Korean J Leg Med.  2009 May;33(1):19-26.

Pathogenesis of Sudden Manhood Death Syndrome

• Huh GY
• Kim YW
• Kim KH
• Ahn YW
• Jo GR
• Jeong SM
• Lee SY

Abstract

Sudden manhood death syndrome (SMDS) is a disorder found in southeast Asia, particularly Thailand, Philippines and Japan, which causes sudden cardiac death during sleep. In Korea, SMDS cases have been occasionally encountered in forensic autopsy practice. However, the incidence and pathogenesis has been rarely studied. This study to review chronologically the proposed pathogenesis of SMDS; pathology of cardiac conduction system, sleeping and breathing disorder, K+ and thiamine deficiency, mental stress, testosterone, hyperlipidemia, and narrow circumference of coronary artery. Brugada syndrome and SMDS are phenotypically, genetically and functionally the same disorder and Brugada syndrome has been a subject of intensive study since its early description in 1992. While Brugada syndrome is an inherited cardiac disorder caused by mutations in the SCN5A gene encoding the cardiac sodium channel alpha-subunit. less than 20% of its patients are known to be associated with SCN5A mutations. Moreover, the postmortem molecular screening of SCN5A mutations on formalin fixed paraffin embbeded cardiac tissues from SMDS cases has not been rewarding due to technical problems. The role of structural heart disease and sodium channel dysfunction in the induction of electrical instability in SMDS and Brugada syndrome is still debatable.

Keyword

sudden manhood death syndrome; brugada syndrome; pathogenesis; SCN5A

MeSH Terms

Asia, Southeastern
Autopsy
Brugada Syndrome
Coronary Vessels
Death, Sudden, Cardiac
Formaldehyde
Heart Diseases
Humans
Hyperlipidemias
Incidence
Japan
Korea
Mass Screening
Paraffin
Philippines
Respiration
Reward
Sodium Channels
Testosterone
Thailand
Thiamine Deficiency
Formaldehyde
Paraffin
Sodium Channels
Testosterone