[P1-97] Prognosis and Neuroimaging of Acute Encephalopathy after Status Epilepticus in Dravet Syndrome
Purpose: To investigate the prognosis and neuroimaging of acute encephalopathy(AE) after status epilepticus（SE）of patients with Dravet Syndrome(DS).
Method: The clinical data of DS patients who had acute encephalopathy (coma≥24h) after SE were retrospectively collected in Peking University First Hospital from February 2008 to August 2016.
Results: Twenty-two patients（9 males and 13 females）with AE were collected among 412 DS patients during follow-up. The onset age of AE was between 6 months to 10 year-old. The duration of SE varies between 40 minutes to 8 hours. Twenty-one patients had high fever，the temperature was normal only in one patient. Coma lasted from 2 days to 20 days. Nine patients died after the AE, and 13 survived with massive neurological regression. The median time of follow-up was 2 years and 3 months. Nine of 13 survivors had varied improvement in motor, language and cognition, while the remaining 4 had no significant improvement. After AE, spasm occurred in 2 survivors. The neuroimaging of 13 survivors demonstrated diverse cortical atrophy during recovery phase，among which 4 showed cerebellar atrophy，one had right pontine atrophy，4 accompanied by signal abnormalities in subcortical and periventricular white matter，2 showed right hippocampal sclerosis，and one showed signal abnormalities in bilateral basal ganglia.
Conclusions: Acute encephalopathy is more prone to occur in Dravet patients who had high fever. It could result in even death in severe cases. Survivors left severe neurological sequelae.
Method: The clinical data of DS patients who had acute encephalopathy (coma≥24h) after SE were retrospectively collected in Peking University First Hospital from February 2008 to August 2016.
Results: Twenty-two patients（9 males and 13 females）with AE were collected among 412 DS patients during follow-up. The onset age of AE was between 6 months to 10 year-old. The duration of SE varies between 40 minutes to 8 hours. Twenty-one patients had high fever，the temperature was normal only in one patient. Coma lasted from 2 days to 20 days. Nine patients died after the AE, and 13 survived with massive neurological regression. The median time of follow-up was 2 years and 3 months. Nine of 13 survivors had varied improvement in motor, language and cognition, while the remaining 4 had no significant improvement. After AE, spasm occurred in 2 survivors. The neuroimaging of 13 survivors demonstrated diverse cortical atrophy during recovery phase，among which 4 showed cerebellar atrophy，one had right pontine atrophy，4 accompanied by signal abnormalities in subcortical and periventricular white matter，2 showed right hippocampal sclerosis，and one showed signal abnormalities in bilateral basal ganglia.
Conclusions: Acute encephalopathy is more prone to occur in Dravet patients who had high fever. It could result in even death in severe cases. Survivors left severe neurological sequelae.