Sleep Medicine xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
Sleep Medicine journal homepage: www.elsevier.com/locate/sleep
Video-Clinical Corners
Exploding head syndrome – a rare parasomnia or a dissociative episode? A case report Eva Feketeova a,⇑, Jitka Buskova b, Matej Skorvanek a, Jana Mudra c, Zuzana Gdovinova a a
Faculty of Medicine, P. J. Safarik University and L. Pasteur University Hospital, Kosice, Slovak Republic 1st Faculty of Medicine and General Teaching Hospital, Charles University, Praha, Czech Republic c L. Pasteur University Hospital, Kosice, Slovak Republic b
a r t i c l e
i n f o
Article history: Received 17 November 2013 Received in revised form 14 January 2014 Accepted 17 February 2014 Available online xxxx Keywords: Exploding head syndrome Psychogenic non-epileptic seizures Parasomnia Seizure Focal epilepsy Carbamazepine
1. Introduction Exploding head syndrome (EHS) is characterized by a strange feeling in the head resembling a shooting, bump or blow to the head that occurs during the sleep–wake transition. Such attacks are painless, but they are unpleasant and sometimes terrifying [1]. The disorder is classified among parasomnias, and its etiology remains unknown [2]. 2. Case report A 55-year-old man was admitted to the hospital in order to investigate an increasing number of incidences of a terrifying ‘sudden intense bang or hit on the right side of the head, awakening him from sleep’. The episodes were occurring within an hour of falling asleep and without any obvious causal factor. They lasted from a few seconds to several minutes about once a week for 12 years. They were described as being an unpleasant experience but not a scary one. The patient was able to fall asleep within a few minutes after such episodes and to recall the experience in the morning. The awakenings did not cause daytime sleepiness. ⇑ Corresponding author. Address: Faculty of Medicine, P. J. Safarik University and L. Pasteur University Hospital, Tr. SNP 1, 04011 Kosice, Slovak Republic. Tel.: +421 1911628128. E-mail address: [email protected] (E. Feketeova).
The paroxysms started in 1999, when the patient described ‘a mental breakdown’ accompanied by alcohol excess with sleep deprivation, which resulted in a generalized tonic clonic seizure (GTCS). Because of the close incidence of the night episodes and the GTCS, their uniform character and short duration, the paroxysms were considered to be simple partial seizures. Carbamazepine (CBZ) was prescribed to the patient that same year. In 2012 the patient overcame a pulmonary embolism with the necessity of long-term warfarin treatment. To avoid potential drug interactions, CBZ was tapered off and replaced with levetiracetam. The changes in therapy were accompanied by increased frequency of the night episodes, and the patient was sent for a video-polysomnography (v-PSG) recording in March 2013. Standard blood tests and a detailed neurological exploration (brain magnetic resonance imaging, angiography) were normal. A 48 h v-PSG with a full-scalp electroencephalogram showed no epileptic origin of the attacks, and the two captured episodes (one during night sleep, the second during daytime sleep following sleep deprivation, Fig. 1, Video 1) were awakenings from N2 sleep. PSG showed normal sleep duration and architecture (total sleep time, 6 h 43.5 min; apnea–hypopnea index, 0.2/h; periodic leg movements index, 5.3/ h). The paroxysms were considered to be EHS. The patient was informed about their benign nature, and withdrawal of anti-epileptics was recommended. Three weeks later the paroxysms changed their nature: sleep-fixed attacks were replaced by right arm paresthesias with discharges irradiating over the right side of the face and lasting from several minutes up to 120 min. Terrifying sensory symptoms were relieved by knocking or cycling while lying down (Video 2). The attacks were occurring during daytime wakefulness or after daytime naps, when the patient was still lying in bed. They did not wake him from sleep; they occurred during sedating activities and never during working tasks. Having 13–20 attacks a day in May 2013, the patient was again admitted to the hospital. The attacks were considered to be paroxysmal non-epileptic seizures (episodes resembling epileptic seizures). Psychological examination revealed affective instability, obsessive tendencies, intrapsychical tension, paranoid reactions, anxiety, and sensory symptoms on a background of melancholia with prominent neurolability, suggesting psychogenic origin of non-epileptic seizures: psychogenic non-epileptic seizures (PNES). The psychiatrist diagnosed dissociative convulsions and recommended psychotherapy.
http://dx.doi.org/10.1016/j.sleep.2014.02.011 1389-9457/Ó 2014 Elsevier B.V. All rights reserved.
Please cite this article in press as: Feketeova E et al. Exploding head syndrome – a rare parasomnia or a dissociative episode? A case report. Sleep Med (2014), doi:http://dx.doi.org/10.1016/j.sleep.2014.02.011
2
E. Feketeova et al. / Sleep Medicine xxx (2014) xxx–xxx
Fig. 1. Exploding head syndrome (EHS) attack during daytime nap following sleep deprivation: polysomnographic (PSG) record. (A) A 30 s stage of N2 sleep preceding the EHS attack. (B) A 30 s PSG recording during the EHS episode – movement artifacts and following the awake state. There is no epileptic ictal activity or post-ictal electroencephalographic (EEG) attenuation or slowing present. Sequence of the channels on PSG: Fp1–F7, F7–T3, T3–T5, T5–O1, Fp2–F8, F8–T4, T4–T6, T6–O2, Fp1–F3, F3–C3, C3–P3, P3–O1, Fp2–F4, F4–C4, C4–P4, P4–02–scalp EEG electrodes placed according to the international 10–20 electrode placement system in bipolar longitudinal montage, REOG/LEOG L/R oculograms, EMG3–chin EMG), RED line Video 1 sequence.
Prescribing alprazolam and pregabalin for anxiety, together with psychotherapy, resulted in the disappearance of the daytime PNES and the reappearance (however, in reduced frequency in comparison with March 2013) of the EHS episodes in August 2013.
3. Discussion Exploding head syndrome is thought to likely represent a sensory variant of a hypnic jerk or sleep start [3]. Since the clinical description in 1988, there have been a few subsequent publications describing its clinical and polygraphic characteristics [4–6]. Polysomnography usually shows the EHS events arising from drowsiness [6]; however, the beginning from N2, as in our case, has previously been documented in a case report by Kallweit et al. [5]. The diagnosis of EHS in the presented case was based on a typical history and could not be explained by another sleep disorder or other disorder: the attacks were not accompanied by pain and thus could not be a variant of a specific headache; the short duration without vegetative symptoms did not support panic attacks; and the history and video-PSG recording excluded the diagnosis of par-
tial simple seizures despite their uniform character for 12 years and previously documented GTCS. PSG showed no wakefulness preceding the attacks, which is important for the diagnosis of a sleep-related dissociative disorder. Stress and anxiety seemed to trigger EHS and daytime PNES in the present case. The ability to eliminate daytime attacks and reduce night-time spells by psychotherapy supported by medication strongly suggests their role in the pathophysiology of both conditions. The importance of stress has already been suggested in the EHS [1,6,7] and NREM parasomnias [8]. The role of stress in PNES is also notable; however, stress is not considered to be more frequent or of higher intensity than in other patients, and PNES patients seem to lack effective coping mechanisms [9]. The question remaining unclear in this case is whether it represents a coincidence of ‘benign’ parasomnia EHS and PNES, or whether EHS and PNES are simply different manifestations of a dissociative disorder triggered by stress? The cornerstones of management in EHS are reassurance and education, as this is a benign condition that remits over time in most patients. There have been reports describing success with topiramate [10], clonazepam [11], clomipramine [6,12], and nifedipine [13]; however, pharmacological treatment is typically not
Please cite this article in press as: Feketeova E et al. Exploding head syndrome – a rare parasomnia or a dissociative episode? A case report. Sleep Med (2014), doi:http://dx.doi.org/10.1016/j.sleep.2014.02.011
E. Feketeova et al. / Sleep Medicine xxx (2014) xxx–xxx
needed. Long-term management of EHS attacks in the present case shows that there is no therapeutic response in the long-term administration of CBZ, even when titrated up to maximum tolerated dose during 12 years of treatment. Short-term complex treatment of the underlying mental disorder was more successful in reducing the frequency of EHS attacks, but did not completely eliminate them at three months’ follow-up. Detailed psychological exploration of EHS cases in the future could reveal important mechanisms in EHS pathophysiology, and systematic and long-term follow-up of case records could answer the questions for long-term management and prognosis. Funding sources This study was supported by the Agency of the Ministry of Education of the Slovak Republic for the Structural Funds of the European Union, Operational Programme Research and Development (Contract No. 034/2009/2.1/OPR&D). Conflict of interest The ICMJE Uniform Disclosure Form for Potential Conflicts of Interest associated with this article can be viewed by clicking on the following link: http://dx.doi.org/10.1016/j.sleep.2014.02.011.
3
References [1] Pearce JM. Clinical features of the exploding head syndrome. J Neurol Neurosurg Psychiatry 1989;52:907–10. [2] American Academy of Sleep Medicine. ICSD-2 – International Classification of Sleep Disorders, diagnostic and coding manual. 2nd ed. Westchester, IL: AASM; 2005. [3] Pearce JM. Exploding head syndrome. Lancet 1988;2(8605):270–1. [4] Ganguly G, Mridha B, Khan A, Rison RA. Exploding head syndrome: a case report. Case Rep Neurol 2013;5:14–7. [5] Kallweit U, Khatami R, Bassetti CL. Exploding head syndrome – ‘‘more than snapping of the brain’’? Sleep Med 2008;9:589. [6] Sachs C, Svanborg E. The exploding head syndrome: polysomnographic recordings and therapeutic suggestions. Sleep 1991;14:263–6. [7] Thorpy MJ, Plazzi G. The parasomnias and other sleep-related movement disorders. Cambridge: Cambridge University Press; 2010. [8] Pressman MR. Factors that predispose, prime and precipitate NREM parasomnias in adults: clinical and forensic implications. Sleep Med Rev 2007;11:5–30. [9] Testa SM, Krauss GL, Lesser RP, Brandt J. Stressful life event appraisal and coping in patients with psychogenic seizures and those with epilepsy. Seizure 2012;21:282–7. [10] Palikh GM, Vaughn BV. Topiramate responsive exploding head syndrome. J Clin Sleep Med 2010;6:382–3. [11] Salih F, Kleingebiel R, Zschenderlein R, Grosse P. Acoustic sleep starts with sleep onset insomnia related to a brainstem lesion. Neurology 2008;70:1935. [12] Chakravarty A. Exploding head syndrome: report of two new cases. Cephalalgia 2008;28:399–400. [13] Jacome DE. Exploding head syndrome and idiopathic stabbing headache relieved by nifedipine. Cephalalgia 2001;21:617–8.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.sleep.2014. 02.011.
Please cite this article in press as: Feketeova E et al. Exploding head syndrome – a rare parasomnia or a dissociative episode? A case report. Sleep Med (2014), doi:http://dx.doi.org/10.1016/j.sleep.2014.02.011
Contents lists available at ScienceDirect
Sleep Medicine journal homepage: www.elsevier.com/locate/sleep
Video-Clinical Corners
Exploding head syndrome – a rare parasomnia or a dissociative episode? A case report Eva Feketeova a,⇑, Jitka Buskova b, Matej Skorvanek a, Jana Mudra c, Zuzana Gdovinova a a
Faculty of Medicine, P. J. Safarik University and L. Pasteur University Hospital, Kosice, Slovak Republic 1st Faculty of Medicine and General Teaching Hospital, Charles University, Praha, Czech Republic c L. Pasteur University Hospital, Kosice, Slovak Republic b
a r t i c l e
i n f o
Article history: Received 17 November 2013 Received in revised form 14 January 2014 Accepted 17 February 2014 Available online xxxx Keywords: Exploding head syndrome Psychogenic non-epileptic seizures Parasomnia Seizure Focal epilepsy Carbamazepine
1. Introduction Exploding head syndrome (EHS) is characterized by a strange feeling in the head resembling a shooting, bump or blow to the head that occurs during the sleep–wake transition. Such attacks are painless, but they are unpleasant and sometimes terrifying [1]. The disorder is classified among parasomnias, and its etiology remains unknown [2]. 2. Case report A 55-year-old man was admitted to the hospital in order to investigate an increasing number of incidences of a terrifying ‘sudden intense bang or hit on the right side of the head, awakening him from sleep’. The episodes were occurring within an hour of falling asleep and without any obvious causal factor. They lasted from a few seconds to several minutes about once a week for 12 years. They were described as being an unpleasant experience but not a scary one. The patient was able to fall asleep within a few minutes after such episodes and to recall the experience in the morning. The awakenings did not cause daytime sleepiness. ⇑ Corresponding author. Address: Faculty of Medicine, P. J. Safarik University and L. Pasteur University Hospital, Tr. SNP 1, 04011 Kosice, Slovak Republic. Tel.: +421 1911628128. E-mail address: [email protected] (E. Feketeova).
The paroxysms started in 1999, when the patient described ‘a mental breakdown’ accompanied by alcohol excess with sleep deprivation, which resulted in a generalized tonic clonic seizure (GTCS). Because of the close incidence of the night episodes and the GTCS, their uniform character and short duration, the paroxysms were considered to be simple partial seizures. Carbamazepine (CBZ) was prescribed to the patient that same year. In 2012 the patient overcame a pulmonary embolism with the necessity of long-term warfarin treatment. To avoid potential drug interactions, CBZ was tapered off and replaced with levetiracetam. The changes in therapy were accompanied by increased frequency of the night episodes, and the patient was sent for a video-polysomnography (v-PSG) recording in March 2013. Standard blood tests and a detailed neurological exploration (brain magnetic resonance imaging, angiography) were normal. A 48 h v-PSG with a full-scalp electroencephalogram showed no epileptic origin of the attacks, and the two captured episodes (one during night sleep, the second during daytime sleep following sleep deprivation, Fig. 1, Video 1) were awakenings from N2 sleep. PSG showed normal sleep duration and architecture (total sleep time, 6 h 43.5 min; apnea–hypopnea index, 0.2/h; periodic leg movements index, 5.3/ h). The paroxysms were considered to be EHS. The patient was informed about their benign nature, and withdrawal of anti-epileptics was recommended. Three weeks later the paroxysms changed their nature: sleep-fixed attacks were replaced by right arm paresthesias with discharges irradiating over the right side of the face and lasting from several minutes up to 120 min. Terrifying sensory symptoms were relieved by knocking or cycling while lying down (Video 2). The attacks were occurring during daytime wakefulness or after daytime naps, when the patient was still lying in bed. They did not wake him from sleep; they occurred during sedating activities and never during working tasks. Having 13–20 attacks a day in May 2013, the patient was again admitted to the hospital. The attacks were considered to be paroxysmal non-epileptic seizures (episodes resembling epileptic seizures). Psychological examination revealed affective instability, obsessive tendencies, intrapsychical tension, paranoid reactions, anxiety, and sensory symptoms on a background of melancholia with prominent neurolability, suggesting psychogenic origin of non-epileptic seizures: psychogenic non-epileptic seizures (PNES). The psychiatrist diagnosed dissociative convulsions and recommended psychotherapy.
http://dx.doi.org/10.1016/j.sleep.2014.02.011 1389-9457/Ó 2014 Elsevier B.V. All rights reserved.
Please cite this article in press as: Feketeova E et al. Exploding head syndrome – a rare parasomnia or a dissociative episode? A case report. Sleep Med (2014), doi:http://dx.doi.org/10.1016/j.sleep.2014.02.011
2
E. Feketeova et al. / Sleep Medicine xxx (2014) xxx–xxx
Fig. 1. Exploding head syndrome (EHS) attack during daytime nap following sleep deprivation: polysomnographic (PSG) record. (A) A 30 s stage of N2 sleep preceding the EHS attack. (B) A 30 s PSG recording during the EHS episode – movement artifacts and following the awake state. There is no epileptic ictal activity or post-ictal electroencephalographic (EEG) attenuation or slowing present. Sequence of the channels on PSG: Fp1–F7, F7–T3, T3–T5, T5–O1, Fp2–F8, F8–T4, T4–T6, T6–O2, Fp1–F3, F3–C3, C3–P3, P3–O1, Fp2–F4, F4–C4, C4–P4, P4–02–scalp EEG electrodes placed according to the international 10–20 electrode placement system in bipolar longitudinal montage, REOG/LEOG L/R oculograms, EMG3–chin EMG), RED line Video 1 sequence.
Prescribing alprazolam and pregabalin for anxiety, together with psychotherapy, resulted in the disappearance of the daytime PNES and the reappearance (however, in reduced frequency in comparison with March 2013) of the EHS episodes in August 2013.
3. Discussion Exploding head syndrome is thought to likely represent a sensory variant of a hypnic jerk or sleep start [3]. Since the clinical description in 1988, there have been a few subsequent publications describing its clinical and polygraphic characteristics [4–6]. Polysomnography usually shows the EHS events arising from drowsiness [6]; however, the beginning from N2, as in our case, has previously been documented in a case report by Kallweit et al. [5]. The diagnosis of EHS in the presented case was based on a typical history and could not be explained by another sleep disorder or other disorder: the attacks were not accompanied by pain and thus could not be a variant of a specific headache; the short duration without vegetative symptoms did not support panic attacks; and the history and video-PSG recording excluded the diagnosis of par-
tial simple seizures despite their uniform character for 12 years and previously documented GTCS. PSG showed no wakefulness preceding the attacks, which is important for the diagnosis of a sleep-related dissociative disorder. Stress and anxiety seemed to trigger EHS and daytime PNES in the present case. The ability to eliminate daytime attacks and reduce night-time spells by psychotherapy supported by medication strongly suggests their role in the pathophysiology of both conditions. The importance of stress has already been suggested in the EHS [1,6,7] and NREM parasomnias [8]. The role of stress in PNES is also notable; however, stress is not considered to be more frequent or of higher intensity than in other patients, and PNES patients seem to lack effective coping mechanisms [9]. The question remaining unclear in this case is whether it represents a coincidence of ‘benign’ parasomnia EHS and PNES, or whether EHS and PNES are simply different manifestations of a dissociative disorder triggered by stress? The cornerstones of management in EHS are reassurance and education, as this is a benign condition that remits over time in most patients. There have been reports describing success with topiramate [10], clonazepam [11], clomipramine [6,12], and nifedipine [13]; however, pharmacological treatment is typically not
Please cite this article in press as: Feketeova E et al. Exploding head syndrome – a rare parasomnia or a dissociative episode? A case report. Sleep Med (2014), doi:http://dx.doi.org/10.1016/j.sleep.2014.02.011
E. Feketeova et al. / Sleep Medicine xxx (2014) xxx–xxx
needed. Long-term management of EHS attacks in the present case shows that there is no therapeutic response in the long-term administration of CBZ, even when titrated up to maximum tolerated dose during 12 years of treatment. Short-term complex treatment of the underlying mental disorder was more successful in reducing the frequency of EHS attacks, but did not completely eliminate them at three months’ follow-up. Detailed psychological exploration of EHS cases in the future could reveal important mechanisms in EHS pathophysiology, and systematic and long-term follow-up of case records could answer the questions for long-term management and prognosis. Funding sources This study was supported by the Agency of the Ministry of Education of the Slovak Republic for the Structural Funds of the European Union, Operational Programme Research and Development (Contract No. 034/2009/2.1/OPR&D). Conflict of interest The ICMJE Uniform Disclosure Form for Potential Conflicts of Interest associated with this article can be viewed by clicking on the following link: http://dx.doi.org/10.1016/j.sleep.2014.02.011.
3
References [1] Pearce JM. Clinical features of the exploding head syndrome. J Neurol Neurosurg Psychiatry 1989;52:907–10. [2] American Academy of Sleep Medicine. ICSD-2 – International Classification of Sleep Disorders, diagnostic and coding manual. 2nd ed. Westchester, IL: AASM; 2005. [3] Pearce JM. Exploding head syndrome. Lancet 1988;2(8605):270–1. [4] Ganguly G, Mridha B, Khan A, Rison RA. Exploding head syndrome: a case report. Case Rep Neurol 2013;5:14–7. [5] Kallweit U, Khatami R, Bassetti CL. Exploding head syndrome – ‘‘more than snapping of the brain’’? Sleep Med 2008;9:589. [6] Sachs C, Svanborg E. The exploding head syndrome: polysomnographic recordings and therapeutic suggestions. Sleep 1991;14:263–6. [7] Thorpy MJ, Plazzi G. The parasomnias and other sleep-related movement disorders. Cambridge: Cambridge University Press; 2010. [8] Pressman MR. Factors that predispose, prime and precipitate NREM parasomnias in adults: clinical and forensic implications. Sleep Med Rev 2007;11:5–30. [9] Testa SM, Krauss GL, Lesser RP, Brandt J. Stressful life event appraisal and coping in patients with psychogenic seizures and those with epilepsy. Seizure 2012;21:282–7. [10] Palikh GM, Vaughn BV. Topiramate responsive exploding head syndrome. J Clin Sleep Med 2010;6:382–3. [11] Salih F, Kleingebiel R, Zschenderlein R, Grosse P. Acoustic sleep starts with sleep onset insomnia related to a brainstem lesion. Neurology 2008;70:1935. [12] Chakravarty A. Exploding head syndrome: report of two new cases. Cephalalgia 2008;28:399–400. [13] Jacome DE. Exploding head syndrome and idiopathic stabbing headache relieved by nifedipine. Cephalalgia 2001;21:617–8.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.sleep.2014. 02.011.
Please cite this article in press as: Feketeova E et al. Exploding head syndrome – a rare parasomnia or a dissociative episode? A case report. Sleep Med (2014), doi:http://dx.doi.org/10.1016/j.sleep.2014.02.011
