title: Hirano body-rich subtypes of Creutzfeldt-Jakob disease.
abstract: BACKGROUND
  In definite Creutzfeldt-Jakob disease (CJD), morphological and immunohistochemical patterns are useful to identify molecular subtypes. Severe cerebellar pathology and hippocampal involvement helps to identify VV subtypes. The rare VV1 variant (<1%), more frequent in young individuals, is additionally characterized by the presence of ballooned neurones in affected areas. In 1985, Cartier et al. described a family cluster of three individuals with an ataxic CJD form, showing, in addition to severe cerebellar and hippocampal involvement, the presence of frequent Hirano bodies (HB) in CA1 pyramidal neurones. HB are frequently found in aged individuals with Alzheimer pathology although they are not a specific finding.
  
  
  OBJECTIVE
  In this study, we evaluated the presence of HB in hippocampi of 54 genetically and molecularly characterized CJD cases, aiming to elucidate whether additional morphological features could be helpful to point to molecular subtypes.
  
  
  RESULTS
  We identified nine cases (four VV1, one out of three MV2K, three out of six MV2K+2C and one MV carrying a 96-base pair insertion) with abundant, partly bizarre and clustered HB in CA1 sector, not observed in other subtypes. The presence of HB was independent of hippocampal involvement by the disease itself.
  
  
  CONCLUSIONS
  Clusters of abundant HB might be found in rare CJD subtypes such as VV1, MV2K/MV2K+2C and some genetic cases. In addition to histopathological and PrP immunohistochemical deposition patterns, their presence might be a useful additional morphologic feature that could point to the molecular subtype, especially when genetic and/or Western blot analyses are not available.
authors: Neurological Tissue Bank-University of Barcelona CCiT, Hospital Clínic, IDIBAPS Vall d'Hebron Research Institute and Pathology Department, Spain.
authors: E
authors: E
authors: Martinez-Saez
authors: E
authors: E
authors: Gelpi
authors: M J
authors: MJ
authors: Rey
authors: I
authors: I
authors: Ferrer
authors: T
authors: T
authors: Ribalta
authors: T
authors: T
authors: Botta-Orfila
authors: C
authors: C
authors: Nos
authors: J
authors: J
authors: Yagüe
authors: R
authors: R
authors: Sanchez-Valle
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D000328:Adult; D000367:Age Factors; D000368:Aged; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification* / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006624:Hippocampus / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018620:PrPSc Proteins / Q000378:metabolism
chemical_list: D048948:14-3-3 Proteins; D018620:PrPSc Proteins