- Email: [email protected]
P4 The value of the brain mapping method in the primary generalized epileptic seizures
38s
Communications affichdes/Posters
P3 ELECTRODIAGNOSTIC STUDY OF LAFORA'S DISEASE A. ¥ u s t a , S. A n t o n * , S e r v i c e s of N e u r o l o g y a n d *Clinical Electrophysiology. C l i n i c a P u e r t a de H i e r r o ; S a n M a r t i n d e P o r r e s , 4; 2 8 0 3 5 M a d r i d , Spain
P4 THE VALUE OF THE BRAIN MAPPING METHOD PRIMARY G E N E R A L I Z E D J i Miszczak
and E. Mempel*
Aviational Medicine, Oepartment
L a f o r a ' s d i s e a s e is n o w a w e l l - d e f i n e d c l i n i cal a n d p a t h o l o g i c a l d i s o r d e r . We h a v e s t u d i e d 4 c a s e s c o n f i r m e d b y c e r e b r a l b i o p s y in w h o m s e r i a l E E G s t u d i e s w e r e c a r r i e d out a n d r e l a t e d t o t h e c l i n i c a l e v o l u t i o n of t h e p a t i e n t s . The family m e m b e r s of t w o s i b l i n g s w h o w e r e b o t h a f f e c t e d b y t h e d i s e a s e w e r e a l s o s u b j e c t e d to s t u d y . T h e r e s u l t s a l l o w us to e s t a b l i s h e l e c t r o c l i n i c a l c r i t e r i a f o r t h e d i f f e r e n t s t a g e s of L a fora's disease. In t h e i n i t i a l s t a g e , l a s t i n g a r o u n d 12 t o 18 m o n t h s , the b a c k g r o u n d c e r e b r a l b i o e l e c t r i c a c t i v i t y w a s f o u n d to b e c o n s e r v e d , w i t h g e n e r a l i z e d d i s c h a r g e s of s h a r p e l e m e n t s , s i m i l a r t o t h o s e a p p e a r i n g in p r i m a r y g e n e r a l i z e d epilepsy. In t h e e v o l u t i o n a r y p e r i o d , slow, p r e d o m i n a n t l y p o s t e r i o r waves appear, a c c o m p a n i e d by irregular, multifocal paroxysmal discharges, pred o m i n a n t l y of t h e s a m e l o c a l i z a t i o n , w h i c h w e r e intensely induced by light stimulus. In t h e t e r m i n a l p e r i o d , d i f f u s e , n o n r e a c t i v e theta and delta activities become evident and the alpha rhythm disappears. In an a s y m p t o m a t i c m e m b e r of t h e f a m i l y s t u d ied, p a r o x y s m a l d i s c h a r g e s , r e l a t e d to c r y i n g , w e r e found.
EPILEPTIC
Warsaw,
of Polish Academy
The brain m a p p i n g
of Sciences,
method was applied
patients with g e n e r a l i z e d
epileptic
clinical picture.
The aim of this
was to find out the advantages
in seizures
described by e l e c t r o e n c e p h a l o g r a p h i c
and
investigation
of this method
as compared with the standard
eeg technique.
The recordings were performed
on the selected
group of twenty patients with generalized epileptic patients).
seizures:
GM (IO patients)
three m e a s u r i n g
systems:
and Oantec-Coneerto. using at least results
Brainimager
The
that the brain mapping method
brings up several
important
information which
of standard
eeg
In some cases of generalized
seizures
the triggering
Neuroscan,
Each patient was examined
two of these systems.
indicate
epileptic
and PM (lO
The brain mapping was done wi±h
investigations,
ELECTROCLINICAL ICTAL SYMPTOMATOLOGY IN LENNOX-GASTAUT SYNDROME. Yauaq~bB, Waheed G, Daif AK, Tahan AR, Obeid T., Departments of Neurology and NeuroohysioloFy, Kin~ Khalid University ~ospital, Riyadh 11472, Saudi Arabia. We analysed the electroclinical ictal symptomatology of soecific seizures in 21 oatients with Lennox-Gastaut Syndrome. 100 seizures were observed durin~ the video split long recording. The seizures were those of atypical absences, myoclonias and atonic seizures, and tonic seizures as their frequency of oecurance. The tonic seizures were the most difficult to appreciate clinically and were of short duration and manifested as upward ~aze of the eyeballs, tonic backward retraction of the neck and clinching of the teeth. No automatism occurred and these seizures were very brief to appreciate any loss of consciousness. Loss of muscular tone was seen in some patients. The ictal EEG showed bilateral medium voltage (I00 uV) fast rhythm at 9-12 c/see and duration of 15 seconds or less. Myoclonias were variable involvin~ the limbs, trunk, neck or eyelids. Some were brief and rapid while others were massive myoclonlas. The EEG showed discharges of spikes and wave at 3-5 c/sec of short duration. Atonic seizures, though sometimes were difficult to differentlate from those of myoclonias clinically, the EEG discharges were different and showed slow wave with or without spikes at a frequency of less than 3 c/see. The duration of dis~ char~es were longer than those of myoclonias. Atypical absences manifested alone or were accompanied by loss of muscular tone, gestural automatism or vertical nystagmus of the eyes. The EEG discharges were variable. In some patients, the discharges were irregular, diffuse slow spike waves 2-2.5 c/see with fragmentation; during this fragmentation consciousness was regained. In patients with slow discharges of spike wave less than 2 c/see, consciousness was maintained. In two patients, the discharges were those of fast activity at 9-12 Hz lasting 15 sees. In other patients, the discharRes during the attack were combination of flattening, fast rhythm and slow spike waves. Some of our observations during the absences have not been described before in literature.
of
*Neurosurgical
Poland
widen the i n t e r p r e t a t i o n
P5
Institute
Warsaw,
IN THE
SEIZURES
it was possible
zone of epileptic
to follow the direction the brain hemispheres.
to show up
activity
and
of their propagation
in
P6 MISDIAGNOSED LENNOX-GASTAUT SYNDROME. Waheed G, Daif AK Yaqub B. Neurology and Neurophyslology Divisions, King Khalid University Hospital, P.O. BOX 7805(38), Riyadh 11472 Saudl Arabia. We present Ictal electroclinical symptomatology as observed by VEEG of three patients which were referred as LennoxGastaut syndrome. The first patient is 3 years old boy. He h as axial atonla ineludin~ the neck, as the only type of seizure in long video monitoring. His Ictal EEG showed high generalised irregular delta waves 2-2.5 c/see, lasting for 2-4 seconds. The ictal seizure and EEG discharges were stereotyped in all the attacks. Level of consciousness could not be determined The second patient is 6 years old, he has slow atonia involving only the neck and the eyelid with atypical absence~ His ictal EEG showed generalised high voltage discharges of delta adtivlty 2-2.5 c/see, sometimes intermixed with low voltage spikes lasting for 4-6 sees. All electroclinlcal seizures were stereotyped. These 2 patients have myoclonic~stat~c epilepsy of early childhood. The third patient was 17 years old with seizure starting at age of 6 years refrred as atypical absence, GTC~ and partial seizures. Long recording in awake and sleep state did not show any clinical seizure, however, her EEG showed in the awake state, high voltage slow spike wave discharges 2-3 c/see for 25 seconds without any clinical manifestations. These discharges were only seen when the eyes were closed, opening the eyes abolished the discharges. During sleep, there was continuous bilateral diffuse slow spike wave 2-2.5 c/see persistinR throughout the non REM stage. The spike index ran~ed from 90-95%. During the REM stage, the electrical status disappeared leading to 35Z spike wave index. This oatient had all the criteria for continuous spike waves during sleep. The above patients will be shown and the differentiation of these syndromes wlth Lennox-Gastaut syndrome will be elaborated.
Communications affichdes/Posters
P3 ELECTRODIAGNOSTIC STUDY OF LAFORA'S DISEASE A. ¥ u s t a , S. A n t o n * , S e r v i c e s of N e u r o l o g y a n d *Clinical Electrophysiology. C l i n i c a P u e r t a de H i e r r o ; S a n M a r t i n d e P o r r e s , 4; 2 8 0 3 5 M a d r i d , Spain
P4 THE VALUE OF THE BRAIN MAPPING METHOD PRIMARY G E N E R A L I Z E D J i Miszczak
and E. Mempel*
Aviational Medicine, Oepartment
L a f o r a ' s d i s e a s e is n o w a w e l l - d e f i n e d c l i n i cal a n d p a t h o l o g i c a l d i s o r d e r . We h a v e s t u d i e d 4 c a s e s c o n f i r m e d b y c e r e b r a l b i o p s y in w h o m s e r i a l E E G s t u d i e s w e r e c a r r i e d out a n d r e l a t e d t o t h e c l i n i c a l e v o l u t i o n of t h e p a t i e n t s . The family m e m b e r s of t w o s i b l i n g s w h o w e r e b o t h a f f e c t e d b y t h e d i s e a s e w e r e a l s o s u b j e c t e d to s t u d y . T h e r e s u l t s a l l o w us to e s t a b l i s h e l e c t r o c l i n i c a l c r i t e r i a f o r t h e d i f f e r e n t s t a g e s of L a fora's disease. In t h e i n i t i a l s t a g e , l a s t i n g a r o u n d 12 t o 18 m o n t h s , the b a c k g r o u n d c e r e b r a l b i o e l e c t r i c a c t i v i t y w a s f o u n d to b e c o n s e r v e d , w i t h g e n e r a l i z e d d i s c h a r g e s of s h a r p e l e m e n t s , s i m i l a r t o t h o s e a p p e a r i n g in p r i m a r y g e n e r a l i z e d epilepsy. In t h e e v o l u t i o n a r y p e r i o d , slow, p r e d o m i n a n t l y p o s t e r i o r waves appear, a c c o m p a n i e d by irregular, multifocal paroxysmal discharges, pred o m i n a n t l y of t h e s a m e l o c a l i z a t i o n , w h i c h w e r e intensely induced by light stimulus. In t h e t e r m i n a l p e r i o d , d i f f u s e , n o n r e a c t i v e theta and delta activities become evident and the alpha rhythm disappears. In an a s y m p t o m a t i c m e m b e r of t h e f a m i l y s t u d ied, p a r o x y s m a l d i s c h a r g e s , r e l a t e d to c r y i n g , w e r e found.
EPILEPTIC
Warsaw,
of Polish Academy
The brain m a p p i n g
of Sciences,
method was applied
patients with g e n e r a l i z e d
epileptic
clinical picture.
The aim of this
was to find out the advantages
in seizures
described by e l e c t r o e n c e p h a l o g r a p h i c
and
investigation
of this method
as compared with the standard
eeg technique.
The recordings were performed
on the selected
group of twenty patients with generalized epileptic patients).
seizures:
GM (IO patients)
three m e a s u r i n g
systems:
and Oantec-Coneerto. using at least results
Brainimager
The
that the brain mapping method
brings up several
important
information which
of standard
eeg
In some cases of generalized
seizures
the triggering
Neuroscan,
Each patient was examined
two of these systems.
indicate
epileptic
and PM (lO
The brain mapping was done wi±h
investigations,
ELECTROCLINICAL ICTAL SYMPTOMATOLOGY IN LENNOX-GASTAUT SYNDROME. Yauaq~bB, Waheed G, Daif AK, Tahan AR, Obeid T., Departments of Neurology and NeuroohysioloFy, Kin~ Khalid University ~ospital, Riyadh 11472, Saudi Arabia. We analysed the electroclinical ictal symptomatology of soecific seizures in 21 oatients with Lennox-Gastaut Syndrome. 100 seizures were observed durin~ the video split long recording. The seizures were those of atypical absences, myoclonias and atonic seizures, and tonic seizures as their frequency of oecurance. The tonic seizures were the most difficult to appreciate clinically and were of short duration and manifested as upward ~aze of the eyeballs, tonic backward retraction of the neck and clinching of the teeth. No automatism occurred and these seizures were very brief to appreciate any loss of consciousness. Loss of muscular tone was seen in some patients. The ictal EEG showed bilateral medium voltage (I00 uV) fast rhythm at 9-12 c/see and duration of 15 seconds or less. Myoclonias were variable involvin~ the limbs, trunk, neck or eyelids. Some were brief and rapid while others were massive myoclonlas. The EEG showed discharges of spikes and wave at 3-5 c/sec of short duration. Atonic seizures, though sometimes were difficult to differentlate from those of myoclonias clinically, the EEG discharges were different and showed slow wave with or without spikes at a frequency of less than 3 c/see. The duration of dis~ char~es were longer than those of myoclonias. Atypical absences manifested alone or were accompanied by loss of muscular tone, gestural automatism or vertical nystagmus of the eyes. The EEG discharges were variable. In some patients, the discharges were irregular, diffuse slow spike waves 2-2.5 c/see with fragmentation; during this fragmentation consciousness was regained. In patients with slow discharges of spike wave less than 2 c/see, consciousness was maintained. In two patients, the discharges were those of fast activity at 9-12 Hz lasting 15 sees. In other patients, the discharRes during the attack were combination of flattening, fast rhythm and slow spike waves. Some of our observations during the absences have not been described before in literature.
of
*Neurosurgical
Poland
widen the i n t e r p r e t a t i o n
P5
Institute
Warsaw,
IN THE
SEIZURES
it was possible
zone of epileptic
to follow the direction the brain hemispheres.
to show up
activity
and
of their propagation
in
P6 MISDIAGNOSED LENNOX-GASTAUT SYNDROME. Waheed G, Daif AK Yaqub B. Neurology and Neurophyslology Divisions, King Khalid University Hospital, P.O. BOX 7805(38), Riyadh 11472 Saudl Arabia. We present Ictal electroclinical symptomatology as observed by VEEG of three patients which were referred as LennoxGastaut syndrome. The first patient is 3 years old boy. He h as axial atonla ineludin~ the neck, as the only type of seizure in long video monitoring. His Ictal EEG showed high generalised irregular delta waves 2-2.5 c/see, lasting for 2-4 seconds. The ictal seizure and EEG discharges were stereotyped in all the attacks. Level of consciousness could not be determined The second patient is 6 years old, he has slow atonia involving only the neck and the eyelid with atypical absence~ His ictal EEG showed generalised high voltage discharges of delta adtivlty 2-2.5 c/see, sometimes intermixed with low voltage spikes lasting for 4-6 sees. All electroclinlcal seizures were stereotyped. These 2 patients have myoclonic~stat~c epilepsy of early childhood. The third patient was 17 years old with seizure starting at age of 6 years refrred as atypical absence, GTC~ and partial seizures. Long recording in awake and sleep state did not show any clinical seizure, however, her EEG showed in the awake state, high voltage slow spike wave discharges 2-3 c/see for 25 seconds without any clinical manifestations. These discharges were only seen when the eyes were closed, opening the eyes abolished the discharges. During sleep, there was continuous bilateral diffuse slow spike wave 2-2.5 c/see persistinR throughout the non REM stage. The spike index ran~ed from 90-95%. During the REM stage, the electrical status disappeared leading to 35Z spike wave index. This oatient had all the criteria for continuous spike waves during sleep. The above patients will be shown and the differentiation of these syndromes wlth Lennox-Gastaut syndrome will be elaborated.