To die in one's sleep. . . so basic to the cauldron of human fears,
subject matter to ancient rhymes and holy sonnets, the arts and literature,
laden with layers of mythology, coloring folklore with creatures of
the night, concoctions of superstitions, and nostrums of nightmare
preventions. Cloaked in mystery and dread, it it has acquired a motley
of names and acronyms – Sudden Unexplained Death Syndrome. SUDS,
Sudden Unexpected Death in Sleep, Sudden Unexplained Nocturnal Death
Syndrome, SUNDS, nightmare deaths, sudden night deaths
– as science pokes through the muddle of myth and folklore,
searching for etiologies and pathogenesis that can shed light into
this mysterious fatal affliction that visits presumably healthy young
men in their sleep, more commonly in the Southeast Asian and Pacific
Rim countries and Polynesian populations believed to have migrated
from South East Asia centuries ago.
First reported in the Philippines in 1917, 'sudden
night deaths' or SUDS (sudden Unexpected Death in Sleep) has been
attributed to bangungot (bangungut
- from the Tagalog root words of "bangon," to rise; and
"ungol," to moan) – a syndrome, wrapped in folklore
and myth, that consists of a nightmare, commonly occurring in nocturnal
sleep, frequently after a heavy meal that is often accompanied by
alcohol, most often in young males, aged 25-44, presumably healthy,
without any known cardiac illness.
Risk
profile
Young males
Age: 25-44
A heavy meal
Alcohol use
Presumably healthy with
no prior heart problems
Fainting
Family history of unexpected death |
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In
the Philippines, bangungut (SUDS) has been so linked to gluttonous
eating and bacchanalian drinking, to the exclusion of other symptoms
or warning signs. Fainting and family history do not raise red
flags. But South East Asian studies suggest that a history of
fainting with a positive family history, increases the chance
of dying of SUDS in the next five years
A review of SUDS cases (Munger and Booton) from Death Certificates
filed in Manila during 1948-1982 showed the same characteristics:
96% male, mean age 33 years, modal time of death 3:00 a.m. The
deaths were seasonal, peaking in December-January, and the SUDS
victims were more likely than diseased controls to have been born
outside of the Manila area.
A 2003 UP health survey on SUDS among young Filipinos reported
43 deaths per 100,000 annually. |
How often bangungut becomes fatal is unknown. Many cases are never
reported, especially in the rural areas where dying in your sleep
is an accepted event in the folklore of death. Many know others who
died in their sleep. Many more are 'survivors' of one or more attacks,
with descriptive details of bangungot -type nightmares– of sleep
paralysis, of falling from a mountain or into a deep abyss, of the
creature in the dark standing by the bedside. How many of these are
actually near-death or near-bangungut experiences or are they merely
generic ingredients to culture-flavored nightmares?
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| Note: I have to begrudge the Ilocandia
myth makers. Why the need to invent "Batibat?"
To die in a bangungut nightmare is uugghh-dreadful enough.
But to die with the the fat and vengeful "batibat"
seating on your face. Oh, mercy me. |
"Batibat" clay sculpture
by Alvin Barcelona, Brgy. Lumingon, Tiaong, Quezon (4"x5") |
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Although there are witness reports of "moaning, groaning, gasping,
choking, frothing, and labored breathing," as often, patients
are found dead, in seeming peaceful slumber, without the sounds of
terror or any evidence of a terminal struggle.
In a "macho-culture" with a penchant
for drinking, often to oblivion, and accompanying this libatory indulgence
with a smorgasbord of "pulutan," pancreatitis became the
popular and preferred "point-to diagnosis." (see pancreatitis,
below).
In a country with more than 7000 islands and more
than 70 indigenous communities, where albularyos and medicos minister
to the end-days, diagnosing fainting spells by tawas
and treating them with a bulong and/or orasyon,
where the night worlds are ruled by the frightful creatures of myths
and superstitions – the tikbalangs, kapres, asuwangs, white
ladies and pontianaks, where death's ways are accepted with funereal
fatalism as God's will, karma, or bangungut. — alas, the true
incidence of bangungut / SUDS is probably a-long-time-coming before
it gets revealed to the scrutiny of science.
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SUDS IN OTHER COUNTRIES
Of course, nightmares are as old as sleep
itself, and sleep-deaths are not unique to one particular race. Cultures
wrap it in their own mythologies and superstitions. There are reports
of SADS in other countries, unrelated to Southeast Asian populations.
However, it was the Southeast Asian cultures and migrant populations
that originally provided the mystique and attention to the nightmare-death
syndrome.
| Thailand,
LAI TAI, once believed
to be linked to eating rice cakes, or the phi am
or 'widow ghost" foraging the night for healthy young men.
Prevalence is 26-40 per 100,000 population. Recent studies link
SUDS in Thailand and Laos to the Brugada syndrome. (see below) |
Hmong of Vietnam,
TSOB TSUANG -
the 1970s, the U.S. coined the acronym SUND (sudden unexpected
nocturnal death) and SUDS (sudden unexpected death syndrome)
from reports of 38 mysterious deaths among Southeast Asian refugees,
mainly from the Hmong of Vietnam.
A case control study in the Ban Vinai refugee camp in northeaster
Thailand revealed associations between sudden death in sleep
and membership in the Green-Hmong subgroup with a family history
of sudden death and non-fatal sleep disturbances. |
| French Polynesia,
SUDS cases attributed to Polynesian migration history from South
East Asia centuries ago. In a study of 150 men with a history
of fainting, cardiac arrest, or an abnormal EKG. Of these, 32
were found to have the abnormal EKG pattern; in 6 of 11, ventricular
fibrillation could be induced. 70 % had an older male relative
who died unexpectedly in the night. |
| Japan,
POKKURI — a
SUDS misnomer, neither "sudden or unexpected," a ritual
of the elderly Japanese who go on a winter pilgrimage to the
pokkuri-dera (Buddhist temple of sudden death, theKichi-denji
Temple in Nara City). Not wishing to suffer or be a burden with
an extended illness, they actually pray for a discrete and peaceful
death while sleeping, to 'pokkuri,'
to just "pop-off." |
RELATED REPORTS
• In the 1980s, Lancet reported SUDS deaths
among Thai construction workers in Singapore.
• Sporadic reports of deaths among Cambodians and Laotian men
in North America and refugee camps in Thailand.
• A 2006 UPS report in England of SADS being about eight times
higher than previously thought. Aged 4 to 64, none had a history of
heart disease, almost one in five had a family history of sudden unexplained
deaths before the age of 45. Death was suspected to be from a genetic
heart defect causing malignant abnormalities in heart rhythm. |
|
A FEW
SLEEP FACTOIDS
SLEEP PARALYSIS
Sleep paralysis is not unique
to the bangungut syndrome. It is a common and ancient sleeping disorder
attributed to demons and malevolent spirits, characterized by a feeling
of immobility, being pinned down by an invisible force, unable to
move and unable to scream for help. Inevitably, it becomes embellished
with the creatures of culture and superstition. (See Batibat,
above)
Sleep paralysis usually occurs
at the threshold of wakefulness and sleep, hypnagogic when one is
falling asleep, hypnopompic when one is waking up, with the accompaniments
of falling sensation, chest pressure, and presences of shadowy creatures.
Dreaming occurs chiefly in REM
states, to a lesser extent in NREM sleep. Sleep is cyclic with 4 or
5 REM periods during the night accounting for one-fourth of the total
hour of sleep ( 1 1/2 to 2 hours). Studies suggest alcohol disturbs
the REM cycles of sleep. What effects on the REM dreams? What effects
on the stage 3 or 4 when sleep terrors (pavor nocturnus)
occur?
Nightmares occur during REM sleep.
Sleep terrors in stage 3 and 4 sleep.
NIGHT HAG
Night hag, or a waking dream,
a modern-day name given to a sleep associated with nightmares and
sleep paralysis. It is believed to be a nightmarish dream-state in
the threshold of sleep and waking, sometimes replete with ogres and
creatures, holding the person down or paralyzing him in fear. Some
survive these episodes with "marks and bruises," attributing
the experience to the paranormal.
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NUTRITIONAL DEFICIENCIES
AND MYTHS,
STRESS AND ENVIRONMENTAL FACTORS
Of the Thai workers in Singapore, nutritional deficiencies
were implicated, particularly thiamine and potassium. However, there
is no information as to whether potassium levels were obtained, or
how severe the hypokalemia was.
Besides gorging and drinking before bedtime, certain foods myths abound.
Some warn against big noodle meals before bedtime. Too, rice cakes
at bedtime. In one province, seasoned imbibers are afraid to combine
beer and balut, from an anecdotal account of a father and two sons
who sccumbed to bangungut deaths - the deadly combination, prologue
to their dreadful end.
Beer
and balut
Gives you bangungut.
Stress has been considered in
the etiology of SUDS, reported in usually high numbers in healthy
young Southeast Asian men, especially in various refugee populations
migrants communities. With increasing length of residence in the US,
SUDS rates markedly declined. But if stress alone is causative, how
to explain its relative absence in the many other races ravaged by
war and poverty, and driven to dispersions and economic diaspora.
The occurrence of SUDS in Asian populations, the
decline among SEA refugees after immigration to the US, the elevated
risk among migrants in Manila and seasonality of occurrence (Munger
and Booton) implicates stress, environmental factors and nutritional
deficiencies |
THE
SCIENCE
THE OLD AND THE NEW
Inevitably, science went forging
and fishing into the sea of folklore and myths. In the recent decades,
battling through ghouls and night hags, armed with the old and new
tools of technology and the disciplines of research, digging into
new world of genetic mutations, it has emerged partially victorious,
providing us alternatives to the old and overused pancreatitis-diagnosis,
with new theories and etiologies — the Whys-and-Whats and-Hows
of unexpected sudden nocturnal deaths, and further adding to its lengthening
lists of acronyms and syndromes (SADS: Sudden Arrhythmia Death Syndrome
from Brugada, the Long QT, or Short QT syndromes).
THE OLD . . .
ACUTE
HEMORRHAGIC
PANCREATITIS
In the Philippines, the cause of
SUDS / bangungut has been linked to acute hemorrhagic pancreatitis
attributed to a hearty or gluttonous meal often downed by a hefty
amount of alcohol. A high salt diet, contributed to by native condiments
- patis and bagoong - has been implicated. Stories
are told of bangungut attacks with the combined indulgence of beer
and balut.
| As the sole cause of
bangungut, pancreatitis is a hard-sell. Especially if caused by
excessive alcohol use, acute pancreatitis is rarely, if ever "silent,"
but rather quite dramatic and severe in its presentation, more
likely to present itself with more than an "ungol" (groan). |
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|
PANCREATITIS is the inflammation of the pancreas,
the gland that sits behind the stomach, concerned with digestive enzyme
functions and production of insulin. It is connected by a ductal system
to the liver and the gallbladder, an anatomic detail of concern in
gallstone disease. Pancreatitis can be either acute or chronic. In
acute pancreatitis, there may be a history of excessive alcohol intake
or a heavy meal preceding the attack or a history of biliary colic
(gallstones) in the past. The rest are due to infections, vasculitis,
drugs, trauma, hypercalcemia, hypertriglyceridemia. Although occasionally
mild, often the symptomatology is rather dramatic in acute pancreatitis,
with nausea, vomiting, fever, abdominal pain, distention, sweating,
rapid pulse,and shock.
Clinically, it is a hard-sell of a diagnosis
if it is to be solely implicated for cause of bangungut deaths.
As mentioned, the acute attacks have a rather dramatic constellation
of symptoms, that runs the course of hours to days, with a severity
that forces the patient to seek help or medical attention. Of course,
the pancreatitis could have been "silent" if the alcohol
was used in 'overdose' amounts, enough to cause respiratory depression,
seizures, shock, coma, and death.
Still, it will not explain the many
cases of "bangungut" deaths in the seemingly "peaceful"
scenarios of dying. Also, there are many similar "night deaths"
where autopsies failed to show any pathologic cause. And for these,
the usual fall-back is the waste-basket diagnosis of "cardio-respiratory
arrest."
. . . AND THE NEW
The
Brugada & QT Syndromes:
The long and short of it
The
human body has a system of quasi independent electrical circuits that
can be recorded through medically dedicated tools: the EMG-NCS (electromyography
for muscle and nerve conduction studies), the EEG (electroencephalography
for the study of brain waves) and the EKG or ECG (electrocardiography
for a print out of the heart's electrical rhythm). Fascinating, all
– electrical systems working in unison, with an endogenous power
source that usually lasts a "lifetime of average use and abuse."
Of these, it is the EKG that can provide information critical to the
immediate assessment and management of life-and-death
situations: heart
attacks and potentially fatal cardiac irregularities. To boot, it
can provide clues of rhythm disturbances and
PQRST-wave configuration abnormalities that can alert the astute clinicians
into raising red-flags, recognizing and preventing potential problems.
The ECG has been the gateway tool in the diagnoses of clinical entities
constituting a group called SADS (sudden
arryhthmia death syndrome) recognizable by its potentially fatal electrocardiographic
presentations (Wolff-Parkinson-White syndrome, idiopathic ventricular
fibrillation,
or arrhythmias caused by electrolyte imbalances). Recent studies and
discoveries have focused on emerging entities — the Brugada
syndrome, the long-QT and the short-QT syndromes — with its
hereditary genetic defects affecting the cardiac ion channels, stripping
some layers from the myths of sudden unexpected nocturnal deaths,
providing science, etiologies and treatment options.
BRUGADA SYNDROME
In
1998, a hereditary gene defect was found, involved in the coding of
protein components in the sodium channels in heart cells. The channel
defect causes abnormal electrical conduction in the heart with resultant
ventricular arrhythmias, including full-blown ventricular fibrillation
leading to death. Of the patients studied, many presented with unexplained
fainting spells.
It presents with a characteristic hereditary
EKG abnormality, the Brugada sign: a right bundle branch block with
a right precordial injury pattern from V1 to V3.
(Left insert: The Vi – V3 tracings compare the normal on the
left with the Brugarda pattern on the right.) Fortunately,
it is rare. In Japan, where it is found in less than 1% of the population;
and in the U.S.,in less than 0.5%. Of these, most will not have any
problems. But the EKG pattern, coupled with a history of unexplained
fainting, a history of a relative dying young, raises a red-flag risk
of sudden death.
The syndrome has been linked to SUDS,
causing sudden death in apparently healthy young people over the age
30. A study of patients with SUNDS and their families, screened
for genetic mutations in SCN5A, the gene known to cause the Brugada
syndrome, suggested that SUNDS and the Brugada syndrome are phenotypically,
genetically, and functionally the same disorder. <http://hmg.oxfordjournals.org/cgi/content/full/11/3/337>
In Thailand, where the estimated prevalence
of SUDS is 26-38 per 100,000 population, a study on patients with
the Brugada syndrome showed a low heart rate variability at night
that may predispose to the occurrence of ventricular fibrillation
episodes.
The prognosis for high risk patients - abnormal
EKG with history of syncopal attacks or near-sudden death resuscitation
- is very poor, with a third at risk of a polymorphic ventricular
tachycardia within two years. Treatment is with an implantable defibrillator.
Risks for asymptomatic patients with typical electrocardiographic
patterns are the same; a third will develop ventricular tachycardia
or fibrillation within two years. Again, at present, only the implantable
cardioverter-defibrillator protects against sudden cardiac death.
THE LONG QT SYNDROME
(LQTS)
Like the Brugada syndrome, with its characteristic
hereditary ion channel and ECG abnormality, hereditary prolongation
of the QT interval is also associated with SADS (Sudden Arryhthmia
Death Syndrome). LQTS is caused by mutations of the genes for cardiac
potassium and sodium ion channels. The most common type is the autosomal
dominant Romano-Ward syndrome. While it can present with syncope and
seizures, In 30 to 40 percent of patients, sudden death is the only
event. While the cardiac events are usually precipitated by physical
and emotional stress, deaths can also occur in sleep. A history of
unexplained sudden death in a young family member is a clue.
QT prolongation is characteristic and
predisposes to ventricular tachyarrhythmia – polymorphic ventricular
tachycardia, or torsade de pointes, which can lead to ventricular
fibrillation and sudden cardiac deaths. Sometimes, only a borderline
prolonged QT interval is seen. Another clue is the presence of T-wave
alternans, the severity of which correlates with cardiac events.
Some of the highest rates of inherited
long QT syndrome occurs in Southeast Asian and Pacific Rim countries.
The syndrome has more treatment options
than the Brugada syndrome: Beta blockers, potassium, avoidance of
certain drugs, permanent pacemakers, and implantable defibrillators.
THE SHORT QT SYNDROME
A relatively new syndrome, first described
in 1999, belonging to the ion-channel disorders called "channelopathies,"
hereditary short QT syndrome is an autosomal-dominant clinical-electrocardiographic
entity with associated gene mutations. It is characterized by a short
QT, frequently tall-peaked T-waves similar to a "hyperkalemic-T,"
inducible ventricular fibrillation, episodes of syncope, a high tendency
for paroxysmal atrial fibrillation or life-threatening arryhthmias,
without any underlying structural heart disease.
Three forms of the syndrome have been identified
affecting different channels. Indicated therapy for those who have
survived a cardiac arrest or those with a history of syncope, is the
implantable cardioverter-defibrillator. In its absence or inaccessibility,
drug therapy has the potential for prolonging the QT interval. However,
drug therapy has been shown to be beneficial only for the first form,
and ineffective for the other forms.
Although it has been linked to deaths in infants,
children and young adults with a family background history of sudden
cardiac death, it is possible that many survive into a later adulthood,
into the mean age of deaths linked to SUDS/ SUNDS or bangungut.
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SOURCES
• Sudden adult
death syndrome .Dr Trisha Macnair
• Batibat. Philippine Mythology. Wikipedia
• Why would a middle-aged man faint? T. Dajer. Discovery. November
2006
• The Brugada Syndrome. http://www.brugada.org/about/disease-history.html
• Sudden and unexplained death in adult Filipinos . Ronald Munger
& Elizabeth Booton . International Journal of Epidemiology. 1996
• Heart rate variability in patients with Brugada syndrome in
Thailand, R. Krittayaphonga, G. Veerakulb, K. Nademaneec and C. Kangkagate
• Nightmare death syndrome / David Hambling / http://www.forteantimes.com/strangedays/medicalbag/335/nightmare_death_syndrome.htm
• Sudden Arrhythmia Death Syndrome: Importance of the Long QT
Syndrome . J.MEYER, MD. et al/ AmAcadFamPhysician./ August 2003
• Genetic and biophysical basis of sudden unexplained nocturnal
death syndrome (SUNDS), Human Molecular Genetics, 2002, Vol. 11
• Asian genetic killer found in Polynesian men. Danny Kingsley
– ABC Science Onlin <http://www.abc.net.au/science/news/stories/s877742.htm
• Long QT Syndrome . Ali A Sovari, MD, <http://www.emedicine.com/med/topic1983.ht>
• Brief Review of the Recently Described Short QT Syndrome and
Other Cardiac Channelopathies . Andrés Ricardo Pérez
Riera, M.D.**Cardiology Division, ABC's Faculty of Medicine, ABC Foundation,
Santo André, São Paulo, Brazil,
• "'Bangungot', the sequel", Tan, Michael .18 April
2002.Philippine Daily Inquirer
• Short QT syndrome . Ramon Brugada, Kui Hong, Jonathan M. Cordeiro
and Robert Dumaine. http://www.cmaj.ca/cgi/content/full/173/11/1349
• Sudden death in sleep of Laotian-Hmong refugees in Thailand:
a case-control study. R G Munger. m J Public Health.1987 Sept; 77(9):
1187–1190.
• Sudden
Arrhythmia Death Syndrome: Importance of the Long QT Syndrome. John
Meyer et al. <http://www.aafp.org/afp/20030801/483.html>
• http://www.emergencyekg.com/life_preserver.cfm
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