Sudden unexplained death syndrome

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Sudden unexplained death syndrome was first noted in 1977 among Hmong refugees in the US.[1][2] 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:[3] publication of this data provoked a diplomatic incident.

Contents

[edit] Features

The condition appears to affect primarily young Hmong men from Laos (median age 33)[4] and northeastern Thailand (where the population are mainly of Laotian descent).[5][6] 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.

[edit] Pathology

The cause of this syndrome is currently believed to be a form of Brugada Syndrome.[7]

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).[8] and one study has shown evidence for a long-QT syndrome in populations at risk.[9] Thiamine deficiency is common in the risk population because of diet, and is also a cause of a prolonged QT-interval;[10] but proof that inducible ventricular arrhythmia is the cause of this disease came only with the publication of the DEBUT trial in 2003.[11]

[edit] Treatment

The only proven way to prevent death is by implantation of a cardiovertor defibrillator. Oral antiarrhythmics such as propranolol are ineffective.[11]

[edit] Folk beliefs

This phenomenon is well known among the Hmong people of Laos,[12] 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.

[edit] Synonyms

  • Sudden Unexpected Nocturnal Death Syndrome (SUNDS)
  • Bangungot (Philippines)[13]
  • Dab tsog (Laos)[12]
  • Laitai (Thailand)[5]
  • Pokkuri disease (Japan)[14]

[edit] References

  1. ^ Centers for Disease Control (CDC) (1981). "Sudden, unexpected, nocturnal deaths among Southeast Asian refugees" 30 (47): 581–4,589. PMID 6796814. 
  2. ^ Parrish RG, Tucker M, Ing R, Encarnacion C, Eberhardt M (1987). "Sudden unexplained death syndrome in Southeast Asian refugees: a review of CDC surveillance". MMWR CDC Surveill Summ 36 (1): 43SS–53SS. PMID 3110586. 
  3. ^ Goh KT, Chao TC, Chew CH (1990). "Sudden nocturnal deaths among Thai construction workers in Singapore". Lancet 335: 1154. doi:10.1016/0140-6736(90)91153-2. PMID 1971883. 
  4. ^ Munger RG (1987). "Sudden death in sleep of Laotian-Hmong refugees in Thailand: a case-control study". Am J Public Health 77 (9): 1187–90. 
  5. ^ a b Tatsanavivat P, Chiravatkul A, Klungboonkrong V, Chaisiri S, Jarerntanyaruk L, Munger RG, Saowakontha S (1992). "Sudden and unexplained deaths in sleep (Laitai) of young men in rural northeastern Thailand". Int J Epidemiol 21 (5): 904–10. doi:10.1093/ije/21.5.904. PMID 1468851. 
  6. ^ Tungsanga K, Sriboonlue P (1993). "Sudden unexplained death syndrome in north-east Thailand". Int J Epidemiol 22 (1): 81–7. doi:10.1093/ije/22.1.81. PMID 8449651. 
  7. ^ Nademanee K, Veerakul G, Nimmannit S, et al. (1997). "Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men". Circulation 96: 2595–2600. 
  8. ^ Kirschner RH, Eckner FA, Baron RC (1986). "The cardiac pathology of sudden, unexplained nocturnal death in Southeast Asian refugees". JAMA 256 (19): 2700–5. doi:10.1001/jama.256.19.2700. PMID 3773176. 
  9. ^ Munger RG, Prineas RJ, Crow RS, Changbumrung S, Keane V, Wangsuphachart V, Jones MP (1991). "Prolonged QT interval and risk of sudden death in South-East Asian men". Lancet 338 (8762): 280–1. doi:10.1016/0140-6736(91)90419-P. PMID 1677112. 
  10. ^ Munger RG, Booton EA (1990). "Thiamine and sudden death in sleep of South-East Asian refugees". Lancet 335 (8698): 1154–5. doi:10.1016/0140-6736(90)91154-3. PMID 1971884. 
  11. ^ a b Nademanee K, Veerakul G, Mower M, et al. (2003). "Defibrillator Versus beta-Blockers for Unexplained Death in Thailand (DEBUT): a randomized clinical trial". Circulation 107 (17): 2221–6. doi:10.1161/01.CIR.0000066319.56234.C8. 
  12. ^ a b Adler SR (1995). "Refugee stress and folk belief: Hmong sudden deaths". Soc Sci Med 40 (12): 1623–9. doi:10.1016/0277-9536(94)00347-V. PMID 7660175. 
  13. ^ Munger RG, Booton EA (1998). "Bangungut in Manila: sudden and unexplained death in sleep of adult Filipinos". Int J Epidemiol 27 (4): 677–84. doi:10.1093/ije/27.4.677. PMID 9758125. 
  14. ^ Gotoh K (1976). "A histopathological study on the conduction system of the so-called "Pokkuri disease" (sudden unexpected cardiac death of unknown origin in Japan". Jpn Circ J 40 (7): 753–68. PMID 966364.