detecting interictal epileptiform activity are to be maximized. Noninvasive recordings from the sphenoidal region may be justified in selected patients but this does not require the use of minisphenoidal electrodes.

Addendum Since the submission of our paper for publication, Sadler and Goodwin [9} have reported a study similar to ours to determine the relative sensitivity of different electrode placements for detecting spike discharges in patients with complex partial seizures. However, their data are presented in such a way that direct comparison with our findings is difficult, and it is not possible to determine whether the combination of electrodes that we found most useful was similarly helpful in their experience. From personal communication with these authors, however, we can comment further about their experience with nasopharyngeal electrodes. Among the patients in their series in whom recordings were made from nasopharyngeal electrodes, a total of 159 spikes were detected but only 2 (1.3%) of these were seen exclusively in the nasopharyngeal electrodes. The reason for such a low yield probably relates in part to selection factors. Thus, we studied a group of unselected patients with complex partial seizures whereas Sadler and Goodwin intentionally selected patients who were known from previous EEG studies to have spike discharges in the lateral anterior temporal region.

References 1. Homan RW, Jones MC, Rawat S. Anterior temporal electrodes in complex partial seizures. Electroencephalogr Clin Neurophysiol 1988;70:105-109 2. Sperling MR, Engel J Jr. Electroencephalographic recording from the temporal lobes: a comparison of ear, anterior temporal, and nasopharyngeal electrodes. Ann Neurol 1985;17:510-513 3. Levin R, Leaton EM, Lee SI. The value of nasopharyngeal recording in psychiatric patients. Biol Psychiatry 1986;21:1236-1238 4. Sadler M, Goodwin J. The sensitivity of various electrodes in the detection of epileptiform potentials (EPs) in patients with partial complex (PC) seizures. Epilepsia 1986;27:627 (Abstract) 5. Laxer KD. Mini-sphenoidal electrodes in the investigation of seizures. Electroencephalogr Clin Neurophysiol 1984;58:127-129 6. Silverman D. The anterior temporal electrode and the ten-twenty system. Electroencephalogr Clin Neurophysiol 1960;12:735737 7. Mavor H, Hellen MK. Nasopharyngeal electrode recording. Am J Electroencephalogr Tech 1964;4:43-50 8. Gloor P. Contributions of electroencephalography and electrocorticography to the neurosurgical treatment of the epilepsies. Adv Neurol 1975;8:59-105 9. Sadler RM, Goodwin J. Multiple electrodes for detecting spikes in partial complex seizures. Can J Neurol Sci 1989;16:326-329

Evelid Twitching Seizures and GenerahzedY Tonic-Clonic Convulsions: A Syndrome of Idiopathic Generahzed Epilepsy John W. Miller, MD, PhD,* and James A. Ferrendelli, MD*I This is a report of two neurologically normal patients who had primary generalized seizures consisting of irregular fluttering or twitching movements of the eyelids accompanied by generalized, rhythmic 9- to 15-H~electroencephalographic discharges as well as infrequent generalized tonic-clonic seizures. This is a syndrome of idiopathic generalized epilepsy that responds to treatment with valproic acid. Miller JW, Ferrendelli JA. Eyelid twitching seizures and generalized tonic-clonic convulsions: a syndrome of idiopathic generalized epilepsy. Ann Neurol 1990;27:334-336 Various eyelid movements are known to occur during seizures. Myoclonic twitches or closure of the upper lids are a well-recognized correlate to absence seizures with generalized spike and wave discharges El-41. A less-discussed phenomenon is eyelid twitching o r myoclonus with a generalized, rhythmic, rapid electroencephalographic (EEG) discharge. We describe 2 patients with this type of seizure.

Case Reports Patient 1

A woman was first seen on May 4, 1987 at the age of 39 years. At age 16 she had her first generalized tonic-clonic convulsion. Subsequently she had six more convulsions. All were similar; they often were preceded by sleep deprivation or occurred during pregnancy. For a few hours or even days prior to each convulsion she had numerous brief episodes of eyelid twitching. She was intially treated with phenobarbital, and subsequently acetazolamide and phenytoin without seizure control. A cranial computed tomographic (CT) scan in 1981 was normal. When seen, the patient was taking phenytoin, 200 mg twice daily; phenobarbital, 60 mg twice daily; and acetazolamide, 250 mdday. She was the product of a normal labor and delivery and denied any family history of seizures (she has two sisters and one child) or any history of head trauma From the 'Department of Neurology and Neurological Surgery (Neurology) and the tDeparunent of Pharmacology, Washington University School of Medicine, St. Louis, MO. Received Jul 20, 1989. Accepted for publication Sep 12, 1989. Address correspondence to Dr Miller, Department of Neurology, Washington University School of Medicine, 660 South Euclid, St. Louis. MO 631 10.

334

Copyright 0 1990 by the American Neurological Association

or neurological illness. She was a college graduate. The results of a complete examination were normal. Acetazolamide was stopped, but the other medications were continued. Four days after this she developed episodes of lid twitching, and an EEG study was performed. Acetazolamide was restarted, and seizures stopped. At the time of writing, she was seizure free on valproic acid (VPA) monotherapy (divalproex sodium, 500 mg three times daily).

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Patient 2 A woman was seen on August 22, 1988, at the age of 20 years. At age 13 she developed a mass in the left side of the neck, and was found to have nodular sclerosing Hodgkin's lymphoma, stage IIA. She was given radiation treatment to the left side of the neck and mandible, and has had no recurrence to date. At age 15 she suffered a generalized tonicclonic seizure without warning. Results of a cranial CT scan and cerebrospinal fluid (CSF) examination were normal, and she was started on phenytoin, 300 mglday. At age 17 she stopped taking phenytoin and a month later had another convulsion. Another CT scan was normal, and phenytoin was restarted. At age 19 during pregnancy she began to have numerous daily episodes of eyelid twitching and blinking without other body movements, which persisted despite a temporary increase of phenytoin to 500 mglday. These spells lasted a few seconds; neither she nor her mother were aware of interruption of consciousness during them. When first seen, after delivery, the patient was taking phenytoin, 300 mdday, with continuing daily seizures. She denied any other type of seizures. She was the product of a normal labor and delivery, and had common migraine but denied head trauma or family history of seizures. She was a high school graduate. The only significant finding on examination was gum hyperplasia. She initially declined to take valproic acid and was given carbamazepine, but the lid twitching episodes increased. Three months later treatment was changed to divalproex sodium, 1,250 mglday in four divided doses, which resulted in marked reduction in these episodes.

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Patient 1 A 33-minute EEG recording was performed with video monitoring on May 8, 1987. The technical guidelines of the American Electroencephalographic Society 151 were followed for all recordings. This recording revealed a normal symmetrical 10-Hz alpha rhythm that was interrupted by frequent generalized , toll-Hz epileptiform acbursts of 50- to 2 0 0 - ~ V 7tivity (Fig 1). These bursts lasted from 0.5 to 6.5 seconds, and often terminated with generalized spike and slow wave activity. They occurred an average of 7 times per minute and were not induced or affected by eye opening or closing or photic stimulation. Each burst was accompanied by continuous irregular flickering movements of the upper eyelids. Occasionally forehead movement was also seen, but other facial or limb muscles were not involved. Eye movements were not seen during the episodes, which occurred with eyes open, and no electrical artifact from eye

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movement was apparent during the spells with eyes closed. She was alert and talking, but paused during each discharge. She stated that she was aware of the episodes and perceived the lid movements, but complained of losing track of her thoughts with the spells. Nevertheless, on five occasions when words were spoken by the technician during EEG discharges she correctly repeated them after the bursts, albeit with hesitation. Patient 2 This patient had a routine EEG on September 1, 1988. This revealed a symmetrical 10-Hz alpha rhythm as well as normal drowsy and sleep background activity. During 5 minutes of hyperventilation with eyes closed there were two bursts of generalized paroxysmal fast activity (Fig 2) with a duration of 2 seconds, a frequency of about 15 Hz, and an amplitude of approximately 50 FV. These were accompanied by her typical symptoms of lid flutter. Late in the recording, another half-second burst of similar activity occurred during drowsiness with eyes closed. Photic stimulation did not activate any abnormalities.

Discussion These 2 patients' conditions were remarkably similar. Both developed epilepsy in midadolescence, with episodes of eyelid flutter and also infrequent generalized tonic-clonic seizures. In both, VPA was the most effective treatment. The EEG discharges accompanying these patients' nonconvulsive seizures were also similar (Figs 1 and 2), differing only in frequency and amplitude. Such rapid generalized epileptiform discharges have been previ-

Brief Communication: Miller and Ferrendelli: Eyelid Twitching Seizures 335

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automatisms and limb jerking, unresponsive to VPA; and (3) myoclonus absences [13}, which may begin with eye fluttering, but progress to myoclonic limb jerks and automatisms, and are also unresponsive to VPA. Panayiotopoulos [141 described a neurologically intact woman who had episodes of eyelid twitching with generalized paroxysmal fast activity since the age of 5. This patient had the most similarity to our patients but her seizures were induced by interruption of visual hation, while our patients’ seizures were not triggered by any visual stimulus. These eyelid flutter seizures can only be correctly identified if they occur during an EEG recording. Thus, although this syndrome must be quite rare, it must also go unrecognized. We believe this condition is a form of idiopathic generalized epilepsy, falling in category 2.1 in the International Classification [151, and is related to other forms of presumably familial epilepsy with myoclonic, absence, and/or generalized tonic-clonic seizures with age-related onset.

References ously described in various clinical situations. Discharges in the alpha frequency range have been called “the epileptic recruiting response” [b],with faster discharges being referred to as “the grand mal pattern” [7], and “generalized paroxysmal fast activity” [S}. The distinction between different frequencies of such discharges is in some respects arbitrary, since the discharges usually consist of a mixture of frequencies, and the frequency also varies between different discharges in the same patient, even those occurring with the same type of clinical seizure {9-111. In fact, 8- to 10-Hz generahzed discharges are more often associated with the onset of grand mal seizures [b, 111, while generalized paroxysmal fast activity is seen with various seizure types, with the vast majority of patients having mental retardation and many, the Lennox-Gastaut syndrome {S, lo}. Although many bursts of generalized paroxysmal fast activity are subclinical, face, limb, or body movements may also be seen [lo]. In one series {lo], eyelid fluttering was seen as at least a partial clinical manifestation in only 3 of 113 such bursts in 14 patients. The 2 patients reported here are clearly different because they had normal intelligence and normal findings on neurological examination, and because the EEG bursts had a single, stereotyped clinical accompaniment. There are a few previously reported cases of nonconvulsive seizures with similar EEG discharges in neurologically normal patients, all significantly different from our patients. Delgado-Escueta 112) noted three seizure types associated with 8- to 12-Hz generalized bursts: (1) absences with staring and automatisms, responsive to VPA; (2) myoclonic absences with 336 Annals of Neurology Vol 27 No 3 March 1990

1. Penfield W, Jasper H . Epilepsy and the functional anatomy of the human brain. Boston: Little, Brown, 1954. 896 p 2. Lennox WG. Epilepsy and related disorders, vol 1. Boston: Little, Brown, 1960. 574 p 3. Bickford RG, Klass DW. Eye movement and the electroencephalogram. In: Bender MB, ed. The oculomotor system. New York: Harper and Row, 1964:293-302 4. Orren MM, Mirsky AF. Relation between ocular manifestations and onset of spike-and-wave discharges in petit mal epilepsy. Epilepsia 1975 ;16:77 1-7 79 5. American Electroencephalographic Society. Minimal technical requirements for performing clinical electroencephalography. J Clin Neurophysiol 1986;3(suppl 1):l-6 6. Gastaut H , Broughton R. Epileptic seizures-clinicaland electroencephalographic features, diagnoJis and treatment. Springfield, I L Thomas, 1972:36-37 7. Gibbs FA, Gibbs EL, Lennox WG. Electroencephalographic classification of epileptic patients and control subjects. Arch Neurol Psychiatry 1943;50:111-128 8. Brenner RP, Atkinson R. Generalized paroxysmal fast activity: electroencephalographic and clinical features. Ann Neurol 1982;11:386-390 9. Jasper H, Kershman J. Classification of the EEG in epilepsy. Electroencephalogr Clin Neurophysiol 1949;suppl 2:123-131 10. Rodin E, Smid N, Mason K. The grand mal pattern of Gibbs, Gibbs, and Lennox. Electroencephalogr Clin Neurophysiol 1976;40:401-406 11. Chatrian GE, Petersen MC. The convulsive patterns provoked by Indoklon, Metrazol and electroshock: some depth electrographic observations in human patients. Electroencephalogr Clin Neurophysiol 1960;12:715-725 12. Delgado-Escueta AV. Epileptogenic paroxysms: modern approaches and clinical correlations. Neurology 1979;29:10141022 13. Nadel A, Waddel G, Volow M, Delgado-Escueta AV. Unconsciousness, automatisms, and myoclonic jerks with diffuse rhythmic 9-hz discharges. Epilepsia 1975;16:511-520 14. Panayiotopoulos CP. Fixation-off-sensitive epilepsy in eyelid myoclonia with absence seizures. Ann Neurol 1987;22:87-89 15. Dreifuss FE, Martinet-Lage M, Roger J, et al. Proposal for classification of epilepsies and epileptic syndromes. Epilepsia 1985;26:268-278