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Volume 45, Number 2, April 2012

Lethal toxic encephalopathy due to childhood shigellosis or Ekiri syndrome

Babak Pourakbari, Setareh Mamishi *, Leila Kohan, Leila Sedighi, Shima Mahmoudi, Fatemeh Fattahi, Mostafa Teymuri

Received: October 13, 2010    Revised: February 13, 2011    Accepted: March 18, 2011   


Corresponding author:

Department of Pediatric Infectious Diseases, Children’s Medical Center Hospital, School of Medicine, Tehran University of Medical Sciences, 62 Gharib St., Keshavarz Blvd., Tehran, Iran.
E-mail address: (S. Mamishi).


Background and purpose: 

Lethal toxic encephalopathy due to shigellosis or Ekiri syndrome is a rare complication of shigellosis
with a high fatality rate. Data are very limited on factors that can predict this encephalopathy, so we evaluated clinical and laboratory characteristics for these patients. In this study children with extreme toxicity and convulsions followed by rapid neurological deterioration resulting in brain edema and fatal outcome without sepsis and severe dehydration were selected as having lethal toxic encephalopathy. There were 1295 children with shigellosis during the 10 years of the study. Five children (0.4%) had lethal toxic encephalopathy due to shigellosis. Death occurred following rapid neurological detoriation resulting in brain edema despite intensive treatment. Evidence of brain edema may be a prediction factor for death. Early recognition of encephalopathy and measures to prevent brain edema may improve patient outcome.


Key words:

Ekiri; Shigellosis; Toxic encephalopathy