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In Response to the Letter to the Editors

Copyright © 2005 Sage Publications I greatly appreciate the valuable comments of Dr Fernández-Torre in response to our case of Creutzfeldt-Jakob disease (CJD) presenting as refractory nonconvulsive status epilepticus [1]. Two recent publications, by Fernández-Torre et al [2] and Cohen et al [3], each describe a case of nonconvulsive status epilepticus attributable to sporadic CJD. The medical literature now includes several documented cases of status epilepticus as a presentation of CJD [1-5]. The common features of our cases are the electroencephalographic finding of continuous sharp wave activity, the necessity for multiple anticonvulsants, and the lack of neurologic improvement after resolution of electroencephalographic seizures [1,2]. Electroencephalographic patterns are challenging to interpret, and a recent article described patterns and progression of electroencephalographic findings during the course of CJD [6]. In the intensive care unit, nonconvulsive status epilepticus is typically attributable to a neurologic injury or toxic-metabolic derangement. In cases without a clear predisposing factor, we suggest consideration of CJD even in patients without preceding dementia and myoclonus. Evaluation for CJD in critically ill patients is an exceptional situation; specific diagnostic testing, although not definitive, may be beneficial. In suspected cases of CJD, the presence of cerebrospinal fluid 14-3-3 brain protein and specific cerebral diffusion-weighted magnetic resonance imaging abnormalities may support the probable diagnosis of CJD [7]. The recent publication of these analogous cases supports our recommendation to intensivists to consider CJD when nonconvulsive status epilepticus is refractory and unexplained.