Neurophysiol Clin (1992) 22, 281-286

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© Elsevier, Paris

Memoir

Electrical status e p i l e p t i c u s o n eye c l o s u r e : a case report P Veggiotti, M Viri, G Lanzi Divisione di Neuropsichiatria Infantile, Fondazione "'Istituto Neurologico C Mondino'; Via Palestro 3, 27100 Pavia, Italy

(Received 2 September 1991; accepted 25 January 1992)

Summary - The authors present the clinical case of a boy manifesting eyelid myocloniason eye closure. The corresponding electroencephalographic recording was characterized by unceasing spike activity, constituting a sort of electrical status epilepticus. There was no loss of consciousness or differences between results of neuropsychologicaltests with eyes open and closed. eye closure / eyelid myocionias / spike-wave activity / electrical status epilepticus / neuropsychological tests

R~sumd - l~tat de mal ~lectroenc~phalographique ~ la fermeture des yeux: presentation d'un cas clinique. Les auteurs ddcrivent le cas clinique d'un enfant pr~sentant des myoclonies palp~brales ~ la fermeture des yeux. L "EEG correspondant, est caract6ris6 par une activit~ de PO continue, repr&entant une sorte d'~tat de real ~lectroenc~phalographique. ll n 'y a pas perte de eonnaissance, et les Opreuves neuropsychologiques effeetu6es avee les yeux ouverts ou ferm~s, ne pr~sentent pas de differences significatives. fermeture des yeux / ~tat de mal ~pileptique / ~preuve neuropsychologiques / pointes-ondes

Introduction

Paroxystic E E G abnormalities and induction of epileptic seizures on eye closure have been recognized for years (Green, 1968; Vignaendra et al, 1976; De Marco e t al, 1981; Aguglia et al, 1985; Rafal et al, 1986; Gobbi e t al, 1989; Cooper and Soo, 1991). Gastaut and Tassinari in 1966 had already demonstrated the induction of eyelid myoclonia and absence on eye closure with corresponding E E G recordings of generalized discharges. These clinical episodes were often self-induced by patients with severe mental retardation (Darby et al, 1980; Watanabe e t al, 1985). Subsequently, other authors demonstrated that blinking, inhibition of central vision, complete darkness and photic stimulation induce eyelid myoclonia with

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absence a n d b o t h generalized a n d p o s t e r i o r E E G a b n o r m a l i t i e s ( J e a v o n s , 1982; P a n a y i o t o p o u l o s , 1987). W e r e p o r t the clinical case o f a b o y in which eyelid closure i n d u c e d eyelid m y o c l o n i a w i t h o u t absence o f a n d with unceasing epileptic discharges, c o n s t i t u t i n g a t y p e o f electrical status epilepticus.

Patient and methods

Case report Birth was uncomplicated following a normal pregnancy. Development was normal and the patient had a regular scholastic record. Family history was not positive for epilepsy. The first episode, at age 11 years, during spontaneous sleep, manifested with a guttural noise followed by generalized tonic-clonic seizure lasting about 1 min. Therapy was begun with sodium valproate, but the patient did not take medication regularly. Carbamazepine, at a daily dose of 600 rag, was added after occurrence of four new episodes similar to the first. Numerous EEGs performed during the same period revealed the presence of generalized spike-waves and poly-spike waves. Phenobarbital was later added to the therapy at a daily dose of 100 mg. However, four other clinical episodes occurred which were identical to those which had occurred previously. The patient was admitted to our division at age 12 years. Examinations performed (haematological, neuroradiological and evoked potential studies) were all found to be normal. WISC R mental evaluation revealed an IQ of 83 (verbal score 88; performance 80) with a slight drop in arithmetic and block design. Language study was normal. Therapy with sodium valproate (1200 mg daily) was administered. No clinical episode occurred in the following 6 months up to the time of the present report in successive controls, despite unaltered epileptic abnormalities.

Clinical, E E G and neuropsychological aspects EEGs were performed in our division during two distinct periods in November 1990 and March 1991. After a latent period lasting between 0.5 and 2 s, eye closure induced eyelid myoclonia (eyes remained semi-closed) associated with upward rotation of eyeballs without suspension of contact. These clinical episodes were never self-induced. Corresponding EEG activity was characterized by diffuse high voltage spikes, atypical polyspike waves and sharp waves on all derivations, which disappeared immediately on eye opening (fig 1). Only sporadic abnormalities of generalized spike-waves were present with eyes open. During eye closure, paroxystic activity was interrupted by brief periods during which there was background alpha band activity. However, the abnormalities always occupied a variable duration of 85-100% of the time the patients had eyes closed; the time at which the latter was observed also varied from day to day. Prolonged eye closure (up to 45 min) does not lead to termination of discharges. In fact,

Epileptic status on eye closure

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Fig 1. Awake: A, eyes opened; V, eyes closed.

frequency of the latter tends to increase with time, without any influence on consciousness. Although eyelid myoclonias are always present, their frequency is variable. More accurate evaluation of consciousness and difference in neuropsychological performance was achieved by a series of tests on the lack of epileptic activity (table I) performed both with eyes closed and opened with no significant difference in results. Tests did not reveal any modification in quality or quantity of epileptic abnormalities.

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284 Table I. Neuropsychological tests. Sensory path way

Stereognosis Grapghostesia Digital gnosis Baresthesia Pallesthesia Spatial discrimination

Short-term memory

Digit spain Repetition of words pairs Repetition of short phrases

EEG during spontaneous sleep was physiological without evidence of paroxystic activity. The patient was submitted to traditional and specific activation techniques to detect inducing factors. Hyperpnea induced a slight increase in percentage of abnormalities while SLI, with eyes open, did not induce any paroxystic activity. Blinking (spontaneous, voluntary or induced by corneal reflex) always induced brief appearances of poly-spike waves. Abnormalities were interrupted for a few seconds by a sudden unexpected sound of varying nature. However, adaptation to the same noise (after 2-3 times), no longer blocked pathologic activity. Intravenous administration of diazepam (1 mg) completely abolished abnormalities with eyes closed, which initially reappared, with a lower frequency, after about 3 h. Activation techniques proposed by Panayiotopoulos (1987) were also performed during two distinct periods : under basal conditions and after bilateral application of an ocular blepharostat of Barraquer to impede involuntary blinking reflex.

Results The results indicated that : 1), if central vision is abolished (by application of a band over glasses or underwater mask) paroxystic abrtormalities d o not occur; 2), voluntary ocular movements do not induce electroencephalographic abnormalities; 3), after a latency period of 1 0 - 2 0 s, absolute darkness with eyes open induces electroencephalographic abnormalities with same morphology and distribution but with lower frequency than those induced by eye closure tending to decrease With prolonged EEG recording in total darkness. These also occur with application of a Barraquer blepharostat.

Discussion and conclusion Comparison of the present case with subjects previously described in the literature revealed several significant differences.

Epileptic status on eye closure

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Clinical aspect The most relevant clinical aspect was that our patient never suffered from complete or incomplete loss of consciousness, despite presence of epileptic abnormalities with concomitant eyelid myoclonia. Moreover, the lack of differences in results of neuropsychological tests performed by our patient with eye open and closed is in partial disagreement with recent reports (Kastelejn et al, 1990; Brinciotti et al, 1989). Although our patient's intellectual level was within normal limits, there was a drop in some WISC subtest scores (especially arithmetic and block design), indicating a defect in abstract skills with a scarce perceptional organization together with difficulty in concentration. In addition, there was no history of self-induction of the seizures, as often reported by other authors. Finally, we consider that it is important to emphasize that the epileptic abnormalities completely disappeared during spontaneous sleep and immediately reappeared upon awakening in conjunction with eye closure. Neurophysiological data The most relevant aspect is that the abnormalities did not terminate and remained unvaried in quality and quantity during eye closure, resembling electrical status epilep-ticus that only disappeared with intravenous administration of diazepam. The various activation techniques performed led us to the following conclusions regarding the ethiopathogenesis of this case: the absence of photosensitivity is in contrast with the classic description of eyelid myoclonia; meanwhile, the negative results in the trials proposed by Panayiotopoulos (1987) exclude the possibility that our patient could come under the category of what this author defines as 'fixation off sensitive' (FOS) epilepsy. Various authors have hypothesized that many pathogenetic mechanisms are involved in subjects with epileptic abnormalities on eye closure including proprioceptive input from orbicular muscles (Vignaendra et al, 1976), eyeball rotation (Shanzer et al, 1965), or, finally, the suppression of retinal illumination (Green, 1968 ; Lewis, 1972). Both voluntary and forced blinking provoked epileptic abnormalities in our patients together with eyelid myoclonia. However, absolute darkness, even after application of an ocular Barraquer blepharostat which impedes blinking, causes the above-mentioned abnormalities, suggesting a diagnosis of scoto-sensitivity in our patient. Given the complexity of the case presently described, we are of the opinion that further investigations are necessary to better determine the origin of the epileptic abnormalities observed. In any case, the clinical, neurophysiological and neuropsychological peculiarities of this case lead us to classify it as an atypical form of eyelid myoclonia. -

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Acknowledgments We thank L D'Arrigo for translation of text, M Sebastio for technical assistence and C Castellani for ocular consulation.

References Auguglia U, Tinuper P, Gobbi G, Gastaut H (1985) Absence status appearing on eyeclosure. Clin Electroencephalogr 16, 111-118 Brinciotti M, Matricardi M, Paolella A, Porro G, Benedetti P (1989) Neuropsychological correlates of subclinical paroxysmal EEG activity in children with epilepsy. Funct Neurol 4/3, 235-239 Cooper J, Soo IK (1991) Reactive occipital epileptiform activity: it is benign. Epilepsia 32, 63-68 Darby CE, Korte RA, Binnie CD, Wilkins AJ (1980) The self-induction of epileptic seizures eye closure. Epilepsia 21, 31-42 De Marco P, Miottello P (1981) Eye closure epilepsy: report of an uncomplicated case. Clin Electroencephalogr 12, 66-68 Gastaut H, Tassinari CA (1966) Triggering mechanisms in epilepsy: the electroclinical point of view. Epilepsia 7, 85-138 Gobbi G, Bruno L, Marinetti S, Parmeggiani A, Tullini A, Salvi F, Tassinari CA, Santanelli P, Bureau M, Dravet CH, Roger J (1989) Eye closure seizures. In: Reflex Seizures and Reflex Epilepsic (Beaumanoir A, Gastaut H, Maquet R, eds) Mdd Hyg, Geneva, 181-191 Green JB (1968) Seizures on closing the eyes.

Electroencephalographic studies. Neurology 18, 391-396 Jeavons PM (1982) Photosensitive epilepsy. In: A Textbook o f epilepsy (Laidlaw J, Richens A, eds) Churchill Livingstone, Edinburgh, 195-211 Lewis JA (1972) Eye closure as a motor trigger for seizures. Neurology 22, 1145-1150 Kastelejn-Nolst Trenite DGA, Smit AM, Velis DM, WiUemse J, Van EDME Boas W (i 990) On-line detection of transient neuropsychological disturbances during EEG discharges in children with epilepsy. Dev Med Child Neurol 31, 45-50 Panayiotopoulos C (1987) Fixation-offsensitive epilepsy in eyelid myoclonia with absence seizures. Ann Neurol 22, 87-89 Rafal RD, Laxer KD, Janowsky JS (1986) Seizures triggered by blinking in a nonphotosensitive epileptic. J Neurol Neurosurg Psychiatry 49, 445-447 Shanzer S, April R, Atkin A (1965) Seizures induced by eye deviation. Arch Neuro113, 621-626 Vigrqaendra V, Ghee T, Loh, Lee C, Lim Slew Tin (1976) Epileptic discharges triggered by blinking and eye closure. Electroencephalogr Clin Neurophysiol 40, 491-498 Watanabe K, Negoro T, Matsumoto A (1985) Self-induced photogenic epilepsy in infants. Arch Neurol 42, 406-407

Electrical status epilepticus on eye closure: a case report.

The authors present the clinical case of a boy manifesting eyelid myoclonias on eye closure. The corresponding electroencephalographic recording was c...
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