Epilepsy & Behavior 6 (2005) 452–455 www.elsevier.com/locate/yebeh
Case Report
Hush sign: A new clinical sign in temporal lobe epilepsy Gulnihal Kutlu a,*, Erhan Bilir b, Atilla Erdem c, Yasemin B. Gomceli a, G. Semiha Kurt d, Ayse Serdaroglu e a
Department of Neurology, Ministry of Health, Ankara Training and Research Hospital, Ankara, Turkey b Department of Neurology, Medical Faculty, Gazi University, Ankara, Turkey c Department of Neurosurgery, Medical Faculty, Ankara University, Ankara, Turkey d Department of Neurology, Medical Faculty, Gaziosmanpasa University, Ankara, Turkey e Department of Pediatrics, Medical University, Gazi University, Ankara, Turkey Received 23 October 2004; revised 2 December 2004; accepted 17 January 2005 Available online 16 March 2005
Abstract Neurologists have been analyzing the clinical behaviors that occur during seizures for many years. Several ictal behaviors have been defined in temporal lobe epilepsy (TLE). Ictal behaviors are especially important in the evaluation of epilepsy surgery candidates. We propose a new lateralizing sign in TLE originating from the nondominant hemisphere—the ‘‘hush’’ sign. Our patients were 30- and 21-year old women (Cases 1 and 2, respectively). Their epileptogenic foci were localized to the right mesial temporal region after noninvasive presurgical investigations. Case 1 had no cranial MRI abnormality, whereas cranial MRI revealed right hippocampal atrophy in Case 2. These women repeatedly moved their right index fingers to their mouth while puckering their lips during complex partial seizures. We have named this ictal behavior the ‘‘hush’’ sign. Anterior temporal lobectomy with amygdalohippocampectomy was performed in both patients, and pathological examinations revealed hippocampal sclerosis. The ‘‘hush’’ sign no longer occurred after seizures were controlled. They were seizure free as of 30 and 31 months of follow-up, respectively. We believe that the ‘‘hush’’ sign may be supportive of a diagnosis of TLE originating from the nondominant hemisphere. This sign may occur as a result of ictal activation of a specific brain region in this hemisphere. ! 2005 Elsevier Inc. All rights reserved. Keywords: Ictal behavior; Hush sign; Temporal lobe epilepsy
1. Introduction Neurologists have been investigating clinical behaviors that occur during seizures for many years. Several ictal behaviors have been defined in temporal lobe epilepsy (TLE) by video/EEG monitoring [1–5]. Many are very important in the evaluation of candidates for epilepsy surgery [3]. Useful ictal lateralizing signs in patients with TLE include contralateral dystonic posturing [4,6], unilateral hand automatisms [1,5], early ipsilateral head deviation [7], forced contralateral head deviation *
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[email protected] (G. Kutlu).
1525-5050/$ - see front matter ! 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.yebeh.2005.01.006
[1,4,7], ictal speech [8–10], ictal vomiting [11,12], unilateral eye blinking [3], postictal nose wiping [2], automatisms with preserved consciousness [13], and postictal dysphasia/aphasia [14]. Based on our observations of two patients with TLE, we propose a new clinical sign—the ‘‘hush’’ sign.
2. Case 1 This 30-year-old woman with medically intractable, complex partial seizures since age 4 had a history of febrile convulsions at the age of 3. Physical and neurological examinations were normal. Under treatment
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with oxcarbazepine 1800 mg/day, she had at least one complex partial seizure per week, and occasional secondary generalization was also observed. Cranial MRI was normal. Interictal EEG revealed isolated sharp waves and polymorphic delta activity in the right temporal region. She was evaluated as a candidate for surgery at the Gazi University Epilepsy Center, where she was continuously monitored with simultaneous closed-circuit video/surface EEG. She had five typical complex partial seizures during monitoring. The epileptogenic focus was determined to be in the right mesial right temporal region. We analyzed her clinical behaviors during seizures. A nonforced deviation of the head to the right and right-hand automatism were observed. Then, during each seizure, the patient was observed to make a ‘‘hush’’ sign, puckering her lips and moving her right index finger to her mouth (see Figs. 1–3). Meanwhile, she also called her mother and asked whether she had pressed the button or not. In the last stage, secondary generalization was observed. Although she was responsive at the beginning of the seizures, she remembered neither the seizures nor calling her mother and making a ‘‘hush’’ sign. We did not see in any seizure either oral or manual automatisms. The ictal EEG first showed delta waves in the temporal region for 2 seconds, followed by theta activity that spread from the right temporal area to the entire right hemisphere. Therefore, the right temporal area was considered the epileptogenic focus. The left hemisphere was determined to be the dominant side after neuropsychological evaluation. She underwent a right anterior temporal lobectomy with amygdalohippocampectomy at Ankara University Medical Faculty Department of Neurosurgery after neuropsychological and Wada tests. Pathological assessment revealed hippocampal sclerosis. She remains seizure free 30 months into follow-up. The ‘‘hush’’ behavior was no longer observed. Her antiepileptic medication was gradually stopped after 24 months.
3. Case 2 This 21-year-old woman with medically intractable, complex partial seizures since age 11 had no history of febrile convulsions or head trauma or family history of seizures. Physical and neurological examinations were unremarkable. Under treatment with oxcarbazepine 1800 mg/day and lamotrigine 200 mg/day, she had approximately one or two complex partial seizures per week and occasional secondarily generalized seizures. Cranial MRI revealed right hippocampal atrophy. Sharp and slow waves in the right temporal region were observed on interictal EEG. As for Case 1, she was evaluated as a candidate for surgery at Gazi University Epilepsy Center. She had three complex
Fig. 1. (A–C) ‘‘Hush’’ sign: moving the right index finger to the mouth.
partial seizures during monitoring. During the seizures she suddenly became unresponsive and, as did Case 1, moved her right index finger to her mouth and puckered her lips; contralateral dystonia was noted in her left arm. The right temporal region was determined to be the epileptogenic focus. Similar to Case 1, we did not observe any seizure with only oral or manual automatisms. Ictal EEG initially showed diffuse nonspecific slowing for 10 seconds, followed by rhythmic theta and sharp waves from the right temporal area.
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Fig. 2. (A,B) ‘‘Hush’’ sign: puckering of lips.
Fig. 3. (A,B) ‘‘Hush’’ sign: puckering of lips.
After that, rhythmic theta wave and sharp waves were observed in the right temporal area. The left hemisphere was the dominant side as in Case 1. The patient underwent right anterior temporal lobectomy with amygdalohippocampectomy at Ankara University Medical Faculty Department of Neurosurgery after completing neuropsychiatric and Wada tests. Pathological assessment revealed hippocampal sclerosis. She remains seizure free 31 months into follow-up. The ‘‘hush’’ behavior was no longer observed. We stopped her antiepileptic treatment gradually after 24 months.
Some ictal behavioral characteristics that have been considered supportive of a diagnosis of right TLE were previously defined: ictal speech [8–10], ictus emeticus [11], automatisms with preserved responsiveness [13], and ictal urinary urge [15]. Preserved responsiveness is defined as the ability of a patient to respond verbally or to follow motor commands throughout the seizure. Ebner et al. reported seven patients with preserved responsiveness in the presence of automatisms. They investigated 15 seizures. The ictal EEG abnormality was localized over the right temporal area in nine seizures and over the right hemisphere in five seizures, and was not localized in one seizure. They concluded that automatisms with preserved responsiveness lateralized to the right side in TLE [13]. Gabr et al. reviewed video/EEG records of 100 complex partial seizures in 35 patients who underwent anterior temporal lobectomy. They observed ictal speech in 79 seizures. They classified this speech as vocalization, normal speech, or abnormal speech. Normal speech occurred ictally in 34.2% of patients, and in 83% of these, it originated from the nondominant hemisphere. However, they observed normal speech in 12.5% patients with seizures originating from the
4. Discussion Several ictal lateralizing signs have been investigated by video/EEG monitoring and associated with the epileptogenic focus [1–5]. However, the majority of these behavioral patterns are not characteristic of TLE [1,2]. In this study we identified what may be a new lateralizing sign of seizures with right temporal lobe onset. Because it is a stereotypic clinical feature, we have named it the ‘‘hush’’ sign.
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dominant hemisphere [9]. Case 1 spoke to her mother during automatisms. Her preserved responsiveness during automatisms also implied the right (nondominant) temporal region. Both Cases 1 and 2 underwent anterior temporal lobectomy with amygdalohippocampectomy, and hippocampal sclerosis was reported on pathological assessment. They were seizure free at 30 and 31 months into follow-up, respectively. Antiepileptic medication had been stopped 24 months into follow-up. In this study both Cases 1 and 2 repeatedly moved their right index fingers to their mouths while puckering their lips. This finding might be a type of hand automatism. However, the fact that these women also puckered their lips during seizures led us to believe this could be a rather complex movement. Automatisms with preserved responsiveness support a right hemispheric focus. Patients with automatisms with preserved responsiveness may be conscious during their seizures but they cannot later remember what they had experienced during the seizures. Our patients may have made the ‘‘hush’’ sign consciously. The reason for this movement is unknown. However, they may not have wanted their seizures to be observed by other people so this ‘‘hush’’ sign might be a reactive reflex. This ictal behavioral pattern could reflect the patient trying to avoid being seen having a seizure in public. On the other hand, the ‘‘hush’’ sign may have occurred as a result of ictal activation of a specific brain region of the nondominant hemisphere. We propose that the ‘‘hush’’ sign supports the diagnosis of epilepsy originating from the nondominant temporal lobe. However, further cases will need to be described before this sign can be confidently localized to the temporal lobe and lateralized to the nondominant hemisphere.
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References [1] Kotagal P, Lu¨ders H, Morris HH, et al. Dystonic posturing in complex partial seizures of temporal lobe onset: a new lateralizing sign. Neurology 1989;39:196–201. [2] Hirsch HJ, Lain AH, Walczak TS. Postictal nosewiping lateralizes and localizes to the ipsilateral temporal lobe. Epilepsia 1998;39:991–7. [3] Benbadis SR, Kotagal P, Klem GH. Unilateral blinking: a lateralizing sign in partial seizures. Neurology 1996;46:45–8. [4] Bleasel A, Kotagal P, Kankirawatana P, Rybicki L. Lateralization value and semiology of ictal limb posturing and version in temporal lobe and extratemporal lobe epilepsy. Epilepsia 1997;38:168–74. [5] Chee MW, Kotagal P, Van Ness PC, Gragg L, Murphy D, Lu¨ders HO. Lateralizing signs in intractable epilepsy: blinded multiple observer analysis. Neurology 1993;43:2519–25. [6] Newton M, Berkovic S, Austin M, et al. Dystonia, clinical lateralization and regional cerebral blood flow changes in temporal lobe seizures. Neurology 1992;42:371–7. [7] Kernan JC, Devinsky O, Luciano DJ, Vazquez B, Perrine K. Lateralizing significance of head and eye deviation in secondary generalized tonic–clonic seizures. Neurology 1993;43:1308–10. [8] Yen DJ, Su MS, Yiu CH, et al. Ictal speech manifestation in temporal lobe epilepsy: a video/EEG study. Epilepsia 1996;37:45–9. [9] Gabr M, Lu¨ders H, Dinner D, Morris H, Wyllie E. Speech manifestation in lateralization of temporal lobe seizures. Ann Neurol 1989;25:82–7. [10] Koerner M, Laxer K. Ictal speech, postictal language dysfunction and seizure lateralization. Neurology 1988;46:45–8. [11] Kramer RE, Lu¨ders H, Goldstick LP, et al. Ictus emeticus: an electroclinical analysis. Neurology 1988;38:1048–52. [12] Van Buren JM. The abdominal aura: a study of abdominal sensations occurring in epilepsy and produced by depth stimulation. Electroencephalogr Clin Neurophysiol 1963;15:1–19. [13] Ebner A, Dinner DS, Noachtar S, Luder H. Automatisms with preserved responsiveness: a lateralizing sign in psychomotor seizures. Neurology 1995;45:61–4. [14] Privitera M, Kohler C, Cahill W, Yeh HS. Postictal language dysfunction in patients with right or bilateral hemispheric language localization. Epilepsia 1996;37:936–41. [15] Loddenkemper K, Foldvary N, Raja S, Neme S, Lu¨ders HO. Ictal urinary urge: further evidence for lateralization to the nondominant hemisphere. Epilepsia 2003;44:124–6.
