title: Metabolic disorders with clinical and radiologic features of sporadic Creutzfeldt-Jakob disease.
abstract: Two patients with metabolic disorders presented with clinical and radiologic features suggestive of sporadic Creutzfeldt-Jakob disease (sCJD). Case 1 was a 50-year-old man with rapid decline in cognitive, behavioral, and motor function following new-onset seizures. MRI was read as consistent with CJD, and he was referred for a treatment trial, but it was determined that he recently experienced rapid correction of hyponatremia resulting in extrapontine myelinolysis. Case 2 was a 66-year-old woman with poorly controlled diabetes mellitus who was found unconscious after a suspected insulin overdose. Examination showed altered mental status and neuroimaging was remarkable for cortical/striatal hyperintensities suggestive of sCJD. On autopsy, she had hypoglycemic/hypoxic nerve cell loss. Although characteristic MRI findings have high sensitivity and specificity for sCJD, potentially reversible metabolic disorders sometimes present rapidly and can resemble sCJD both clinically and radiologically. These cases highlight the importance of establishing a broad differential diagnosis when evaluating a patient with suspected sCJD.
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Michael H
authors.initials: MH
authors.lastname: Rosenbloom
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: M Carmela
authors.initials: MC
authors.lastname: Tartaglia
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Sven A
authors.initials: SA
authors.lastname: Forner
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Katherine K
authors.initials: KK
authors.lastname: Wong
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Amy
authors.initials: A
authors.lastname: Kuo
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: David Y
authors.initials: DY
authors.lastname: Johnson
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Valerie
authors.initials: V
authors.lastname: Colacurcio
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Bret D
authors.initials: BD
authors.lastname: Andrews
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Bruce L
authors.initials: BL
authors.lastname: Miller
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: DeArmond
authors.affiliation: Center for Memory and Aging (MHR), HealthPartners Department of Neurology, St. Paul, MN; Tanz Centre for Research in Neurodegenerative Disease (MCT), University of Toronto, Ontario, Canada; Memory and Aging Center (SAF, KKW, AK, DYJ, BLM, SJD, MDG), Department of Neurology, University of California, San Francisco; Department of Internal Medicine (VC) and Department of Neurology (BDA), Kaiser Permanente, Oakland, CA.
authors.forename: Michael D
authors.initials: MD
authors.lastname: Geschwind