Nonvasculitic autoimmune inflammatory meningoencephalitis imitating Creutzfeldt-Jakob disease

C. Hoffman Snyder, K. J. Mishark, J. N. Caviness, J. F. Drazkowski, R. J. Caselli

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


Background: Nonvasculitic autoimmune inflammatory meningoencephalitis and Creutzfeldt-Jakob disease can present as rapidly progressive encephalopathies with similar clinical features. Slowing of background rhythm is an electroencephalographic characteristic shown by both, but persistent periodic sharp waves are more specific for Creutzfeldt-Jakob disease and have not been reported in nonvasculitic autoimmune inflammatory meningoencephalitis or related autoimmune meningoencephalitides. Objective: To describe a patient with clinical (rapidly progressive myoclonus, dementia, and Parkinsonism) and electroencephalographic findings (persistent periodic sharp waves) that diagnostically suggest Creutzfeldt-Jakob disease. Design and Setting: A case report at the Mayo Clinic Arizona, Scottsdale. Results: The patient made a dramatic recovery with resolution of the periodic sharp wave complexes after treatment with high-dose corticosteroids. Our case is the first reported case of a patient with probable nonvasculitic autoimmune inflammatory meningoencephalitis and electroencephalographic periodic complexes suggestive of Creutzfeldt-Jakob disease. Conclusion: Rapidly progressive encephalopathy with periodic sharp wave complexes can be associated with a reversible autoimmune syndrome.

Original languageEnglish (US)
Pages (from-to)766-768
Number of pages3
JournalArchives of neurology
Issue number5
StatePublished - May 2006

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology


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