Dementia, amyotrophy, and periodic complexes on the electroencephalogram -: A diagnostic challenge

Background: The clinical diagnosis of neuroclegenerative diseases is a challenge to the neurologist. In many cases the diagnosis becomes neuropathological only after the autopsy. Several consensus criteria have been defined for the clinical diagnosis of different neurodegenerative diseases, among them the various types of dementia as well as prion-induced diseases. When compared with neuropathological findings, these criteria have proved to be reasonably accurate for regular practice, research, and epidemiological studies. The problem arises when a combination of complementary and clinical data are obtained that do not easily match these diagnostic criteria. Case Description: We describe a patient with dementia and periodic complexes on an electroencephalogram, suggesting a diagnosis of sporadic Creutzfeldt-jakob disease. Results: When the condition progressed, signs and symptoms of a motoneuron disease appeared. Thus, 2 different diagnoses were proposed: (1) an amyotrophic variant of a prion-induced disease; or (2) an ELA dementia syndrome with periodic complexes on the electroencephalogram, a finding that previously has not been described.