Seizure 23 (2014) 314–317
Contents lists available at ScienceDirect
Seizure journal homepage: www.elsevier.com/locate/yseiz
Clinical letter
Does ictal whistling help to lateralise Evren Burakgazi *, Usman Moghal, Debra Hughes, Melissa Carran Neurology, Cooper Medical School of Rowan University, 3 Cooper PLaza, Suite 320, Camden, NJ 08103, United States
A R T I C L E I N F O
Article history: Received 11 June 2013 Received in revised form 13 November 2013 Accepted 15 November 2013
We present a right-handed, young female with a history of epilepsy since the age of 11. She also suffered from systemic lupus erythematosus, requiring treatment with hydoxychloroquine. The patient described no warning signs that preceded her seizures. Her seizures were associated with loss of awareness for approximately 1 min followed by post-ictal confusion for 5 min. During some of her seizures, whistling was reported by witnesses. Her seizures clustered around her menstrual cycle. She was treated with different antiepileptic medications, such as carbamazepine, phenytoin, zonisamide, pregabalin, and oxcarbazepine. Her treatment regimen upon admission to the epilepsy monitoring unit included levetiracetam, phenobarbital, and lacosamide. She was admitted for video EEG monitoring for a presurgical evaluation for refractory epilepsy. She experienced two to five seizures per month. Video EEG monitoring with telemetry revealed the timing and symptomatology of her seizures. During the five days of video EEG monitoring, two different types of seizures were recorded. Her seizures were associated with loss of awareness and whistling initiated in the left hemisphere, reaching a maximum onset in the left temporal lobe, specifically at the midtemporal region at T5 electrode. Her second type of seizures, associated with staring and unresponsiveness, were initiated in the right temporal lobe. The left temporal lobe-onset seizures initiated as rhythmic sharp theta waves in the left temporal lobe approximately 10 s after the clinical onset of whistling. The video EEG revealed a spread of the field to the right hemisphere within 10 s, followed by diffuse delta waves. Towards the end of each seizure, delta frequencies exhibited greater amplitude and laterality in the left
* Corresponding author. Tel.: +1 856 938 8579. E-mail address: [email protected] (E. Burakgazi).
posterior quadrant. The total duration of the seizures with left temporal lobe onset was approximately 2 min. Clinically, the patient exhibited whistling followed by staring with a lack of awareness of her surroundings, followed by a gaze preference to the left with head deviation to the same side. She experienced restless movements of her left upper and lower extremities. Whistling was the first identifiable clinical sign observed during these seizures. Whistling was always initiated approximately 10 s before scalp EEG onset. There were no other clinical signs or automatisms recorded. The patient experienced post-ictal confusion for a few minutes afterwards (Figs. 1 and 2; Video 1: left temporal onset seizure). The right temporal lobe-onset seizures initiated as rhythmic sharp theta discharges in the right temporal lobe, with a spread to the left hemisphere at times. Clinically, the patient paused her activities and became unresponsive, with staring but without any automatism. No other clinical symptoms were observed. These seizures lasted approximately 2 min (Figs. 3 and 4; Video 2: right temporal onset seizure). The patient experienced a total of two right temporal lobeonset seizures: one recorded on the first day of admission and the other on the second day. There were a total of five left temporal lobe-onset seizures with whistling, three of which were recorded on the second day and two of which were captured on the third day of hospitalisation. The administration of all of the antiepileptic medications began to be tapered upon admission. Interictal and epileptiform discharges and focal slowing were detected in the temporal lobes of both hemispheres, in addition to the left central and posterior quadrant regions. An MRI scan revealed several small foci of increased signal intensity in flair and T2-weighted images, primarily within the subcortical white matter tracts of both cerebral hemispheres. 1. Discussion Whistling is a complex motor process that involves the coordinated articulation of the lips, the teeth, and the tongue. The process involves various cortical and subcortical areas of the brain. Complex neuronal networks linking the inferior rolandic cortex, the cingulate cortex, the basal ganglia, the amygdala, the thalamus, and the cerebellum have been shown to be involved in whistling behaviour, based on functional imaging.1 The phenomenon of ictal whistling can be described as an automatic behaviour
1059-1311/$ – see front matter . Published by Elsevier Ireland Ltd on behalf of British Epilepsy Association. http://dx.doi.org/10.1016/j.seizure.2013.11.014
E. Burakgazi et al. / Seizure 23 (2014) 314–317
315
Fig. 1. Whistling seizure; clinical onset as marked by purple notations precedes EEG onset of rhythmic discharges over the left temporal area. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
Fig. 2. EEG onset with rhythmic theta discharges over left temporal area.
316
E. Burakgazi et al. / Seizure 23 (2014) 314–317
Fig. 3. Onset of right temporal seizure.
Fig. 4. Progression of right temporal seizure.
E. Burakgazi et al. / Seizure 23 (2014) 314–317
that is associated with temporal lobe epilepsy. To date, there have been five reported cases of ictal whistling, with inconsistent localisation. Lazzarino and Valassi reported the first case of ictal whistling in 1982, suggesting that it originated in the frontal lobe.2 The first two cases of ictal whistling to be abolished with temporal lobe surgery were reported by Raghavendra et al. in 2010, but one of the patients was reported to have experienced right temporal lobe seizures associated with ictal whistling.3 It is clearly understood that automatisms are not highly reliably lateralised. In our case, we determined the possible onset of seizures based on scalp EEG, although scalp EEG temporal lobe seizure onset has been reported to be contralateral to ictal onset when recorded by intracranial electrode monitoring for temporal lobe resection surgery.4 In our patient, two different types of seizures are reported with different clinical and EEG features. Seizures originating from the left temporal lobe were consistently associated with ictal whistling. The right temporal lobe-onset seizures did not manifest with whistling. Ictal whistling is a rare and interesting phenomenon that is most likely related to the recruitment of different related neural networks in different regions of both hemispheres rather than activation of a specific cortical region. However, we suggest that ictal whistling may be a complex automatism related to a release
317
phenomenon of the non-dominant hemisphere, given the complexity of the motor behaviour and the fact that the patient is righthanded.
Conflict of interest The authors declare that they have no conflict of interest. Appendix A. Supplementary data Supplementary material related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.seizure.2013.11.014. References 1. Dresel C, Castrop F, Haslinger B, Wohlschlaeger AM, Hennenlotter A, CeballosBaumann AO, et al. The functional neuroanatomy of coordinated orofacial movements: sparse sampling fMRI of whistling. Neuroimage 2005;28:588–97. 2. Lazzarino LG, Valassi F. Whistling as a manifestation of epilepsy. Riv Neurobiol 1982;28:127–30. 3. Raghavendra S, Mirsattari S, McLachlan RS. Ictal whistling: a rare automatism during temporal lobe seizures. Epileptic Disord 2010;12(2):133–5. 4. Mintzer S, Cendes F, Soss J, Andermann F, Engel Jr J, Dubeau F, Olivier A, et al. Unilateral hippocampal sclerosis with contralateral temporal scalp ictal onset. Epilepsia 2004;45(7):792–802.
Contents lists available at ScienceDirect
Seizure journal homepage: www.elsevier.com/locate/yseiz
Clinical letter
Does ictal whistling help to lateralise Evren Burakgazi *, Usman Moghal, Debra Hughes, Melissa Carran Neurology, Cooper Medical School of Rowan University, 3 Cooper PLaza, Suite 320, Camden, NJ 08103, United States
A R T I C L E I N F O
Article history: Received 11 June 2013 Received in revised form 13 November 2013 Accepted 15 November 2013
We present a right-handed, young female with a history of epilepsy since the age of 11. She also suffered from systemic lupus erythematosus, requiring treatment with hydoxychloroquine. The patient described no warning signs that preceded her seizures. Her seizures were associated with loss of awareness for approximately 1 min followed by post-ictal confusion for 5 min. During some of her seizures, whistling was reported by witnesses. Her seizures clustered around her menstrual cycle. She was treated with different antiepileptic medications, such as carbamazepine, phenytoin, zonisamide, pregabalin, and oxcarbazepine. Her treatment regimen upon admission to the epilepsy monitoring unit included levetiracetam, phenobarbital, and lacosamide. She was admitted for video EEG monitoring for a presurgical evaluation for refractory epilepsy. She experienced two to five seizures per month. Video EEG monitoring with telemetry revealed the timing and symptomatology of her seizures. During the five days of video EEG monitoring, two different types of seizures were recorded. Her seizures were associated with loss of awareness and whistling initiated in the left hemisphere, reaching a maximum onset in the left temporal lobe, specifically at the midtemporal region at T5 electrode. Her second type of seizures, associated with staring and unresponsiveness, were initiated in the right temporal lobe. The left temporal lobe-onset seizures initiated as rhythmic sharp theta waves in the left temporal lobe approximately 10 s after the clinical onset of whistling. The video EEG revealed a spread of the field to the right hemisphere within 10 s, followed by diffuse delta waves. Towards the end of each seizure, delta frequencies exhibited greater amplitude and laterality in the left
* Corresponding author. Tel.: +1 856 938 8579. E-mail address: [email protected] (E. Burakgazi).
posterior quadrant. The total duration of the seizures with left temporal lobe onset was approximately 2 min. Clinically, the patient exhibited whistling followed by staring with a lack of awareness of her surroundings, followed by a gaze preference to the left with head deviation to the same side. She experienced restless movements of her left upper and lower extremities. Whistling was the first identifiable clinical sign observed during these seizures. Whistling was always initiated approximately 10 s before scalp EEG onset. There were no other clinical signs or automatisms recorded. The patient experienced post-ictal confusion for a few minutes afterwards (Figs. 1 and 2; Video 1: left temporal onset seizure). The right temporal lobe-onset seizures initiated as rhythmic sharp theta discharges in the right temporal lobe, with a spread to the left hemisphere at times. Clinically, the patient paused her activities and became unresponsive, with staring but without any automatism. No other clinical symptoms were observed. These seizures lasted approximately 2 min (Figs. 3 and 4; Video 2: right temporal onset seizure). The patient experienced a total of two right temporal lobeonset seizures: one recorded on the first day of admission and the other on the second day. There were a total of five left temporal lobe-onset seizures with whistling, three of which were recorded on the second day and two of which were captured on the third day of hospitalisation. The administration of all of the antiepileptic medications began to be tapered upon admission. Interictal and epileptiform discharges and focal slowing were detected in the temporal lobes of both hemispheres, in addition to the left central and posterior quadrant regions. An MRI scan revealed several small foci of increased signal intensity in flair and T2-weighted images, primarily within the subcortical white matter tracts of both cerebral hemispheres. 1. Discussion Whistling is a complex motor process that involves the coordinated articulation of the lips, the teeth, and the tongue. The process involves various cortical and subcortical areas of the brain. Complex neuronal networks linking the inferior rolandic cortex, the cingulate cortex, the basal ganglia, the amygdala, the thalamus, and the cerebellum have been shown to be involved in whistling behaviour, based on functional imaging.1 The phenomenon of ictal whistling can be described as an automatic behaviour
1059-1311/$ – see front matter . Published by Elsevier Ireland Ltd on behalf of British Epilepsy Association. http://dx.doi.org/10.1016/j.seizure.2013.11.014
E. Burakgazi et al. / Seizure 23 (2014) 314–317
315
Fig. 1. Whistling seizure; clinical onset as marked by purple notations precedes EEG onset of rhythmic discharges over the left temporal area. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
Fig. 2. EEG onset with rhythmic theta discharges over left temporal area.
316
E. Burakgazi et al. / Seizure 23 (2014) 314–317
Fig. 3. Onset of right temporal seizure.
Fig. 4. Progression of right temporal seizure.
E. Burakgazi et al. / Seizure 23 (2014) 314–317
that is associated with temporal lobe epilepsy. To date, there have been five reported cases of ictal whistling, with inconsistent localisation. Lazzarino and Valassi reported the first case of ictal whistling in 1982, suggesting that it originated in the frontal lobe.2 The first two cases of ictal whistling to be abolished with temporal lobe surgery were reported by Raghavendra et al. in 2010, but one of the patients was reported to have experienced right temporal lobe seizures associated with ictal whistling.3 It is clearly understood that automatisms are not highly reliably lateralised. In our case, we determined the possible onset of seizures based on scalp EEG, although scalp EEG temporal lobe seizure onset has been reported to be contralateral to ictal onset when recorded by intracranial electrode monitoring for temporal lobe resection surgery.4 In our patient, two different types of seizures are reported with different clinical and EEG features. Seizures originating from the left temporal lobe were consistently associated with ictal whistling. The right temporal lobe-onset seizures did not manifest with whistling. Ictal whistling is a rare and interesting phenomenon that is most likely related to the recruitment of different related neural networks in different regions of both hemispheres rather than activation of a specific cortical region. However, we suggest that ictal whistling may be a complex automatism related to a release
317
phenomenon of the non-dominant hemisphere, given the complexity of the motor behaviour and the fact that the patient is righthanded.
Conflict of interest The authors declare that they have no conflict of interest. Appendix A. Supplementary data Supplementary material related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.seizure.2013.11.014. References 1. Dresel C, Castrop F, Haslinger B, Wohlschlaeger AM, Hennenlotter A, CeballosBaumann AO, et al. The functional neuroanatomy of coordinated orofacial movements: sparse sampling fMRI of whistling. Neuroimage 2005;28:588–97. 2. Lazzarino LG, Valassi F. Whistling as a manifestation of epilepsy. Riv Neurobiol 1982;28:127–30. 3. Raghavendra S, Mirsattari S, McLachlan RS. Ictal whistling: a rare automatism during temporal lobe seizures. Epileptic Disord 2010;12(2):133–5. 4. Mintzer S, Cendes F, Soss J, Andermann F, Engel Jr J, Dubeau F, Olivier A, et al. Unilateral hippocampal sclerosis with contralateral temporal scalp ictal onset. Epilepsia 2004;45(7):792–802.
