Epilepsy is a common neurological disorder associated with increased morbidity and mortality. Sudden unexpected death in epilepsy, also known as SUDEP, is the main cause of death in patients with epilepsy. SUDEP has an incidence of 1.2 per 1000 person-years in adults and 0.2 per 1000 person-years in children.
Classification | Definition |
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Definite sudden unexpected death in epilepsy (SUDEP) | Sudden, unexpected, witnessed or unwitnessed, non-traumatic, and non-drowning death that occurs in benign circumstances in an individual with epilepsy, with or without evidence for a seizure, and excludes documented status epilepticus, in which post-mortem examination does not reveal a cause of death. |
Definite SUDEP plus | Death satisfying criteria for definite SUDEP, if a concomitant condition other than epilepsy is identified before or after death, if the death might have been due to the combined effect of both conditions, and if autopsy or direct observations or recording of the terminal event did not prove the concomitant condition to be the cause of death. |
Probable SUDEP or probable SUDEP plus | Same definition as definite SUDEP or SUDEP plus, but without autopsy. |
Possible SUDEP | A competing cause of death is present. |
Near-SUDEP or near-SUDEP plus | A patient with epilepsy who survives resuscitation for more than an hour after cardiorespiratory arrest and has no structural cause identified after investigation. |
Not SUDEP | A clear alternative cause of death is identified. |
Unclassified | Incomplete information available; impossible to classify. |
Gene | Description | Evidence for Association with SUDEP |
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SCN1A | Sodium Voltage-Gated Channel Alpha Subunit 1 | Animal model; de novo variants found in SUDEP cases |
SCN2A | Sodium Voltage-Gated Channel Alpha Subunit 2 | De novo variants found in SUDEP cases |
SCN5A | Voltage-Gated Sodium Channel Subunit Alpha Nav1.5 | De novo variant found in SUDEP case |
SCN8A | Sodium Voltage-Gated Channel Alpha Subunit 8 | Animal model; de novo variants found in SUDEP cases |
KCNA1 | Potassium Voltage-Gated Channel Subfamily A Member 1 | Animal model; variant found in SUDEP case |
KCNQ1 | Voltage-Gated Potassium Channel Subunit Kv7.1 | Variants found in SUDEP cases |
KCNH2 | Voltage-Gated Potassium Channel Subunit Kv11.1 | Variants found in SUDEP cases |
DEPDC5 | DEP Domain-Containing Protein 5 | De novo variants found in SUDEP cases |