title: Potassium channel antibody associated encephalopathy presenting with a frontotemporal dementia like syndrome.
abstract: OBJECTIVE
  To describe a patient who presented with features suggestive of frontotemporal dementia (FTD) but with some atypical findings and antibodies to neuronal voltage-gated potassium channels (VGKC-Abs).
  
  
  METHODS
  Case report.
  
  
  METHODS
  Mater Misericordiae University Hospital, Dublin, Ireland.
  
  
  RESULTS
  An 82-year-old man presented with progressive changes in personality, social conduct, and executive function with preservation of memory, deteriorating from baseline to requiring acute hospitalization within 6 months. Transient deterioration (episodic speech arrest) with spontaneous recovery, atypical for frontotemporal dementia, was observed. The patient had an elevated VGKC-Ab titer (2624 pM [normal range, < 100 pM]), elevated protein levels in cerebrospinal fluid, and a negative evaluation for malignancy. Magnetic resonance imaging of brain was normal but [(18)F]-fluorodeoxyglucose positron emission tomographic imaging revealed bifrontal hypometabolism. A marked and sustained improvement with steroid therapy was observed.
  
  
  CONCLUSIONS
  Workup for a potentially reversible autoimmune-mediated encephalopathy, including a VGKC-Ab titer, should be considered in patients presenting with rapidly progressive behavioral and cognitive decline.
authors.affiliation: Department of Neurology, Mater Misericordiae University Hospital, Dublin, Ireland. mckeon.andrew@mayo.edu
authors.forename: Andrew
authors.initials: A
authors.lastname: McKeon
authors.forename: Michael
authors.initials: M
authors.lastname: Marnane
authors.forename: Martin
authors.initials: M
authors.lastname: O'connell
authors.forename: John P
authors.initials: JP
authors.lastname: Stack
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Kelly
authors.forename: Timothy
authors.initials: T
authors.lastname: Lynch
mesh_terms: D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D001925:Brain Damage, Chronic / Q000188:drug therapy / Q000276:immunology* / Q000523:psychology*; D003704:Dementia / Q000523:psychology*; D003937:Diagnosis, Differential; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009474:Neurons / Q000276:immunology; D009483:Neuropsychological Tests; D049268:Positron-Emission Tomography; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D011239:Prednisolone / Q000627:therapeutic use; D019275:Radiopharmaceuticals
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D024642:Potassium Channels, Voltage-Gated; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D011239:Prednisolone