Headache Medicine, v.2, n.4, p.209-211, Oct/ Nov/Dec. 2011 209

Exploding head syndrome – the early steps

Síndrome da cabeça explodindo – os primeiros passos

ABSTRACTABSTRACT

ABSTRACT

Exploding head syndrome is a rare entity associated with

migraine that occurs during sleep onset. A male migraine

with aura patient presented with episodes of abrupt awakening

following perceptions of sounds resembling a speeding up

motorcycle engine interspersed with bursts of exhaust explosions

like noises, accompanied by an exploding sensation in the

head. The patient presented in the evolution of self-limited

period of headache chronicity. This syndrome has been

associated with an atypical form of acoustic aura that often

leads to migraine chronification.

Keywords: Keywords:

Keywords: Acoustic aura; Exploding head syndrome;

Migraine.

RESUMORESUMO

RESUMO

A síndrome da cabeça explodindo é uma entidade rara

associada com a migrânea que ocorre durante o início do

sono. Um paciente do sexo masculino com migrânea com

aura apresenta episódios de despertar súbito após perceber

um som como uma motocicleta acelerando intercalada com

estouros de um escapamento. O paciente evoluiu com período

autolimitado de cronificação da cefaleia. Esta síndrome tem

sido relacionada a uma forma atípica de aura acústica e

aparenta intima relação com cronificação da migrânea.

alavrasalavras

alavras

chave:chave:

chave: Aura acústica; Migrânea; Síndrome da

cabeça explodindo.

CASE REPORTCASE REPORT

CASE REPORT

Elcio Juliato Piovesan

1,2

, Pedro André Kowacs

, Helder Granhold Campos

, Lucas Pires Augusto

Lucas Coluni

, Lineu Cesar Werneck

1,2

Neurology Service, Internal Medicine Department, Hospital de Clínicas da

Universidade Federal do Paraná (UFPR), PR, Brazil

Experimental Laboratory, Health Sciences Sector (LESCS), Universidade Federal do Paraná (UFPR), PR, Brazil

Faculdade de Medicina, Universidade Federal do Paraná, PR, Brazil. Sponsored by a grant from Conselho

Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Piovesan EJ, Kowacs PA, Campos HG, Augusto LP, Coluni L, Werneck LC

Exploding head syndrome – the early steps. Headache Medicine. 2011;2(4):209-11

INTRODUCTION

Exploding head syndrome (EHS) is a rare

phenomenon characterized by a painless loud noise at

the onset of sleep.

(1)

Armstrong-Jones described it for the

first time in 1920 and referred to it as "snapping of the

brain".

(2)

Pearce coined the name "exploding head

syndrome in 1989.

(3)

This is a rare benign sleep-wake

transition disorder of unknown aetiology.

(4)

The attacks are

characterized by a sudden "bomb-like explosion" or

"shotgun" noise felt in the head and in 10-20% of patients

there is also a sensation of "flashing lights".

(4)

The attacks

are not painful but are unpleasant.

(4,5)

This phenomenon

occurs in relaxed wakefulness or at the transition from

wakefulness to sleep.

(5)

The sensation lasts for a few seconds

only and disappears completely when awake, although it

may recur on further attempts to fall asleep.

(6)

The onset is

usually over the age of 50 years old and there is a slight

female preponderance.

(3)

The attacks occur with variable

frequency (from seven in one night to one in a few weeks

or months).

(6)

Symptoms such as nausea and vomiting did

not occur.

(6)

The vast majority of patients with EHS are

migraine with aura patients. Reports of patients with EHS

preceding the onset of migraine attacks suggest that EHS

can be considered as a form of migraine aura.

(7)

Here we present a new EHS case and compare it

characteristics to those of the cases described in the

literature.

210

Headache Medicine, v.2, n.4, p.209-211, Oct/ Nov/Dec. 20

PIOVESAN EJ, KOWACS PA, CAMPOS HG, AUGUSTO LP, COLUNI L, WERNECK LC

CASE REPORT

A 45-year-old man with a five years history of episodic

migraine with fortification spectra aura described a

peculiar sensation in the head, occurring once a year,

similar to the noise of an exploding bomb only at night

while going off to sleep. The "explosion" would wake him

up and disappear completely at the moment he woke

up. This would make him wake up extremely scared and

tachycardic. Regarding the last episode, the patient

described a sound like the one of a motorcycle being

accelerated followed by exhausting pipe bursts. Three of

these sequences of sounds were perceived until the patient

was awake (Figure). The patient observed a close

relationship to anxiety. After EHS episodes, the patient

reported migraine exacerbation, lasting 45 days. The

headache has been well described as migraine:

alternating unilateral, throbbing, disabling and associated

to nausea, phonophobia and photophobia, besides

important and persistent visual phenomena. General

physical examination was normal, as it was the

neurological examination, including mental status, cranial

nerves, muscle strength, muscle tone, stretch and superficial

reflexes, cerebellar function, gait and sensory testing.

Impedance and audiometry tests were normal, as well as

magnetic resonance imaging and magnetic resonance

angiography of the brain.

after an exhaustive literature review, only a few cases seem

to have been shared in almost 100 years of its initial

description.

The population affected by this syndrome is usually

also stricken by migraine with aura. From the standpoint

of pathophysiology, this syndrome cannot be confused

with nocturnal epilepsy since tests such EEG and

polysomnography (PSG) during EHS attacks do not

suggest this etiology.

(8,9)

On video PSG and multiple sleep

latencies test (MSLT), EHS attacks showed at the transition

from wakefulness to sleep (non-rapid eye movement

(NREM) sleep stage 1, NREM1) and from NREM2.

(4)

EHS

occurs at any age but usually occurs after age 50. A

gradual increase of stage 1 sleep occurs with brain

aging.

(6,12)

The basis for EHS is thought to be a delay in

the reduction of activity in selected areas of the brainstem

reticular formation as the patient passes from wakefulness

to sleep.

(6,13)

Many speculations had been done, especially after

the use of drugs that were able to control symptoms in

isolated cases. The existence of a transient calcium channel

dysfunction was hypothesized as a cause, since the

nifedipine,

(10)

flunarazine,

(6)

and topiramate (P type calcium

channel)

(1)

produced improvement of the symptoms. Other

drugs have shown satisfactory results, as clonazepam(

11)

and clomipramine.

(9-13)

The EHS attacks occur in relaxed wakefulness or at

the transition from wakefulness to sleep.

(4,5)

A very interesting

way patients, such as our, have reported that the onset the

EHS is directly associated with a worsening of migraine

taking some clinical aspects of chronicity.

(4)

Recent work

has suggested that EHS is considered an atypical acoustic

aura.

(4,7)

Two hypotheses suggest a momentary disinhibition

of the cochlea or its central connexions in the temporal

lobes,

(3)

sudden involuntary movement of the tympanum

or the tensor tympani,

(3)

rupture of the labyrinthine

membrane or a springing open of the Eustachian tubes.

(3,14)

Our case suggests a central origin since the sounds are

not only more elaborate single explosion.

In summary the exploding head syndrome is extremely

rare, occurs in patients with migraine, seems to be

associated with a clinical worsening of migraine and is

considered a form of acoustic migraine aura.

REFERENCES

1. Palikh GM, Vaughn BV. Topiramate responsive exploding head

syndrome. J Clin Sleep Med. 2010;6(4):382-3.

Figure – Sounds described by the patient

DISCUSSION

In this syndrome, the sudden start of the symptoms

resembles thunderclap headache. As the patient is not yet

dreaming, these sounds occur in a context totally unknown

to the patient. Maybe this is why patients wake up very

scared, looking for the source of the noise. This is a rare

disorder and our experience is limited to one case. Even

Headache Medicine, v.2, n.4, p.209-211, Oct/ Nov/Dec. 2011 211

2. Armstrong-Jones R. Snapping of the brain. The Lancet.

1920;196:720.

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Neurol Neurosurg Psychiatry. 1989;52(7):907-10.

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Correspondence

Elcio Juliato Piovesan, MDElcio Juliato Piovesan, MD

Elcio Juliato Piovesan, MD

Rua General Carneiro, 1103/102

80060-150 – Curitiba, PR, Brasil

piovesan1@hotmail.com

Received: 9/6/2011

Accepted: 10/25/2011

EXPLODING HEAD SYNDROME – THE EARLY STEPS