Diagnosis of Exploding Head Syndrome

Dr. William Bailer
306 Laburnam Cresent
Rochester, NY 14620
SSN: 118-42-0102
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ALL of the information presented here is all of the most relevant that can be found in peer reviewed medical journals (a total of only 6 articles over 25 years), which I compiled in its entirety in this one doc file (except for the full text of the two most recent articles, both published in the last 5 months, that cost $35 each). It includes the entire literature on the subject (including 3 complete articles at the end), and a few descriptions of symptoms from internet forums (which are much more informative than what can be found in the very few medical articles that have been published).

All of the information is only about one specific named rare syndrome, which is not associated with any psychiatric problem, mental disease, or neurological disease. It also is not related to dreams, not imagined, and not hallucinations. It is a physical phenomenon, neurological or mechanical in origin (from the middle ear), and always occurs on the borderline of sleep or duing a disruption of sleep. Never the result of a dream, and never when in a deep sleep. It also never is perceived as a specific identifiable sound that is familiar, in other words, not a spoken word, not a dog bark, not a violin, etc. It is always described as a non-specific sudden, short noise, like a bang, although sometimes a low pitched buzzing noise is associated with it. It is experienced as a perception, not imagined. It is experienced as noise that is as real as any other. The way affected people come to know that it is not external is by the reportage of an awake person near them in the same room or adjoining room (usually husband or wife) who repeatedly report that they do not hear it. People who experience it alone typically look out the window or go outdoors to find out where the noise came from and assume that it was produced by another human, such as a gun shot or an object thrown at the house. The stress of dealing with people who do not believe what the afflicted person claims, especially doctors who assume it to be psychiatric in nature, can lead to alienation and distrust, along with the concurrent symptoms of sleep deprivation.

Symptoms published in medical journals, with dates:

2014 Dec "the perception of abrupt, loud noises when going to sleep or waking up. They are usually painless, but associated with fear and distress."

2014 Sept. "a sudden noise or explosive feeling experienced in the head occurring during the transition from wake to sleep or from sleep to wake." "sensations of explosions in the head during sleep or drowsiness ..."

2013 Jan "affected individuals awaken from sleep with the sensation of a loud bang."
"a tremendously loud noise as originating from within their head, usually described as the sound of an explosion, gunshot, door slamming, roar, waves crashing against rocks, loud voices, a ringing noise, a terrific bang on a tin tray, or the sound of an electrical buzzing. In some cases, an instant flash of what is perceived as video ‘static’ is reported both audibly and visually for a fraction of a second"
"a loud bang, shotgun, or bomb explosion, and some patients have reported a simultaneous flash of light ..."

2010 Aug "loud bang and buzzing noise at sleep onset" "There was video confirmation of her hearing a loud bang sound correlating with the transition from NREM stage 1 sleep to wake"

1991 June "unpleasant, even terrifying sensations of flashing lights and/or sounds during reported sleep ..." "sensations of explosions in the head during sleep or drowsiness"

1989 July "... their complaint had been greeted with incredulity if not frank disbelief." "with no preceding cause in the habits or events of days prior to the attack"

Symptoms of 30 people described in their own words, published in a medical journal:
(copied from an 1989 article below)

Under the column heading "During":
"Twilight" means "while going to sleep" or "almost asleep"
"sleep" means "while asleep," or "waking"

Sex now age Description/Symptoms: Associated with: During:
1 m 49 * loud bang suddenly wakes as if I've stopped breathing. Overactive mind sleep 2 m 40 7 (Doctor) explosion aura of floating. 4 attacks in all. when v. tired twilight 3 m 12y (Dentist) shotgun or thunderclap 20 attacks panic sleep 4 f 72 58 loud metallic noise 2/month twilight 5 m 66 * loud explosion varies, most nights concern, confused twilight 6 m 53 * explosion infrequently sleep 7 f 65 65 violent explosion to R. of centre single attack sat bolt upright twilight 8 f 68 5 (S Registered Nurse) explosive bangs continue after 63 yrs twilight 9 f * 25 clash of cymbals sleep 10 m 73 * many every night, door slamming on waking. dizzy head pain and panic sleep 11 m 68 20 monthly explosion: 'the earth moved" decreased with age twilight 12 m 77 67 explosion, weeks free tinnitus louder on waking sleep 13 f 56 8 violent bang, shaken, head feels tender sleep 14 f 5 8 child frightening explosion 2/month also sudden "drops" and palpitations sleep 15 m 67 65 single episode, sudden explosion in sleep sleep 16 f 78 64 infrequent explosions, electric shocks, tinnitus; sudden. when relaxed twilight 17 f 66 * terrific bang doing crosswords, dropping off [to sleep] twilight 18 f 79 79 isolated big bang hypertension sleep 19 f *yr explosion twilight 20 f 62 61 attacks: loud electric explosion sleep 21 f 26 years enormous roar so loud it could kill me twilight 22 f 56 43 attacks, massive explosion, like ightning striking house. sense of falling 23 f 65 many yrs crack of lightning or whiplash twilight 24 f ? 10 yr fierce explosions as if house crashed. ceased when stopped C2H5OH. sleep 25 f 68 62 noise as if head will burst open sleep 26 m 40 23 (Doctor) bouts of explosions, dropped beats stressed and insomnia. twilight+sleep 27 m 60 59 bomb like explosion attacks awakening [sleep] 28 f 60 58 explosive noise back of head twilight 29 m 60 (Doctor) explosion "like nightjerks" flash of light twilight 30 f 63 10yr loud snap like Xmas cracker heart thumping + silence like London blitz twilight Totals are: While going to sleep/twilight: 14 Awakened while asleep: 15

"Precursors" (also from medical journal articles);
"with no preceding cause in the habits or events of days prior to the attack"
"three afflicted physicians noted their own attacks to start and to recur when they were under personal stress or tired and overworked"
"may be an expression of emotional stress in the awake state."
"He attributed his fatigue to stress at work and the fact that he had to make greater effort to finish his daily tasks."

More descriptions of symptoms:

From comments found in a serious public forum about this specific syndrome at nosleeplessnights.com:
M Wood says:
June 5, 2013 at 6:14 pm
I think I’ve had this condition longer than I realised. For years now I’ve experienced the sound of something hitting the vinyl siding on my house. It usually jerks me awake and upon inquiry from my husband, he never hears a thing so I realised that it was in my mind. Each time it happens it is quite startling and is usually accompanied by rapid heartbeat and fear. After a few minutes spent calming down and realising it was nothing, I can usually get back to sleep. It has happened in twilight stage of sleep and also when waking.

Angela says:
February 17, 2014 at 12:38 am
I am occasionally startled awake in the middle of the night by what sounds like someone knocking loudly on the door, a bang from elsewhere in the house, but nothing like an explosion or screaming sound. My husband doesn’t hear the sounds, so I usually decide I imagined them, but they are still frightening and make it difficult for me to fall back to sleep. ...

Ryan Miller says:
June 30, 2014 at 10:42 pm
I’ve been having EHS episodes for the last 3+ years, usually around once a month. They were pretty terrifying at first. I literally thought someone was firing a shotgun either right outside my bedroom window, or in an apartment downstairs. The first time I very nearly called the police. The second and third time it happened, I realized that it must be in my head, but it is very confusing when your brain lies to you (telling you there is either a gunshot, or maybe something large crashing through the walls or to the ground). To add to the confusion: jerking awake causes my mattress to shake, which makes it feel like the vibration of the explosion is being transmitted from the floor through the bed (and is therefore real).

Sel Healy says:
November 25, 2013 at 5:37 pm
I have experienced this phenomenon for a number of years. It usually occurs just as I am falling asleep. The sound is that of a sharp explosion and is accompanied by a flash of light. It causes me to jump and every muscle to contract as though I had been frightened out of my wits. My heart races and I am momentarily terrified. I suspect it also happens while I am asleep as I occasionally awaken to a terrifyingly loud bang - as though someone has hit the side of the house behind my head. I am a 66 yr old widow who is supporting a daughter and her son and with 15 yrs of severe stress. The sounds started when my teaching career became unbearable.

Ellie says:
March 9, 2014 at 10:00 pm
For years I really struggled falling asleep. I would jerk awake or hear loud bangs and wake up and my husband (who was often awake still) would tell me there had been no noises. At first, I found they diminished when I would eat only protein late in the day. And if the phenomena were occurring, the only way to get it to stop was to get up and eat some protein. Now, I find that if I just take a multi-mineral supplement every day, I am an awesome sleeper. It is so great to be able to sleep! I actually discovered it accidentally when I started taking the minerals for a different reason. If I am without the supplements for more than a day, my symptoms return. Hang in there and keep trying things. There has to be something that works for you!


More comments from other public forums, each paragraph from a different message:

I had it for years and I actually thought it was the arsehole neighbor blowing up stuff. This guy thought every day was the 4th of July and most of his stuff was of the illegal variety. Then I suddenly realized that the cat never reacted and I realized that it wasn't coming from outside. I thought it was coming from in my head but I figured that was an illusion. Have them in spells and then they go away completely. It is hard to go back to sleep with a huge adrenal rush.

I tried to explain this to my doctor but she had never heard of it

"I usually have my explosion or gun shot when i’ve been asleep a while."

It always seems to be set-off by a small sound in the room before I’ve entered a deep sleep.

"I have experienced this scary nightime malidy for more that 5 yrs. Sometimes it sounds like an explosion, a bomb exploding. Sometimes it feels electrical, almost like a buzz. And on occasion (very rarely) I will notice a flash of light accompanying the explosion. This is very frightening to say the least. It immediately wakes you with a start from a sound sleep. There is never any pain involved and i am able to fall back to sleep immediately. I have recently made a huge discovery. I was thinking that any time i was on vacation for the last three years, this literally never happens. So, I thought…what am i doing at home that i am not on vacation? I briefly heard recently of the horrors of using aspartame (artificial sweetner). I drink every day glasses of a generic form of Crystal Light (contains aspartame). So, it dawned on me that I NEVER drink anything diet on vacation (mostly water) and wine etc…LOL. Anyway, for the last week I have not touched anything that contained this chemical, just cranberry juice etc…and low and behold. NO explosions!!! Last night i performed a little experiment. I drank two glasses of diet lemonade that contains aspartame. and Son of a u-know-t. FOUR expisodes of explosions last night."

sounds like a very loud clap in my head which instantly wakes me up.

"I was just about to fall into sleep and lose consciousness. Then suddenly, I heard buzzing sound that appeared to originate from within the head. The sound started softly and becoming louder and louder."

"Woke up about half an hour ago after a massive bang shook me awake. Thought someone had broken my window and I was terrified. checked out the house,as I was convinced someone must have broken in."

"I heard a big bang. Don’t know if it was real or just in my head; I looked outside, I looked on the news, but nothing. It sounded like a bomb dropping and exploding."

"Mine is a quick “buzzing” sound. Like a “game show buzzer” when someone gets a wrong answer. It’s very quick and very annoying. It startles me awake with a slight rush in my chest. I will fall back asleep and it happens again…maybe 3 or 4 more times."

"Drop off to sleep and immediately have electrical arcing and buzzing and ringing.. I wake up with it. Recently A BOMB went off in my head… I was afraid to reach down to see if my body was still there. It is too weird."

"Strange, sound quite like a door bang or a gunshot. I’ve been under stress so I’m guessing that’s the reason why I’m experiencing it"

I truly believe anxiety is what triggers them.

"I totally agree that by reducing stress level, the syndrome can be soothed."

The noise is similar to someone smacking my windows with the palms of their hands as hard as they could, to the point just before the window breaks. Its terrifying.

it sounds like a bolt of lightning, like a really loud electrical clack. And that’s the beginning of it. Then it’s a metallic cling, or pang, like a hammer hitting an anvil. And then it’s like an explosion - like a ripping and tearing kabam. And all of those things, they’re all compressed down to three milliseconds. And it’s loud.

"Explosions, breaking glass, cymbols, whistling, crunching sounds the list goes on. I was diagnosed with acute stress reaction in 2002 but this was untreated. I have recently been diagnosed with chronic ptsd as a result of the incident in 2002."

"My experience is “electrical”. It happens either when I’m about to enter actual sleep, or when I’m about to wake up… It’s starts as a low current-like noise that I can actually hear, but then it sounds & feels like someone suddenly ‘turning up the power’… like an electric surge"

"The noise was like a bomb being dropped on the roof of my house, the noise was seriously loud and extremely disturbing. I also experienced a shake.I asked my mum what that noise was and was everything ok…and she looked at me like i was mad and thought i was dreaming…so i thought that too. I just thought I had a nightmare. Then just now it happened again…the EXACT same noise, shake and scared feeling. I asked my sister what the noise was….and she said there was absolutely no noise what so ever. I was so so so so sure of the noise and sensation this time and i thought there was something seriously wrong with me and my brain!!!I"

"The ‘rifle-shots, ‘ loud, short,bangs, or another fast noise (I cannot describe) really began to scare me. They have been happening for about a month or two. Not every night, but at least 2-3 times a week. Scare the bejaysus out of me when they happen."

"sometimes sounds like a very loud bomb exploding, sometimes like a loud, explosive gunshot and sometimes like a very loud bang. Usually I am under some sort of unusual stress when this happens."


-------------------------- End of symptoms --------------------------------------------------------------------------------


The name of the syndrome is, unfortunately, and inappropriately, "Exploding Head Syndrome," which is not even a description of the syndrome. That expression was coined by the first writer who wrote about it in the early 1980's, and it stuck. That is what everyone else since has continued to call it. A more appropriate name may be "Internal sudden loud noise associated with sleep but not dreams, syndrome."

The following information about drugs has been copied directly from medical journals:
All mentions of drugs from all of the relevant articles, exact quotes are in abstracts and articles below these lists:
I looked up each drug and added some notes about it:
Possibly effective or helpful drugs:
(and see "Druge that don't work")
clonazepam a benzodiazepine, for anxiety, and muscle relaxant (clonezapam a common misspelling)
"it really works!!!!!!!!!!!! I tak 1 mg appox. half an hour before I go to bed and haven’t had any attacks since!"
Calcium channel blockers like
nifedipine relaxes blood vessels and heart, lowers blood pressure
flunarizine (not available in the United States)
have been found to be effective in reducing the frequency or resolution of the EHS symptoms."
topiramate anticonvulsant
"lessened the intensity of events but did not diminish their frequency"
"... safe method to treat these events with topiramate medication"
"At a daily dose of topiramate 200 mg, ... bang had significantly improved, and now sounded like a low buzzing noise"
clomipramine a tricyclic antidepressant (clomipramnine as misspelled in some medical articles)
"three patients experienced immediate relief"
tricyclic antidepressants (see the three tricyclics that don't work, below)
"helpful in some patients"
From the forum quoted above, an individual says a mineral supplement works:
"take a multi-mineral supplement every day"
[I sent a message to this individual asking what minerals are in his suppement]
Drugs tried that have not worked:
trimipramine a tricyclic antidepressant
amitriptyline a tricyclic antidepressant
doxepin a tricyclic antidepressant
citalopram a selective serotonin reuptake inhibitor (SSRI) antidepressant
chemical structure unrelated to that of other SSRIs or tricyclics
valproic acid, anticonvulsant
gabapentin anticonvulsant, also used for nerve pain
propranolol beta blocker, slows heart rate which lowers blood pressure
oxycodone opioid pain killer, much like codeine

This information is not from medical journals, but may be worth trying.
Each treatment has been reported by only one person:
Aspartame avoidance see the one personal report above that avoiding aspartame stopped or reduced the noises
Effexor "I take effexor 37 mg and then I don’t get them. If I stop taking it, they come back."
Mineral supplements see the one personal report above that a mineral supplement stopped or reduced the noises
Exactly which minerals is unknown. I messaged the forum member but no response to my question.

================================================================================

List of all the relevant medical journal articles and their sources:
(including all of the articles (3) that I could find for free are copied below in their entirety, which I have also downloaded as PDF's)
Articles still to find (or buy; most are $35), which do have abstracts:
2014 Dec (or Epub 2014 Mar 13)
Exploding head syndrome.
Sharpless BA
Sleep Med Rev. 2014 Dec;18(6):489-493. doi: 10.1016/j.smrv.2014.03.001. Epub 2014 Mar 13.
("Sleep Medicine Reviews," not the regular Sleep Medicine journal)
* Elsevier Science
* Clinical Key
* Gale Databases I COULD NOT FIND IT IN THE GALE DATABASES as of 12-21-2014; it may be too soon?

Author information
* Department of Psychology, Washington State University, 364 Johnson Tower, Pullman, WA 99163, USA. Electronic address: basharpless@gmail.com.
Abstract (summary)
Exploding head syndrome is characterized by the perception of abrupt, loud noises when going to sleep or waking up. They are usually painless, but associated with fear and distress. In spite of the fact that its characteristic symptomatology was first described approximately 150 y ago, exploding head syndrome has received relatively little empirical and clinical attention. Therefore, a comprehensive review of the scientific literature using Medline, PsycINFO, Google Scholar, and PubMed was undertaken. After first discussing the history, prevalence, and associated features, the available polysomnography data and five main etiological theories for exploding head syndrome are summarized. None of these theories has yet reached dominance in the field. Next, the various methods used to assess and treat exploding head syndrome are discussed, as well as the limited outcome data. Finally, recommendations for future measure construction, treatment options, and differential diagnosis are provided.

2014 Sept
Exploding head syndrome: six new cases and review of the literature. Sage or HighWire $36
Frese A, Summ O, Evers S
Cephalalgia. 2014 Sep;34(10):823-7. doi: 10.1177/0333102414536059. Epub 2014 Jun 6.
Author information
* 1Akademie für Manuelle Medizin, University of Münster, Germany fresea@uni-muenster.de.
* 2Department of Neurology, University of Münster, Germany.
* 3Department of Neurology, Krankenhaus Lindenbrunn, Germany.
Abstract:
BACKGROUND:
Exploding head syndrome (EHS) is characterized by attacks of a sudden noise or explosive feeling experienced in the head occurring during the transition from wake to sleep or from sleep to wake.
METHODS:
We present six new cases extending the clinical experience with the syndrome. We also reviewed all available cases from the scientific literature and evaluated the typical features of EHS.
RESULTS:
The female to male ratio is 1.5 to 1. The median age at onset is 54. On average, one attack per day to one attack per week occurs. Some patients suffer from several attacks per night. In about half of all patients, a chronic time course can be observed but episodic or sporadic occurrence is also common. The most frequent accompanying symptoms beside the noise are fear and flashes of light. Polysomnographic studies do not reveal any specific sleep pattern associated with EHS. Tricyclic antidepressants are helpful in some patients. However, most patients do not need treatment because of the benign nature of the syndrome.
CONCLUSION: EHS is a well-defined disease entity with a benign nature.

1991 June
The exploding head syndrome: polysomnographic recordings and therapeutic suggestions.
Sachs C, Svanborg E Sweden, NO PDF OR ELECTRONIC VERSION
Sleep. 1991 June ; 14(3): 263-6
Author information: Department of Neurology, Karolinska Hospital, Stockholm, Sweden.
Abstract
Attention has recently been drawn to a condition termed the exploding head syndrome, which is characterized by unpleasant, even terrifying sensations of flashing lights and/or sounds during reported sleep. Nine patients complaining of sensations of explosions in the head during sleep or drowsiness were investigated with polysomnographic recordings. None of them had any neurological disorder. Five patients reported explosions during the recording sessions. According to the recordings, the attacks always took place when the patients were awake and relaxed. In two cases abrupt electroencephalographic (EEG) and electromyographic changes indicating increasing alertness were recorded at the time of the reported attacks. In the remaining three cases no EEG changes were seen. Thus, there were no indications of an epileptic etiology to the condition. In all patients the symptoms ameliorated spontaneously with time. The severity of the symptoms was reduced by reassurance of the harmlessness of the condition. Clomipramine was prescribed to three patients who all reported immediate relief of symptoms. It is concluded that symptoms of this type are probably not true hypnagogic phenomena but may be an expression of emotional stress in the awake state.
[hypnagogia: experience of the transitional state from wakefulness to sleep:]

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The available HTML versions of downloaded articles also downloaded in PDF form:
---------
2013 Jan
This is a complete available HTML version, also downloaded in PDF form:
Case Rep Neurol. 2013 Jan-Apr; 5(1): 14-17.
Published online Jan 22, 2013. doi: 10.1159/000346595
PMCID: PMC3573786
Exploding Head Syndrome: A Case Report
Gautam Ganguly, Banshari Mridha, Asif Khan, and Richard Alan Rison*
Neurological Consultants Medical Group, Los Angeles County Medical Center, University of Southern California - Keck School of Medicine, Los Angeles, Calif., USA
*Richard Alan Rison, MD, Medical Director, PIH Health Hospital Stroke Center, 12401 Washington Blvd., Whittier, CA 90606 (USA), E-Mail ude.csu@nosir

Abstract
Introduction
Exploding head syndrome (EHS) is a rare parasomnia in which affected individuals awaken from sleep with the sensation of a loud bang. The etiology is unknown, but other conditions including primary and secondary headache disorders and nocturnal seizures need to be excluded.
Case Presentation
A 57-year-old Indian male presented with four separate episodes of awakening from sleep at night after hearing a flashing sound on the right side of his head over the last 2 years. These events were described ‘as if there are explosions in my head’. A neurologic examination, imaging studies, and a polysomnogram ensued, and the results led to the diagnosis of EHS.

Conclusion
EHS is a benign, uncommon, predominately nocturnal disorder that is self-limited. No treatment is generally required. Reassurance to the patient is often all that is needed.
Key words: Exploding head syndrome, Parasomnia, Headache disorder
Introduction
Nocturnal headaches comprise a variety of primary and secondary headaches including migraines, cluster headaches, and hypnic headaches. Exploding head syndrome (EHS) is an odd and rare parasomnia that can mimic nocturnal headache syndromes and seizures. Patients experience a tremendously loud noise as originating from within their head, usually described as the sound of an explosion, gunshot, door slamming, roar, waves crashing against rocks, loud voices, a ringing noise, a terrific bang on a tin tray, or the sound of an electrical buzzing. In some cases, an instant flash of what is perceived as video ‘static’ is reported both audibly and visually for a fraction of a second [1].

We present a case of a male patient diagnosed with EHS following history taking, neurologic examination, and investigations.

Case Presentation
A 57-year-old, right-handed Indian male presented for evaluation of four separate episodes of awakening after hearing a flashing sound on the right side of his head over the last 2 years. He described these events ‘as if there are explosions in my head’.

The patient stated that all episodes occurred in the early part of his sleep and that these flashing sounds awaken him with a sudden electric jolt-like sound in the right side of his head. He was able to pinpoint the moment when the symptoms started during the night. Once awakened, he did not feel any pain or headaches. All four episodes had identical symptoms and he was in the supine position sleeping in his bed at night when the events occurred. These episodes were not associated with any autonomic symptoms, focal weakness, visual or speech disturbances, seizures, postictal confusion, or incontinence.

His other neurological complaints included mild forgetfulness and increased tiredness and fatigue, mostly towards the end of the day. He denied any difficulty in falling asleep or any disturbance in the legs to suggest restless leg syndrome. He was known to snore, but there was no evidence of any witnessed apneas or snorting. He generally woke up feeling refreshed, but as the day progressed he became more tired but not sleepy. He attributed his fatigue to stress at work and the fact that he had to make greater effort to finish his daily tasks. He denied any other history of regular headaches, migraines, or cluster headaches in the past. His family history was non-contributory except for hypertension and diabetes.

The patient's past medical history was significant for hypertension, hypercholesteremia, long-standing type 2 diabetes mellitus, and a hemorrhagic stroke 3 years ago with secondary mild cognitive impairment, but fortunately without physical impairment.

On physical exam, he was afebrile and normotensive without tachypnea. His body mass index was 30 with a Mallampati score of 1, and his general physical examination was unremarkable. His neurological exam suggested a right temporal homonymous hemianopsia from his previous stroke, but the rest of the results was within normal limits.

The investigations included a magnetic resonance image and a magnetic resonance angiogram of the brain, which did not show any new vascular events. The magnetic resonance image of the brain demonstrated left temporal-occipital gliosis corresponding with the patient's prior stroke. The magnetic resonance angiogram of the brain was within normal limits. An electroencephalogram (EEG) revealed slowing in the left parietal region on an otherwise normal background. There was no evidence of any epileptiform discharges. A polysomnogram did not reveal any clinically significant obstructive sleep apnea/hypopnea syndrome.

No specific treatment other than reassurance was given. The patient was followed up 6 months later for a routine visit and did not have an interval recurrence of symptoms.


Discussion
EHS is an uncommon, usually nocturnal parasomnia that arises from the transition between different sleep stages. Patients complain of a sensation of a sudden explosive noise in the head, usually while falling asleep, which then awakens them. The sensation is very brief (typically for a few seconds only) and disappears completely when awake, although it may recur on further sleep attempts. The noise has been described as a loud bang, shotgun, or bomb explosion, and some patients have reported a simultaneous flash of light as deep sleep is entered from the lighter stages of sleep. There is no actual headache or persistent pain, but some patients may experience a brief, mild jab-like sensation. The syndrome is often so disturbing that affected individuals are understandably quite concerned. Although EHS has been reported in patients as young as 10 years, the age of onset is usually after age 50 as in hypnic headache, and the condition has a slight female preponderance. The attacks occur with variable frequency for a few weeks or months. Attacks can be one-time events or can recur with attacks increasing or decreasing over time, sometimes with no incidents over long periods of time [1, 2].

In the present case, the differential diagnoses included nocturnal headache syndromes and seizures. Nocturnal headache syndromes include hypnic headache, cluster headaches, and migraine. All of these headache disorders usually cause the patient to awaken with an actual headache, which our patient did not have. Similarly, cephalgias occurring from subarachnoid etiologies, space occupying lesions, or obstructive sleep apnea result in persistent moderate to severe headaches, which our patient did not have either. Nocturnal seizures are prone to occur in the non-rapid eye movement sleep, but patients are mostly amnestic about the seizures. In contrast, our patient had a clear recollection of the events, without any postictal confusion and a with normal EEG. Additionally, imaging studies along with a polysomnogram also excluded the above causes. In particular, there was no evidence of periodic limb movements of sleep or another parasomnia such as non-rapid eye movement sleep without atonia. Given the history and investigations, it was therefore felt that our patient's diagnosis was EHS.

The cause of EHS is unknown. Some possibilities are a sudden movement of a middle ear component or the Eustachian tube, or perhaps a brief temporal lobe complex partial seizure (though EEG studies have generally been reported as normal) [3]. There is a correlation with stress or extreme fatigue. EHS has also been linked to rapid withdrawal from benzodiazepines and selective serotonin reuptake inhibitors [4] (which our patient was not taking).

EHS is benign and self-limited, and no specific drug therapy is warranted. Reassurance reduces anxiety; however, in some patients, EHS is very troublesome and can cause sleep-onset insomnia. For this small subgroup of patients, medications including calcium channel blockers like nifedipine [5] or flunarizine (not available in the United States) [6], topiramate [7], or clomipramine have been found to be effective in reducing the frequency or resolution of the EHS symptoms.

Conclusion
EHS is a benign, uncommon, predominately nocturnal parasomnia that can mimic primary and secondary headache disorders along with seizures. No treatment is generally required as the condition is self-limiting. Reassurance to the patient is often all that is needed.

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2010 Aug
This is a complete available HTML version, also downloaded in PDF form:
J Clin Sleep Med. Aug 15, 2010; 6(4): 382-383.
PMCID: PMC2919670
Topiramate Responsive Exploding Head Syndrome
Gaurang M. Palikh, M.D. and Bradley V. Vaughn, M.D.
Department of Neurology, University of North Carolina School of Medicine, Chapel Hill, NC
Address correspondence to: Gaurang Palikh, M.D., Neurology Resident, Department of Neurology, University of North Carolina at Chapel Hill, 170 Manning Drive POB BLDG., CB #7025, Chapel Hill, NC 27599-7025Phone: (919) 966-4401Fax: (919) 966-6501, ; Email: ude.cnu.ygoloruen@gletap
Bradley V. Vaughn, M.D., Vice Chair, Department of Neurology, Chief of the Division of Sleep and Epilepsy, Professor of Neurology and Biomedical Engineering, University of North Carolina School of Medicine, CB# 7025, 2122 Physicians Office Building, Department of Neurology, University of North Carolina, Chapel Hill, NC 27599-7025Phone: (919) 966-8178Fax: (919) 966-2922, ; Email: ude.cnu.ygoloruen@bnhguav
Abstract
Exploding head syndrome is a rare phenomenon but can be a significant disruption to quality of life. We describe a 39-year-old female with symptoms of a loud bang and buzz at sleep onset for 3 years. EEG monitoring confirmed these events occurred in transition from stage 1 sleep. This patient reported improvement in intensity of events with topiramate medication. Based on these results, topiramate may be an alternative method to reduce the intensity of events in exploding head syndrome.
Exploding head syndrome (EHS) is a rare phenomenon characterized by a painless loud noise at the onset of sleep. The phenomenon was reported initially in 1920 by Armstrong-Jones when he referred to it as “snapping of the brain.” The term exploding head syndrome was coined by Pearce in 1989 in a paper in which he described 40 patients with EHS.2 Given the rarity of the disorder, no treatment studies have been done. A thorough review of the literature revealed a few case reports and opinion papers. Events described in the literature start spontaneously and are not associated with hypnic jerks. Although treatments have been reported, no trials have been performed. In these cases and case series, there was no mention of topiramate being prescribed for these events.

REPORT OF CASE
A 39-year-old female reported symptoms of a loud bang and buzzing noise at sleep onset for 3 years. She said that, if the sound was external, her “husband should be able to hear it downstairs when she was up in her bedroom.” Associated with this noise, she experienced brief jerking movement of her head, leg, or arms at sleep onset on a daily basis. She noted these symptoms for years; because of the increase in intensity and frequency, she saw a neurologist. The patient had become anxious about these events, fearing that they were a hallmark of more serious medical issues. Her neurological exam, laboratory test results, and neuroimaging were normal. Because of the stereotypic nature of the events and the level of disturbance to the patient, she was admitted for continuous video EEG monitoring for 4 days. Coincidently, the patient's neurologist prescribed topiramate 50 mg twice a day for migraine prophylaxis.

The topiramate was tapered off during the first 2 days of monitoring. There was video confirmation of her hearing a loud bang sound correlating with the transition from NREM stage 1 sleep to wake (Figure 1). She had normal sleep architecture, and a total of 11 typical episodes were recorded, most associated a leg jerk. No evidence of seizure activity was noted during monitoring. The patient had been placed on topiramate for migraine headaches, and this was increased to 150 mg daily. Two months after admission, she reported improvement in the intensity of the noise. At a daily dose of topiramate 200 mg, the patient reported the bang had significantly improved, and now sounded like a low buzzing noise. The frequency of the events was unchanged, but the intensity of the events decreased to the point of being mildly noticeable. She had marked improvement in subjective ability to fall asleep and felt these events were no longer disruptive. Figure 1 [inserted] [There are only very slight changes evident in Figure 1. No large disturbance.] Electroencephalographic recording during an event during which the patient heard the loud noise. DISCUSSION We describe a case of EHS in which topiramate diminished the intensity but not the frequency of the events. Our case has classical features of EHS and some interesting observations. For our patient, some of her events were associated with a hypnic jerk. This was not described by Pearce in his original description. Our patient also had history of migraines. Migraines or headaches were also mentioned in three of seven reports; otherwise, no linkage has been made between EHS and headaches. Our patient also noted a family history of these events. The patient's mother and daughter have similar symptomatology, rasing the possibility that this may be hereditary. There is no literature on the inheritance of this phenomenon. Jacome postulated that EHS might arise from transient calcium channel dysfunction, such as seen in familial hemiplegic migraines resulting from mutation in CACNA1A gene located in chromosome 19. The gene encodes for the alpha 1A subunit of the neuronal P/Q type voltage-gated calcium channel. Mechanism of topiramate is on P type calcium channel. However, to date, no mechanism has been linked to EHS. Various therapies have been tried to improve the EHS events. Case reports have implicated that clonezapam, clomipramnine, and nifedipine6 have been helpful. There are two case reports by Chakravarty that show benefit with flunarizine. Treatments with doxepin, citalopram, trimipramine, and amitriptyline were unsuccessful.3 Jacome found no benefit from valproic acid, amitriptyline, propranolol oxycodone, or gabapentin. Our case had improvement in intensity of events with the use of topiramate. Exploding head syndrome is a rare phenomenon but can be disruptive. This case report demonstrates and alternative and safe method to treat these events with topiramate medication. ---------------------------------------------------------------------------------------------------- Downloaded only in PDF form: 1989 July Text below is the most relevant text copied from the PDF: J Neurol Neurosurg Psychiatry. 1989 Jul ; 52(7): 907-10 Clinical features of the exploding head syndrome. 50 patients Pearce JM [Pearce J M S] From the Department ofNeurology, Hull Royal Infirmary, Hull, UK SUMMARY Fifty patients suffering from the "exploding head syndrome" are described. This hitherto unreported syndrome is characterised by a sense of an explosive noise in the head usually in the twilight stage of sleep. The associated symptoms are varied, but the benign nature of the condition is emphasised and neither extensive investigation nor treatment are indicated. Relevant segments copied from the PDF article: "a sense of an explosive noise in the head usually in the twilight stage of sleep." "The syndrome in essence consists of an hitherto unreported benign symptom characterised by a sense of explosion in the head, confined to the hours of sleep, which is harmless but very frightening for the sufferer. It receives no mention in current texts." [as of 1989, but maybe also much later] One case report: She described: "Being wakened by a sudden bang in the head, as if my head was bursting with a flash of light over both fields of vision, after which I would be dazed for a split second" and would "Come round, terrified, my heart thumping. There was no pain, just a frightening sense of explosion". 50 cases [including from 40 patients who sent letters to the author from 3 continents after the first published description of the syndrome by this same author in 1988] Table 2 Showing salient clinicalfeatures of 30 patients Under the heading "During": "Twilight" means "while going to sleep" or "almost asleep" "sleep" means "while asleep," or "waking" Sex Age Onset Description: Associated with: During: I m 49 * loud bang suddenly wakes as if I've stopped breathing. Overactive mind sleep 2 m 40 7 (Doctor )explosion aura of floating. 4 attacks in all. When v. tired twilight 3 m 12y ago (Dentist) shotgun or thunderclap 20 attacks panic sleep 4 f 72 58 loud metallic noise 2/month twilight 5 m 66 * loud explosion varies, most nights concern, confused twilight 6 m 53 * explosion infrequently sleep 7 f 65 65 violent explosion to R. of centre single attack sat bolt upright twilight 8 f 68 5 (SRN) explosive bangs continue after 63 yrs twilight 9 f * 25 clash of cymbals sleep 10 m 73 * many every night, door slamming on waking dizzy head pain and panic sleep 11 m 68 20 monthly explosion: 'the earth moved" decreased with age twilight 12 m 77 67 explosion, weeks free tinnitus louder on waking sleep 13 f 56 8 violent bang, shaken, head feels tender sleep 14 f 58 child frightening explosion 2/month also sudden "drops" and palpitations sleep 15 m 67 65 single episode sudden explosion in sleep sleep 16 f 78 64 infrequent explosions, electric shocks, tinnitus; sudden when relaxed twilight 17 f 66 * terrific bang doing crosswords dropping off [to sleep] twilight 18 f 79 79 isolated big bang hypertension sleep 19 f * yr explosion twilight 20 f 62 61 attacks: loud electric explosion sleep 21 f 26 years enormous roar so loud it could kill me twilight 22 f 56 43 attacks, massive explosion, like ightning striking house sense of falling 23 f 65 many yrs crack of lightning or whiplash twilight 24 f ? 10 yr fierce explosions as if house crashed ceased when stopped C2H5OH sleep 25 f 68 62 noise as if head will burst open sleep 26 m 40 23 (Doctor) bouts of explosions, dropped beats stressed and insomnia, twilight+sleep 27 m 60 59 bomb like explosion attacks awakening 28 f 60 58 explosive noise back of head twilight 29 (Dr) m 60 explosion "like nightjerks" flash of light twilight 30 f 63 10yr loud snap like Xmas cracker heart thumping + silence like London blitz twilight *age at onset or duration in years as stated. * patients' own words. ... occurs predominantly in the twilight stage as the patient is dropping off to sleep, or, less often if they waken during the night and again fall asleep. Some patients simply report it waking them from sleep but, without sleep monitoring it is not possible to know whether this occurs during stages I to 4 or if they have woken briefly and are falling asleep again in another twilight stage. All sufferers report noise, not pain. The dramatic nature is evident in their words "enormous roar, so loud it could kill me"; some, however, say that it can be mild and infrequent. The terror induced is notable in every case, until some degree of acceptance is achieved after many years in which they have maintained good health. Preceding events are generally unremarkable, but three physicians noted attacks to start and to recur when they were under personal stress or tired and overworked. Associated symptoms are documented by some sufferers. Five (10%) report a simultaneous flash of light; three (6%) reported a curious sensation as if they had stopped breathing and had to make a deliberate effort to breathe again-"an uncomfortable gasp". In almost every case (47/50) the after effects fear, terror, palpitations or forceful heartbeat are mentioned. Five subjects described tinnitus, three of whom had deafness, but the tinnitus was the usual daytime phenomenon and the nocturnal explosion was qualitatively different in each case. ... many said they had been ashamed to mention it to their doctors or that their complaint had been greeted with incredulity if not frank disbelief. The victim is aroused from sleep by a violent sensation of explosion in the head. It occurs abruptly with apparently great force, yet it is not a pain. By the time the sufferer is wide awake it has gone, but not surprisingly it leaves in its wake a sense of great consternation and sometimes momentary difficulty in breathing, tachycardia and sweating. It may occur for a few weeks or months, then spontaneously disappear, or, may recur irregularily every few days, weeks or months for much of a lifetime, yet with no preceding cause in the habits or events of days prior to the attack. What causes the bomb-like noise remains a mystery. A momentary (almost ictal) disinhibition of the cochlea or its central connexions in the temporal lobes might produce such a phenomenon; less likely is a sudden involuntary movement of the tympanum or the tensor tympani. The likeliest explanation is to class it with the other physiological phenomena such as nocturnal myoclonus, which mark the transition from wakefulness to grade I sleep. Normal sleep cycles of non-REM sleep alternate with REM sleep at intervals of about 90 to 100 minutes, with three to five cycles per night. Articles to still find (or buy; most are $35); which do not have abstracts: 2008 July Exploding head syndrome--more than "snapping of the brain"? Kallweit U, Khatami R, Bassetti CL Sleep Med. 2008 Jul ; 9(5): 589 Elsevier Science, Clinical Key, Gale Databases 2001 June Exploding head syndrome. Evans RW, Pearce JM Headache. 2001 Jun ; 41(6): 602-3 Wiley, Blackwell Publishing, EBSCO, Ingenta plc, Ovid Technologies, Inc. 1988 Sept Exploding head. Lancet, Elsevier Science, Gale [no author] Lancet. 1988 Sep 10; 2(8611): 625-6 1988 July Exploding head syndrome. Lancet, Elsevier Science, Gale Pearce JM Lancet. 1988 Jul 30; 2(8605): 270-1 Articles for which downloading or researching is completed: 2010 Jan OF LITTLE RELEVANCE except for what is copied here From an English abstract, the article is in Japanese: Brain Nerve. 2010 Jan;62(1):85-8. [Case of exploding head syndrome]. [Article in Japanese] Okura M1, Taniguchi M, Muraki H, Sugita H, Ohi M. "... video-polysomnography showed 11 EHS attacks occurred during stage N1 and stage N2; 2001 June OF LITTLE RELEVANCE The exploding head syndrome. Green MW Curr Pain Headache Rep. 2001 Jun ; 5(3): 279-80 That is the end of information from medical journals. ---------------------------------------------- END -------------------------------------------------------------------------
Age Onset