during the same 30 sec. period as illustrated in figure 6.
Fig. 8 A section of the record where frontal leads of one twin were being run against the vertex of the other twin, revealing independent asynchronous EKG artefacts. s y n c h r o n o u s . U s i n g t h e t t c a r d i a c '~ l e a d s t h e same r e s u l t s seemed to be t h e case, b u t b y a lucky accident w h e n the scalp leads of l~onnie were r u n to the vertex of D o n n i e two d i s t i n g u i s h a b l e r h y t h m s could be i d e n t i f i e d a n d t h e s e were q u i t e a s y n c h r o n o u s , one a r o u n d 90 b e a t s / m i n , a n d t h e o t h e r a r o u n d 8 3 / m i n . ( f i g . 8).
m e n t s of t h e l e f t leg, s p r e a d i n g in tile course of d e v e l o p m e n t to o t h e r p o r t i o n s of t h e left side a n d f i n a l l y r e v e a l i n g r i g h t - s i d e d clonic seizures. T h e only E E G m a n i f e s t a t i o n s of seizure a c t i v i t y have been conf i n e d to the r i g h t c e n t r a l r e g i o n even w h e n the p a t i e n t was h a v i n g a clonic seizure on t h e i p s i l a t e r a l side. T h i s p h e n o m e n o n h a s been seen b y u s previously in
IAN HAY BROWN
570
a few cases, and of course reported by others (e.g. Penfield and Jasper 1954). The present EEG disturbance coincides very well with the earliest clinical manifestations of the seizure pattern, and this point has been clearly emphasized as being the most important by Penfield. The etiology is a matter of conjecture at the present time although it seems likely that some birth trauma a n d / o r anoxia occurred and that the febrile episodes early in 1957 triggered off the seizure mechanism. However, it should be noted that there is a strong hereditary factor present, for although neither parent has shown convulsive tendencies, there are many members of both sides of the family who have. On the f a t h e r ' s side, an uncle, female cousin and a nephew are known to have convulsions, while two male cousins of the mother are similarly affected. The very nature of the electroencephalographic and clinical phenomena, and the involvement in only one of identical twins suggest that heredity is not the main etiologic agent present. Since the original electroencephalographic examination there have been two further examinations on ]Connie which have been essentially normal. The first of these was prompted by an acute increase in the number of seizures to about 10 a night when the child was placed on phenobarbital, and the second was a follow up after seizures were becoming rather infrequent as a result of Dilantin ® and Tridione ® therapy. The twins are back on circuit with tlie carnival now and latest information indicates the latter combination of medication is proving effective in controlling the seizures. ~ e were interested in the paper by Johnson and Doherty (1957) on the simultaneous E K G recordings on their twins, and the earlier portion of our record seemed to indicate a similarity between the two cases in that the hearts apparently beat in both cases at the same rates. However, the accidental detection of asynchronous rhythms in our case led us to wonder if we in our early observation and Johnson et al. in their case were actually recording impulses from one heart through the chest of the other. This point should be noted by future observers who know more about cardiology than we profess when EKG studies on conjoined twins are conducted~ but thoracopagi present considerable difficulties in this respect and much ingenuity will be required to solve this problem. SUMMARY The history is presented of conjoined Siamese twins, one of whom has developed epileptic seizures. The EEG simultaneous recordings of the twins and a clinical seizure of one twin are presented and dis-
cussed. The chances of directly recording a seizure in an epileptic conjoined twin is estimated as being in the neighborhood of one in 500 million. No attempt has been made in this report to discuss the general problem of conjoined twinning, -heories of development, etc., for which adequate re:,rences exist (Aird 1954, 1955; Dawson 1944; D u n b ~ 1934; Gray et al. 1950; Luckhardt 1941; Mall 19(~; Mortimer et al. 1942; Perlstein et al. 1927; Potter 1952). Acknowledgment is made to I,~. A. Kuhr for the information given on the birth :.~d development of the twins, and to Dr. J. G. Tye f t" copies of X-rays. REFERENCEf AIRD, I. The conjoined twins of I~:ano. Brit. M. J., 1954, 1: 831-837. AIRD, I. Conjoined twins. Med. ~, orld, London, 1955, 83 : 217-221. DAWSON, H. Pygopagus twins; ~cport of dissection of the thorax, abdomen and ]~. lvis. Amer. J. Dis. Child., 1944, 68: 395-404. DUNBAR, A. E. Teratology. A~wr J. Surg., 1934, 24: 152-158. DURR, S. P. Craniopagus twins; report of a case. J.A.M.A., 1952, 150: 93-95. GRAY, C. M., Nix, It. G. and WALI CE, A. J. Thoraeopagus twins; prenatal di~:nosis. Radiology, 1950, 54: 398-400. GROSS]~AN, H. J., SUGAR, O., G];EELEY, P. W. and SADOVE,M. S. Surgical separe! ion in craniopagus. J.A.M.A., 1953, 153: 201-207. ~IOLGATE, J. and IKPE:ME, B. J. Cor~joined twins. Brit. J. Surg., 1956, 43: 626-7. JOHNSON, N. J. and DOHERTY, , E. Simultaneous electrocardiograms in thoraco~,agus twins. A ~ e r . Heart J., 1957, 53: 150-156. LUCKHARDT, A. B. Report of t i e autopsy of the Siamese twins together wit] other interesting information covering their li',,; a sketch of the life of Chang and Eng. Surg. Gynec. Obst., 1941, 72: 116-125. MALL, ]~. P. A study of the ca ~es underlying the origin of human monsters. J. iforp]wl., 1903, 19: 3-368. MORTI~IER, B. and KIRSHBAU)I,J D. Human double monster (so-called Siamese t~ as). Awer. J. Dis. Child., 1942, 64: 697-704. PENFIELD, W. and JASPER, H. Ep.,'epsy and the functional Anatomy of the H u ~ a n Brain. Little, Brown and Co., Boston, 1954. PERLSTEIN, M. A. and LECouNT, S. R. The history and necropsy report of the BoiJemian twins, RosaJosepha Blazek. (Pygopagus t,vins.) Arch. Path., 1927, 3: 171-192. POTTER, E. L. Pathology of the J~ .,etus and the Newborn. Publishers, 1952. SUGAR, O. The Brodie craniops~us twins. Trans. Amer. Neur. Assoc., 78th Meeting, 1953, P. 198-9. WmsoN, It. and STORER,E. H. Sur;~ery in "Siamese ~' twins: a report of three sets of conjoined twins treated surgically. Ann. Surg.. 1957, i45: 718-725.
Reference: BROWN, I. H. Epilepsy in conjoined siamese twins. EEG Clin. Neurophysiol., 1959, 11: 565-570.
®
SIAMESE T W I N S
IAN H a y BaOWN, M . D .
Miami Valley Hospital, Dayton, Ohio (Received f o r p u b l i c a t i o n : J u n e 26~ 1958) INTRODUCTION C o n j o i n e d t w i n s were f i r s t described in t h e y e a r 375 A.D. T h e r e were m a n y n o t a b l e cases f r o m t h a t time oil, b u t t h e t e r m ~ S i a m e s e t w i n s " became popularized following t h e a p p e a r a n c e s of C h a n g a n d E n g (1811-1874) in t h e B a r n u m Circus in this c o u n t r y ( L u c k h a r d t 1941). The incidence of Siamese t w i n s is d i f f i c u l t to estimate~ b u t in l a r g e r cities t h e r e have been r e p o r t s of these b i r t h s o c c u r r i n g iu approxi m a t e l y every 60,000 to 80,000 deliveries ( M o r t i m e r et al. 1951, 1952; P o t t e r 1952), while it h a s been e s t i m a t e d t h a t world wide t h e r e is p r o b a b l y a n incidence of s u r v i v a l of a b o u t one in every 2 million b i r t h s ( A i r d 1954, 1955). The i n t e r e s t in S i a m e s e t w i n s in t h e p a s t h a s been m a i n l y o b s t e t r i c a l a n d a n a t o m i c a l , b u t m o r e r e c e n t l y s u r g e o n s have been a t t e m p t i n g to s e p a r a t e t h e m with v a r y i n g success (e.g. A i r d 1955; G r o s s m a n et al. 1953; H o l g a t e et al. ] 9 5 6 ; Spencer 1956; W i l s o n et al. 1957). I n spite of all m o d e r n advances, tile t o t a l n u m b e r of s u r v i v a l s is small a n d it is believed t h a t at t h i s t i m e t h e r e are only f o u r sets of conjoined t w i n s alive in the U. S. A. I n tile h i s t o r y of D a y t o n , Ohio, t h e r e have been f o u r sets of S i a m e s e t w i n s delivered b u t t h e f i r s t s u r v i v o r s were R o n n i e a n d Donnie G. who were born on October 28, 1951. A l t h o u g h t w i n s h a d been anticipated, the p r ~ c t i t i o n e r h a d considerable d i f f i c u l t y with the delivery. T h e h e a d a n d shoulders of the f i r s t twin were easily delivered b u t w h e n t h e t r u n k failed to exit, the doctor s u s p e c t e d t h a t t h e u m b i l i c a l cord h a d t w i s t e d r o u n d the b o d y a n d in a t t e m p t i n g to correct this he discovered t h a t t h e reason f o r the de]'ly w a s the f u s i o n of the two t r u n k s . E v e n t u a l l y the doctor was able to o b t a i n a s u c c e s s f u l b u t belated delivery. The i n f a n t s were in no a p p a r e n t distress a n d weighed a t o t a l of 1 1 ~ ]bs. I n their early d e v e l o p m e n t t h e r e were no m a j o r difficulties. As cast be seen f r o m the p h o t o g r a p h s (fig. 1), the t w i n s are t h o r a e o p a g u s , b e i n g j o i n e d below tile xiphis t e r n m n . T h e y have i n d e p e n d e n t sets of all u p p e r o r g a n s , i n c l u d i n g h e a r t , liver, etc., b u t t h e y s h a r e a c o m m o n lower bowel a n d have a r u d i m e n t a r y urog e n i t a l a p p a r a t u s a n d a n u s w h i c h is e s s e n t i a l l y a cloaca (fig. 2, 3). A s is so o f t e n t h e case w i t h S i a m e s e twins, R o n n i e a n d D o n n i e were n o t quite s y m n l e t r i c a l or identical a n d in earlier y e a r s Donnie was t h e l a r g e r t w i n a n d t h e i n i t i a t o r of m o s t of t h e i r activities, m e n t a l l y a n d physically. However, in the p a s t few y e a r s the roles have been reversed a n d
Ronnie is now the l a r g e r a n d the leader, in w a l k i n g the t w i n s can move in t h e conventional w:,y, b u t are more p r o f i c i e n t when m o v i n g laterally. F ( r i n c r e a s e d speed t h e y have developed a t e c h n i q u e , , h e r e b y , in
Fig. 1 Early photograph of the twins illustrating the ar~:a of fusion.
[ 565 ]
566 effect, Ronme
IAN HAY BROWN
carries Don~ie and only one set of legs
is u s e d . The twins were well until February, 1957, when Ronnie developed a high fever which lasted a few days. Two months later a similar febrile episode
o c c u r r e d , b u t a s t h e t w i l l s wer(~ i n c i r c u i t w i t h a c a r l d v a l , n o d i a g n o s i s w a s m ' l d e i,, e i t h e r i n s t a n c e a n d t r e a t m e n t w a s n o n - s p e c i f i c . Thes,. i l l n e s s e s d i d r e v e a l , however, the physiologic indepe~dence of the twins for Donnie was completely una~:'ected while Ronnie
l~tg. 2 A catheter inserted a short distance into the cloaca enables dye to fill the cotillion u r e t h r a a n d separate bladders.
Fig. 3 The same catheter as in figure 2 inserted further into the cloaca allows the c( ~mlon rectum to be filled. The separation into 2 transverse colons call be seen.
EPILEPSY
IN CONJOINED SIAMESE TWINS
was febrile a n d sick. I n J u n e , 1957, R o n n i e b e g a n to have w h a t were f i r s t believed to be "cramps" of t h e l e f t leg a t w h i c h t i m e t h e leg w a s s l i g h t l y ra!sed from the ground and appeared "stiff". The "cramps" l a s t e d only a f e w seconds a t a t i m e b u t even so were e m b a r r a s s i n g as t h e y u s u a l l y occurred
567
the t w i n s were r e c o m m e n d e d to r e t u r n h o m e f o r a n e v a l u a t i o n of t h i s possibility. T h e p h y s i c i a n who h a d b r o u g h t the t w i n s into the world some six y e a r s previously r e f e r r e d t h e m to t h e E E G D ( . p a r t m e n t of M i a m i Valley H o s p i t a l in N o v e m b e r 1957, f o r study. On closer q u e s t i o n i n g of t h e p a r e n t s , it w a s
Fig. 4 Electrode placements. in periods of e x c i t e m e n t w h e n R o n n i e w a s r u n n i n g a n d as a consequence he would f a l l on t o p of Donnie. As weeks w e n t by, it w a s n o t e d t h a t R o n n i e ' s l e f t a r m was b e c o m i n g involved w i t h these c r a m p s , a n d l a t e r the p i c t u r e included h e a d - t u r n i n g to t h e left, u s u a l l y a f i x e d stare, w h i c h r a r e l y w a s also to t h e left. I n the course of t h e t r a v e l l i n g a r o u n d , no d e f i n i t i v e diagnosis was made until the twins reached Arkansas where t h e d i a g n o s i s of epilepsy w a s considered a n d
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revealed t h a t ~ ' s h a k i n g " h a d occurred very rarely d u r i n g the n i g h t a n d b a d been h e a r d b u t m i s t a k e n f o r nightmares. TECHNIQUE W i t h the u n u s u a l a s s i g n m e n t g i v e n ~ , some caref u l p l a n n i n g w a s obviously necessary. Iv was decided t h a t in so f a r as t h e h i s t o r y w a s n o t t,,o clear, a n d because of the u n i q u e n e s s of t h e ~ituation, a
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Simultaneous recordings on the twins showing different phases of sleep. Note also the prominent EKG artefact in R'. Cent. lead of Donnio.
IAN HAY BROWN
568
s i m u l t a n e o u s r e c o r d i n g f r o m t h e two b r a i n s w a s m o s t desirable• Silver cup electrodes (of t h e t y p e supplied by t h e G r a s s I n s t r u m e n t C o m p a n y ) were a f f i x e d w i t h b e n t o n i t e over t h e f r o n t a l , central, occipital a n d a n t e r i o r - t e m p o r a l areas. A n electrode was placed on the v e r t e x a n d also one on each e a r ( f i g . 4). A n 8-channel G r a s s console w a s employed f o r m a k i n g s i m u l t a n e o u s r e c o r d i n g s f r o m f o u r c o m p a r a b l e derivations on each twin. B o t h m o n o p o l a r a n d b i p o l a r rec o r d i n g s were m a d e . I n a d d i t i o n a " c a r d i a c " lead
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RESULTS B e f o r e t h e r e c o r d i n g actually b e g a n t h e physiologic i n d e p e n d e n c e of t h e twins w a s i l l u s t r a t e d by t h e f a c t t h a t one became asleep s,,me 10 min. before, a n d in spite of, t h e other. The f i r s t p a g e s of r e c o r d i n g revealed t o t a l l y differ, at p h a s e s of sleep p a t t e r n for each t w i n a n d the t i f f e r e n c e r e m a i n e d t h r o u g h o u t ( f i g . 5). I n t h e ear!y p o r t i o n s of t h e r e c o r d i n g no a b n o r m a l i t i e s were observed in either twin, b u t t h e r e developed a hig.~ a m p l i t u d e slow 4
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l~ig. 6 The f i r s t 30 sec. of s e i z u r e a c t i v i t y b e g i n n i n g in th e r i g h t c e n t r a l r e g i o n of the a f f e c : e d twin. The m u s c l e t e n s i o n a n d movement a r t e f a c t coincide w i t h the c l i n i c a l onset c ' a r i g h t - s i d e d clonic s e i z u r e . ( S a m e sc a le as fig. 7.)
was a d d e d to each i n t r a s c a p u l a r region, the more desirable a n t e r i o r position b e i n g impossible on account of t h e j u x t a p o s i t i o n of the two chests. E v e n so, because of t h e positions t a k e n u p b y the twins, one l y i n g on his back a n d the o t h e r over h i m on his chest, the t a s k w a s n o t easy. I t w a s h o p e d t h a t b o t h w a k i n g a n d sleep records could be obtained, b u t the f o r m e r b e c a m e impossible as t h e boys c o n s t a n t l y f o u g h t a n d rolled over each o t h e r on t h e bed so t h a t Seconal ® h a d to be a d m i n i s t e r e d .
per sec. wave in t h e r i g h t c e n t r a l lead of R o n n i e which w a s followed a f e w seconds later b y a n increase in f r e q u e n c y of t h e s e w a v e s w i t h a s p r e a d essentially a n t e r i o r l y (fig. 6). These were followed by a f u r t h e r increase in f r e q u e n c y an,[ spread, a n d when s u p e r i m p o s e d muscle f a s t a c t i v i t y a p p e a r e d it w a s observed t h a t a r i g h t - s i d e d clonic seizure w a s in prog r e s s i n v o l v i n g the leg predomimLtely, b u t the a r m as well, l a s t i n g a b o u t 50 sec. I n :~ m u c h later portion of the record spike d i s c h a r g e s ~t,re observed in t h e
EPILEPSY
IN CONJOINED SIAMESE TWINS
r i g h t c e n t r a l r e g i o n w i t h a s l i g h t reflection to t h e l e f t c e n t r a l r e g i o n b u t w i t h no s p r e a d to t h e rem a i n i n g r i g h t - s i d e d leads. T h r o u g h o u t no abnorm a l i t i e s were n o t e d in t h e record of D o n n i e ( f i g . 7). T h e E K G riddle seemed to be solved r a t h e r early in the r e c o r d i n g f o r E K G a r t e f a c t could be detected in t h e m o n o p o l a r r e c o r d i n g s of b o t h l~onnie a n d Donnie a n d it w a s observed t h a t t h e s e a r t e f a c t s were
DISCUSSION W i t h t h e exception of t h e case of the c r a n i o p a g u s Brodie t w i n s ( D u r r 1952; G r o s s m a n (~ al. 1953; S u g a r 1953), t h e d a t a here p r e s e n t e d :Ire believed to be the f i r s t e l e c t r o e n c e p h a l o g r a p h i c r~cordings of epilepsy in S i a m e s e twins. I t is also of a t e r e s t f r o m the p u r e l y epileptic viewpoint t h a t t h e soizure h i s t o r y i n d i c a t e d the earliest m a n i f e s t a t i o n s as tonic move-
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Fig. 7 Simultaneous record of the unaffected twin during the same 30 sec. period as illustrated in figure 6.
Fig. 8 A section of the record where frontal leads of one twin were being run against the vertex of the other twin, revealing independent asynchronous EKG artefacts. s y n c h r o n o u s . U s i n g t h e t t c a r d i a c '~ l e a d s t h e same r e s u l t s seemed to be t h e case, b u t b y a lucky accident w h e n the scalp leads of l~onnie were r u n to the vertex of D o n n i e two d i s t i n g u i s h a b l e r h y t h m s could be i d e n t i f i e d a n d t h e s e were q u i t e a s y n c h r o n o u s , one a r o u n d 90 b e a t s / m i n , a n d t h e o t h e r a r o u n d 8 3 / m i n . ( f i g . 8).
m e n t s of t h e l e f t leg, s p r e a d i n g in tile course of d e v e l o p m e n t to o t h e r p o r t i o n s of t h e left side a n d f i n a l l y r e v e a l i n g r i g h t - s i d e d clonic seizures. T h e only E E G m a n i f e s t a t i o n s of seizure a c t i v i t y have been conf i n e d to the r i g h t c e n t r a l r e g i o n even w h e n the p a t i e n t was h a v i n g a clonic seizure on t h e i p s i l a t e r a l side. T h i s p h e n o m e n o n h a s been seen b y u s previously in
IAN HAY BROWN
570
a few cases, and of course reported by others (e.g. Penfield and Jasper 1954). The present EEG disturbance coincides very well with the earliest clinical manifestations of the seizure pattern, and this point has been clearly emphasized as being the most important by Penfield. The etiology is a matter of conjecture at the present time although it seems likely that some birth trauma a n d / o r anoxia occurred and that the febrile episodes early in 1957 triggered off the seizure mechanism. However, it should be noted that there is a strong hereditary factor present, for although neither parent has shown convulsive tendencies, there are many members of both sides of the family who have. On the f a t h e r ' s side, an uncle, female cousin and a nephew are known to have convulsions, while two male cousins of the mother are similarly affected. The very nature of the electroencephalographic and clinical phenomena, and the involvement in only one of identical twins suggest that heredity is not the main etiologic agent present. Since the original electroencephalographic examination there have been two further examinations on ]Connie which have been essentially normal. The first of these was prompted by an acute increase in the number of seizures to about 10 a night when the child was placed on phenobarbital, and the second was a follow up after seizures were becoming rather infrequent as a result of Dilantin ® and Tridione ® therapy. The twins are back on circuit with tlie carnival now and latest information indicates the latter combination of medication is proving effective in controlling the seizures. ~ e were interested in the paper by Johnson and Doherty (1957) on the simultaneous E K G recordings on their twins, and the earlier portion of our record seemed to indicate a similarity between the two cases in that the hearts apparently beat in both cases at the same rates. However, the accidental detection of asynchronous rhythms in our case led us to wonder if we in our early observation and Johnson et al. in their case were actually recording impulses from one heart through the chest of the other. This point should be noted by future observers who know more about cardiology than we profess when EKG studies on conjoined twins are conducted~ but thoracopagi present considerable difficulties in this respect and much ingenuity will be required to solve this problem. SUMMARY The history is presented of conjoined Siamese twins, one of whom has developed epileptic seizures. The EEG simultaneous recordings of the twins and a clinical seizure of one twin are presented and dis-
cussed. The chances of directly recording a seizure in an epileptic conjoined twin is estimated as being in the neighborhood of one in 500 million. No attempt has been made in this report to discuss the general problem of conjoined twinning, -heories of development, etc., for which adequate re:,rences exist (Aird 1954, 1955; Dawson 1944; D u n b ~ 1934; Gray et al. 1950; Luckhardt 1941; Mall 19(~; Mortimer et al. 1942; Perlstein et al. 1927; Potter 1952). Acknowledgment is made to I,~. A. Kuhr for the information given on the birth :.~d development of the twins, and to Dr. J. G. Tye f t" copies of X-rays. REFERENCEf AIRD, I. The conjoined twins of I~:ano. Brit. M. J., 1954, 1: 831-837. AIRD, I. Conjoined twins. Med. ~, orld, London, 1955, 83 : 217-221. DAWSON, H. Pygopagus twins; ~cport of dissection of the thorax, abdomen and ]~. lvis. Amer. J. Dis. Child., 1944, 68: 395-404. DUNBAR, A. E. Teratology. A~wr J. Surg., 1934, 24: 152-158. DURR, S. P. Craniopagus twins; report of a case. J.A.M.A., 1952, 150: 93-95. GRAY, C. M., Nix, It. G. and WALI CE, A. J. Thoraeopagus twins; prenatal di~:nosis. Radiology, 1950, 54: 398-400. GROSS]~AN, H. J., SUGAR, O., G];EELEY, P. W. and SADOVE,M. S. Surgical separe! ion in craniopagus. J.A.M.A., 1953, 153: 201-207. ~IOLGATE, J. and IKPE:ME, B. J. Cor~joined twins. Brit. J. Surg., 1956, 43: 626-7. JOHNSON, N. J. and DOHERTY, , E. Simultaneous electrocardiograms in thoraco~,agus twins. A ~ e r . Heart J., 1957, 53: 150-156. LUCKHARDT, A. B. Report of t i e autopsy of the Siamese twins together wit] other interesting information covering their li',,; a sketch of the life of Chang and Eng. Surg. Gynec. Obst., 1941, 72: 116-125. MALL, ]~. P. A study of the ca ~es underlying the origin of human monsters. J. iforp]wl., 1903, 19: 3-368. MORTI~IER, B. and KIRSHBAU)I,J D. Human double monster (so-called Siamese t~ as). Awer. J. Dis. Child., 1942, 64: 697-704. PENFIELD, W. and JASPER, H. Ep.,'epsy and the functional Anatomy of the H u ~ a n Brain. Little, Brown and Co., Boston, 1954. PERLSTEIN, M. A. and LECouNT, S. R. The history and necropsy report of the BoiJemian twins, RosaJosepha Blazek. (Pygopagus t,vins.) Arch. Path., 1927, 3: 171-192. POTTER, E. L. Pathology of the J~ .,etus and the Newborn. Publishers, 1952. SUGAR, O. The Brodie craniops~us twins. Trans. Amer. Neur. Assoc., 78th Meeting, 1953, P. 198-9. WmsoN, It. and STORER,E. H. Sur;~ery in "Siamese ~' twins: a report of three sets of conjoined twins treated surgically. Ann. Surg.. 1957, i45: 718-725.
Reference: BROWN, I. H. Epilepsy in conjoined siamese twins. EEG Clin. Neurophysiol., 1959, 11: 565-570.
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