Sudden unexplained death syndrome
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Sudden unexplained death syndrome was first noted in 1977 among Hmong refugees in the US.[1][1] The disease was again noted in Singapore, when a retrospective survey of records showed that 230 otherwise healthy Thai men died suddenly of unexplained causes between 1982 and 1990:[1] publication of this data provoked a diplomatic incident.
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Features
The condition appears to affect primarily young Hmong men from Laos (median age 33)[1] and northeastern Thailand (where the population are mainly of Laotian descent).[1][1] There is a strong hereditary component and the victims tend to die in their sleep.
Survivors describe a feeling of intense fear and paralysis. There is a sensation of pressure in the chest, the presence of an alien being in the room and altered sensation.
Pathology
The cause of this syndrome is currently believed to be a form of Brugada Syndrome.[1]
It was noted very early on that the disease had the characteristics of a familial cardiac conduction defect (i.e., a problem with the electrical pathways of the heart).[1] and one study has shown evidence for a long-QT syndrome in populations at risk.[1] Thiamine deficiency is common in the risk population because of diet, and is also a cause of a prolonged QT-interval;[1] but proof that inducible ventricular arrhythmia is the cause of this disease came only with the publication of the DEBUT trial in 2003.[1]
Treatment
The only proven way to prevent death is by implantation of a cardiovertor defibrillator. Oral antiarrhythmics such as propranolol are ineffective.[1]
Folk beliefs
This phenomenon is well known among the Hmong people of Laos,[1] who ascribe these deaths to a malign spirit, dab tsog (pronounced "da cho"), said to take the form of a jealous woman. Hmong men may even go to sleep dressed as women so as to avoid the attentions of this spirit.
Synonyms
- Sudden Unexpected Nocturnal Death Syndrome (SUNDS)
- Bangungut (Phillipines)[1]
- Dab tsog (Laos)[1]
- Laitai (Thailand)[1]
- Pokkuri disease (Japan)[1]
References
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