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Electroencephalography in a case of cerebral vascular malformation
CLINICAL A N D L A B O R A T O R Y NOTES Editor for Western Hemisphere and Far East, R. S. SCHWAB, M.D., Boston, U.S.A. Editor for Europe and Middle East, ~'. BUCHTHAL, M.D., Copenhagen, Denmark ELECTROENCEPHALOGRAPHY
IN A CASE OF CEREBRAL
VASCULAR MALFORMATION (Report of a case) GEORGE M. BURLO, M . D . and STUAaT DANOFF, M . D .
Department o[ Neurosurgery (IV Division) Electroencephalography Laboratory 1 and the Department of Pediatrics, New York University.Bellevue Medical Center, New York (Received for p u b l i c a t i o n : J u n e 7, 1957) (!linical m a n i f e s t a t i o n s of i n t r a e r a n i a l arteriovenous m a l f o r m a t i o n s fire vuried, obviously d e p e n d i n g upon the size a n d location of the a n o m a l y as well as t h e a m o u n t of bleeding. The d i a g n o s i s of this condition is u s u a l l y m a d e on the b a s i s of t h e clinical course a n d a r t e r i o g r a p h y . The following is a r e p o r t of a cqse with provocative e l e c t r o e n e e p h a l o g r a p h i e findings. METHODS E l e c t r o e n c e p h a l o g r a m s were reeorded with a type D O f f n e r m a c h i n e u s i n g 18 electrodes w i t h 10 diff e r e n t m o n t a g e s , 3 of which were c o m m o n referenee a n d 7 series bipol:lr. CASE HISTORY E. M. is an 11V2 y e a r old P u e r t o R i c a n w h i t e male who was p e r f e c t l y well u n t i l M a r c h 8, 1956 w h e n he s u d d e n l y conlplained of a f r o n t a l h e a d a c h e , m a l a i s e , a n o r e x i a a n d a s e n s a t i o n of ~ ' w a r m t h " w i t h v o m i t i n g a f t e r e a t i n g . These s y m p t o m s b e c a m e more severe and a local p h y s i c i a n a d v i s e d h o s p i t a l i z a t i o n because of " n n e m i a " . Itowever, t h e boy w a s kept at home a n d the s y m p t o m s progressed. On M a r c h 10, 1956 a n o t h e r p h y s i c i a n d i a g n o s e d ' ' m e n i n g i t i s ' '. On M a r c h 10, 1956, the p a t i e n t w a s f i r s t seen at Bellevue Hosp i t a l where he was n o t e d to be afebrile, v e r y lethargic, with positive K e r n i g a n d B r u d z i n s k i signs, a rigid neck a n d a mildly i n f l a n m l e d t h r o a t . L u m b a r puncture revealed an initial p r e s s u r e of 230 m m H20, with n o r m a l C. S. F. f i n d i n g s . I n view of these f i n d i n g s , it was felt t h a t the p a t i e n t h a d a mild r i n d u p p e r r e s p i r a t o r y i n f e c t i o n with s e c o n d a r y meningismus. The s i g n s a n d s y m p t o m s became more severe a n d he was a d m i t t e d to Bellevue H o s p i t a l on M a r c h 11, 1956 with a t e m p e r a t u r e of 1 0 0 ° F , pulse 6 4 / m i n . , r e s p i r a t o r y rate 2 0 / m i n . , a n d blood p r e s s u r e 9 6 / 6 0
1 This laboratory is supported by the Sister Elizabeth Kenny Foundation.
nun. t t g . He was m a r k e d l y l e t h a r g i c a n d r e s p o n d e d s l u g g i s h l y to q u e s t i o n i n g a n d p a i n f u l s t i m u l i easily l a p s i n g into l i g h t sleep. H i s speech w a s slurred a n d n a s a l in quality. P o s i t i v e f i n d i n g s on p h y s i c a l exami n a t i o n were a rigid neck, m i n i m a l ptosis of the r i g h t eyelid, d i l a t a t i o n of tile r i g h t pupil, bilateral ankle clonus a n d positive B a b i n s k i ' s , K e r n i g ' s n n d B r u d z i n s k i ' s s i g n s with slight weakness of the left a n n a n d facial nmscles. W h i t e blood cells n u m b e r e d 10.000 with n o r m a l d i f f e r e n t i a l . C. S. F. w a s clear, with an initial pressure of 050 nlm. H oO which rose to 300 ram. It20 on j u g u l a r compression. I t c o n t a i n e d 1 m o n o n u e l e a r cell per ram2, 28 rag. per cent of protein, 80 rag. per cent of s u g a r a n d no b a c t e r i a on culture. On M a r c h 12, 1956 a . m . the ilatient was m u c h more alert a n d r e s p o n d e d readily to q u e s t i o n i n g a n d cx:mfination. The clinical p i c t u r e was changed. M i n i m a l .tthetoid m o v e m e n t s of the left h a n d were now present. T h e r e was no w e a k n e s s a n d no ankle clonus, a n d B a b i n s k i ' s sign p r e s e n t only on the left. Corneal reflexes were also present. I n the a f t e r n o o n of t h e same day the r i g h t ptosis w a s a b s e n t , p u p i l s equal, muscle power equal with p e r s i s t e n t left h a n d t~thetosis a n d questionable left B a b i n s k i a n d m o d e r , t e l y positive K e r n i g a n d B r u d z i n s k i signs. The i m p r e s s i o n at this time was t h a t t h e p a t i e n t h a d either m e n i n g i t i s of n n k n o w n origin, i n t r . , e r n n i a l n e o p l a s m or w~seular a n o m a l y . On M a r c h 13, 1956 t h e r e was no c h a n g e except for the d i s a p p e a r a n c e of the B a b i n s k i sign. W h i t e blood count was 6,950 with a n o r m a l d i f f e r e n t i a l . On M a r c h 15, 1956 the C. S. F. w a s clear with initial p r e s s u r e of 180 ram. H,_,O. It e o n t a i n e d no cells, p r o t e i n 40 nlg. p e r cent, s u g a r 72 m g . per cent a n d no b a c t e r i a on eulture. B y M a r c h 19, 1956 t h e p h y s i c a l e x a m i n a t i o n w a s entirely n o r m a l a n d the p a t i e n t showed a n a l m o s t complete recovery. Two days later, however, he experienced a s u d d e n onset of v o m i t i n g , f r o n t a l headache, e x t r e m e l e t h a r g y w i t h
[ ,"05 1
706
G E O R G E M. B U R L O and S T U A R T D A N O F F
sluggish responses and clouding of the sensorium. Physical e x a m i n a t i o n revealed ptosis of the r i g h t eye, mild weakness of the muscles of the left a r m and face, r i g h t B a b i n s k i ' s sign, no b r u i t s audible, vital signs normal, optic discs n o r m a l on fundoscopic examination. C. S. F. p r e s s u r e was 280 ram. H20; the fluid was markedly xanthochronic b u t sterile with a p r o t e i n of 325 mg. per cent and s u g a r 69 mg. per ~.ent as well as 4 erenated red cells, 21 mononuclear
blood cells, 30 mononuclear cells, 185 ghost cells aa,t 380 mg. per cent of protein. Consultation with a pediatric neurologist on A p r i l 9, 1956 revealed t h a t the child had a left motor weakness and a left h o m o n y m o u s hemianopsia. ]li~ impression was t h a t the p a t i e n t had a r i g h t hemisphere lesion, neoplasm or arteriovenous anomaly probably involving the anterior p a r t of tho optic r:Miatlon i~, the temporal lobe. C
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Fig. 1 A. 1st record. Normal sleep. The minimal asymmetry could be granted no significance. LE, left ear. LLC, left low central, LIIC, left high central, RI~C, right high central, RLC, right low central, RE, right ear, LF, left frontal, RF, right frontal. B. 2nd record. Grossly abnormal, showing a slow wave focus in the right anterior temporal area. X Is 5 cm. inferior to the right anterior temporal lead. C. 3rd record. Normal sleep record. D. 4th record. Normal sleep record, Calibration : 50/zV.
Time-scale : 1 sec. cells. By the next day there was a remarkable imp r o v e m e n t and the p a t i e n t was alert and active with clear sensorium. Neurological examination was entirely n o r m a l except f o r m i n i m a l athetoid movements of the left hand. W h i t e blood count w a s 7,100 with n o r m a l differential. The f i r s t electroencephalogram on ~farch 22, 1956 was n o r m a l (fig. 1 A ) . On March 24, 1956 the C. S. F. p r e s s u r e was 250 ram. H20. The fluid xanthochromic with crenated red
As there had been a clinical change since tht' f i r s t n o r m a l E E G , a repeat was obtained oi1 April 13, 1956 (fig. 1 B ) . The r e p o r t s t a t e d : " T h i s is a grossly abnormM record f o r the age showing a delta slowing focus on the r i g h t a n t e r i o r t e m p o r a l area. This is p r o b a b l y due to some organic condition in the r i g h t hemisphere most likely the posterior f r o n t a l area or tile p a r t of the t e m p o r a l lobe lying j u s t beneath that." '
EEG IN CEREBRAL
VASCULAR
]!he s a m e d a y the C. S. F. p r e s s u r e w a s 130 ram. H,,O. T h e clear sterile f l u i d c o n t a i n e d 4 m o n o n u c l e a r cells/mm.2 p r o t e i n 61 rag. p e r cent, a n d s u g a r 72 rag. per cent. L e f t h o m o n y m o u s h e m i a n o p s i a p e r s i s t e d d u r i n g hospitalization. A p n e u m o e n e e p h a l o g r a m perf o r m e d on A p r i l 19, 1956 was normal. E E G ' s on M a y
MALFORMATION
707
t r a c i n g of the series t h a t n o t e w o r t h y f i n d i n g s were electrical a n d clinical changes. Once this accident m a l f o r m a t i o n was responsible for the c o n c o m i t t a n t electrical a n d clinical changes. Once this accident resolved, the p a t i e n t improved both clinic~flly a n d electrically.
Fig. 2 Angiogram showing a vascular nlalformation on the right middle cerebral artery.
l, 1956 ( f i g . 1 C) a n d on M a y 17, 1956 (fig. I D) were n o r m a l . I t w a s specially n o t e d t h a t the slow wave f o c u s w a s n o t seen in t h e s e t r a c i n g s . A n artcriog r a m on J u n e 6, 1956 revealed a n a r t e r i o v e n o u s anom a l y in t h e r i g h t t e m p o r a l region ( f i g . 2) b u t b e c a u s e of its inaccessible location s u r g e r y w a s n o t advisable. DISCUSSION T h i s case e m p h a s i z e s the i m p o r t a n c e of serial e h . c t r o e n c e p h a l o g r a p h y . It was only on t h e second
SUMMARY I. A case is reported of a n 111/2 y e a r ohl boy with ~m i n t r a c e r e b r a l v a s c u l a r n m l f o r m a t i o n on whom 4 serial e l e c t r o e n c e p h a l o g r a m s were recorded. 2. One of these recordings~ o b t a i n e d at t h e h e i g h t of an e x a c e r b a t i o n of s y m p t o m s helped to localize t h e lesion; the c h a n g e s in the electroencepha l o g r a m s paralleled the clinic',l course a n d yielded useful information. 3. T h e advant:~gc of s(!rial E E G ' s is ('mphaslzed.
Reference: BURLO, G. ~I. a n d DANOFF, S. E l e c t r o e n e e p h a l o g r a p h y in a case of cerebral v a s c u l a r m a l f o r m a t i o n . EEG Clin. Neurophysiol., 1957, 9: 705-707.
IN A CASE OF CEREBRAL
VASCULAR MALFORMATION (Report of a case) GEORGE M. BURLO, M . D . and STUAaT DANOFF, M . D .
Department o[ Neurosurgery (IV Division) Electroencephalography Laboratory 1 and the Department of Pediatrics, New York University.Bellevue Medical Center, New York (Received for p u b l i c a t i o n : J u n e 7, 1957) (!linical m a n i f e s t a t i o n s of i n t r a e r a n i a l arteriovenous m a l f o r m a t i o n s fire vuried, obviously d e p e n d i n g upon the size a n d location of the a n o m a l y as well as t h e a m o u n t of bleeding. The d i a g n o s i s of this condition is u s u a l l y m a d e on the b a s i s of t h e clinical course a n d a r t e r i o g r a p h y . The following is a r e p o r t of a cqse with provocative e l e c t r o e n e e p h a l o g r a p h i e findings. METHODS E l e c t r o e n c e p h a l o g r a m s were reeorded with a type D O f f n e r m a c h i n e u s i n g 18 electrodes w i t h 10 diff e r e n t m o n t a g e s , 3 of which were c o m m o n referenee a n d 7 series bipol:lr. CASE HISTORY E. M. is an 11V2 y e a r old P u e r t o R i c a n w h i t e male who was p e r f e c t l y well u n t i l M a r c h 8, 1956 w h e n he s u d d e n l y conlplained of a f r o n t a l h e a d a c h e , m a l a i s e , a n o r e x i a a n d a s e n s a t i o n of ~ ' w a r m t h " w i t h v o m i t i n g a f t e r e a t i n g . These s y m p t o m s b e c a m e more severe and a local p h y s i c i a n a d v i s e d h o s p i t a l i z a t i o n because of " n n e m i a " . Itowever, t h e boy w a s kept at home a n d the s y m p t o m s progressed. On M a r c h 10, 1956 a n o t h e r p h y s i c i a n d i a g n o s e d ' ' m e n i n g i t i s ' '. On M a r c h 10, 1956, the p a t i e n t w a s f i r s t seen at Bellevue Hosp i t a l where he was n o t e d to be afebrile, v e r y lethargic, with positive K e r n i g a n d B r u d z i n s k i signs, a rigid neck a n d a mildly i n f l a n m l e d t h r o a t . L u m b a r puncture revealed an initial p r e s s u r e of 230 m m H20, with n o r m a l C. S. F. f i n d i n g s . I n view of these f i n d i n g s , it was felt t h a t the p a t i e n t h a d a mild r i n d u p p e r r e s p i r a t o r y i n f e c t i o n with s e c o n d a r y meningismus. The s i g n s a n d s y m p t o m s became more severe a n d he was a d m i t t e d to Bellevue H o s p i t a l on M a r c h 11, 1956 with a t e m p e r a t u r e of 1 0 0 ° F , pulse 6 4 / m i n . , r e s p i r a t o r y rate 2 0 / m i n . , a n d blood p r e s s u r e 9 6 / 6 0
1 This laboratory is supported by the Sister Elizabeth Kenny Foundation.
nun. t t g . He was m a r k e d l y l e t h a r g i c a n d r e s p o n d e d s l u g g i s h l y to q u e s t i o n i n g a n d p a i n f u l s t i m u l i easily l a p s i n g into l i g h t sleep. H i s speech w a s slurred a n d n a s a l in quality. P o s i t i v e f i n d i n g s on p h y s i c a l exami n a t i o n were a rigid neck, m i n i m a l ptosis of the r i g h t eyelid, d i l a t a t i o n of tile r i g h t pupil, bilateral ankle clonus a n d positive B a b i n s k i ' s , K e r n i g ' s n n d B r u d z i n s k i ' s s i g n s with slight weakness of the left a n n a n d facial nmscles. W h i t e blood cells n u m b e r e d 10.000 with n o r m a l d i f f e r e n t i a l . C. S. F. w a s clear, with an initial pressure of 050 nlm. H oO which rose to 300 ram. It20 on j u g u l a r compression. I t c o n t a i n e d 1 m o n o n u e l e a r cell per ram2, 28 rag. per cent of protein, 80 rag. per cent of s u g a r a n d no b a c t e r i a on culture. On M a r c h 12, 1956 a . m . the ilatient was m u c h more alert a n d r e s p o n d e d readily to q u e s t i o n i n g a n d cx:mfination. The clinical p i c t u r e was changed. M i n i m a l .tthetoid m o v e m e n t s of the left h a n d were now present. T h e r e was no w e a k n e s s a n d no ankle clonus, a n d B a b i n s k i ' s sign p r e s e n t only on the left. Corneal reflexes were also present. I n the a f t e r n o o n of t h e same day the r i g h t ptosis w a s a b s e n t , p u p i l s equal, muscle power equal with p e r s i s t e n t left h a n d t~thetosis a n d questionable left B a b i n s k i a n d m o d e r , t e l y positive K e r n i g a n d B r u d z i n s k i signs. The i m p r e s s i o n at this time was t h a t t h e p a t i e n t h a d either m e n i n g i t i s of n n k n o w n origin, i n t r . , e r n n i a l n e o p l a s m or w~seular a n o m a l y . On M a r c h 13, 1956 t h e r e was no c h a n g e except for the d i s a p p e a r a n c e of the B a b i n s k i sign. W h i t e blood count was 6,950 with a n o r m a l d i f f e r e n t i a l . On M a r c h 15, 1956 the C. S. F. w a s clear with initial p r e s s u r e of 180 ram. H,_,O. It e o n t a i n e d no cells, p r o t e i n 40 nlg. p e r cent, s u g a r 72 m g . per cent a n d no b a c t e r i a on eulture. B y M a r c h 19, 1956 t h e p h y s i c a l e x a m i n a t i o n w a s entirely n o r m a l a n d the p a t i e n t showed a n a l m o s t complete recovery. Two days later, however, he experienced a s u d d e n onset of v o m i t i n g , f r o n t a l headache, e x t r e m e l e t h a r g y w i t h
[ ,"05 1
706
G E O R G E M. B U R L O and S T U A R T D A N O F F
sluggish responses and clouding of the sensorium. Physical e x a m i n a t i o n revealed ptosis of the r i g h t eye, mild weakness of the muscles of the left a r m and face, r i g h t B a b i n s k i ' s sign, no b r u i t s audible, vital signs normal, optic discs n o r m a l on fundoscopic examination. C. S. F. p r e s s u r e was 280 ram. H20; the fluid was markedly xanthochronic b u t sterile with a p r o t e i n of 325 mg. per cent and s u g a r 69 mg. per ~.ent as well as 4 erenated red cells, 21 mononuclear
blood cells, 30 mononuclear cells, 185 ghost cells aa,t 380 mg. per cent of protein. Consultation with a pediatric neurologist on A p r i l 9, 1956 revealed t h a t the child had a left motor weakness and a left h o m o n y m o u s hemianopsia. ]li~ impression was t h a t the p a t i e n t had a r i g h t hemisphere lesion, neoplasm or arteriovenous anomaly probably involving the anterior p a r t of tho optic r:Miatlon i~, the temporal lobe. C
LE
- L LC~
"
L LC -
L
H
RHC-
R L C
~
-
~
C
,
~
~
-
A$LEEP LE
~-;
~
~
~
- LA1
LAT-LPF
.........
LPF-
RPF --~
. . . . . . . . . . .
RPF
- RAT ~'~'~'~
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. . . . . . . . .
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Fig. 1 A. 1st record. Normal sleep. The minimal asymmetry could be granted no significance. LE, left ear. LLC, left low central, LIIC, left high central, RI~C, right high central, RLC, right low central, RE, right ear, LF, left frontal, RF, right frontal. B. 2nd record. Grossly abnormal, showing a slow wave focus in the right anterior temporal area. X Is 5 cm. inferior to the right anterior temporal lead. C. 3rd record. Normal sleep record. D. 4th record. Normal sleep record, Calibration : 50/zV.
Time-scale : 1 sec. cells. By the next day there was a remarkable imp r o v e m e n t and the p a t i e n t was alert and active with clear sensorium. Neurological examination was entirely n o r m a l except f o r m i n i m a l athetoid movements of the left hand. W h i t e blood count w a s 7,100 with n o r m a l differential. The f i r s t electroencephalogram on ~farch 22, 1956 was n o r m a l (fig. 1 A ) . On March 24, 1956 the C. S. F. p r e s s u r e was 250 ram. H20. The fluid xanthochromic with crenated red
As there had been a clinical change since tht' f i r s t n o r m a l E E G , a repeat was obtained oi1 April 13, 1956 (fig. 1 B ) . The r e p o r t s t a t e d : " T h i s is a grossly abnormM record f o r the age showing a delta slowing focus on the r i g h t a n t e r i o r t e m p o r a l area. This is p r o b a b l y due to some organic condition in the r i g h t hemisphere most likely the posterior f r o n t a l area or tile p a r t of the t e m p o r a l lobe lying j u s t beneath that." '
EEG IN CEREBRAL
VASCULAR
]!he s a m e d a y the C. S. F. p r e s s u r e w a s 130 ram. H,,O. T h e clear sterile f l u i d c o n t a i n e d 4 m o n o n u c l e a r cells/mm.2 p r o t e i n 61 rag. p e r cent, a n d s u g a r 72 rag. per cent. L e f t h o m o n y m o u s h e m i a n o p s i a p e r s i s t e d d u r i n g hospitalization. A p n e u m o e n e e p h a l o g r a m perf o r m e d on A p r i l 19, 1956 was normal. E E G ' s on M a y
MALFORMATION
707
t r a c i n g of the series t h a t n o t e w o r t h y f i n d i n g s were electrical a n d clinical changes. Once this accident m a l f o r m a t i o n was responsible for the c o n c o m i t t a n t electrical a n d clinical changes. Once this accident resolved, the p a t i e n t improved both clinic~flly a n d electrically.
Fig. 2 Angiogram showing a vascular nlalformation on the right middle cerebral artery.
l, 1956 ( f i g . 1 C) a n d on M a y 17, 1956 (fig. I D) were n o r m a l . I t w a s specially n o t e d t h a t the slow wave f o c u s w a s n o t seen in t h e s e t r a c i n g s . A n artcriog r a m on J u n e 6, 1956 revealed a n a r t e r i o v e n o u s anom a l y in t h e r i g h t t e m p o r a l region ( f i g . 2) b u t b e c a u s e of its inaccessible location s u r g e r y w a s n o t advisable. DISCUSSION T h i s case e m p h a s i z e s the i m p o r t a n c e of serial e h . c t r o e n c e p h a l o g r a p h y . It was only on t h e second
SUMMARY I. A case is reported of a n 111/2 y e a r ohl boy with ~m i n t r a c e r e b r a l v a s c u l a r n m l f o r m a t i o n on whom 4 serial e l e c t r o e n c e p h a l o g r a m s were recorded. 2. One of these recordings~ o b t a i n e d at t h e h e i g h t of an e x a c e r b a t i o n of s y m p t o m s helped to localize t h e lesion; the c h a n g e s in the electroencepha l o g r a m s paralleled the clinic',l course a n d yielded useful information. 3. T h e advant:~gc of s(!rial E E G ' s is ('mphaslzed.
Reference: BURLO, G. ~I. a n d DANOFF, S. E l e c t r o e n e e p h a l o g r a p h y in a case of cerebral v a s c u l a r m a l f o r m a t i o n . EEG Clin. Neurophysiol., 1957, 9: 705-707.