(Tian XJ et al. 2017). Acute encephalopathy (AE) is a term used to describe brain disease, damage, or malfunction that occurs with an abrupt onset and is progressive in nature. In Dravet syndrome, acute encephalopathy can be the result of a prolonged seizure.
This study, which was published in Chinese and is therefore reviewed by abstract only, examined neuroimaging data from 22 patients with Dravet syndrome in China who presented with status epilepticus and a resulting coma of 24 hours or longer. Patient age ranged from 6 months to 10 years, status epilepticus ranged from 40 minutes to 9 hours, and coma duration ranged from 2 days to 20 days. 21 patients had a high fever before status onset and 9 patients died. Of the surviving 13 patients, all had “massive neurologic regression,” but nine patients improved somewhat after a varied follow-up period (median 2 years 3 months). During the acute encephalopathy, 6 patients had MRIs that showed hemisphere edema (swelling), though the abstract does not indicate whether this was found in the patients who eventually survived AE. During the recovery period, all 13 patients had some form of cortical atrophy.
Tian XJ, Zhang YH, Liu AJ, Yang XL, Zeng Q, Yang ZX, Ye JT, Liu XY, Jiang YW, Wu XR. [Clinical and neuroimaging features of acute encephalopathy after status epilepticus in Dravet syndrome]. Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):277-282. doi: 10.3760/cma.j.issn.0578-1310.2017.04.009. Chinese. PubMed PMID: 28441824.