12
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: not found

      Mutations in progranulin (GRN) within the spectrum of clinical and pathological phenotypes of frontotemporal dementia.

      1 ,
      The Lancet. Neurology
      Elsevier BV

      Read this article at

      ScienceOpenPublisherPubMed
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Frontotemporal dementia (FTD) is predominantly a presenile disorder that is characterised by behavioural changes and cognitive impairment, particularly in language and executive functions, and is associated with neurodegeneration in the frontal or temporal cortices, or both. Research into FTD has made many advances over the past 20 years that have important implications for clinical practice. Different clinical variants (ie, behavioural, aphasic, and motor neuron disease variants) are now recognised as part of the clinical spectrum of FTD. Neuropathologically, the disease can be divided into two main pathological subtypes: frontotemporal lobar degeneration (FTLD) with neuronal and glial tau inclusions (FTLD-tau); and FTLD with neuronal inclusions that are positive for ubiquitin (FTLD-U). 20-30% of cases of FTD follow an autosomal dominant pattern of inheritance, and half of which are caused by defects in MAPT, CHMP2B, and VCP.

          Related collections

          Author and article information

          Journal
          Lancet Neurol
          The Lancet. Neurology
          Elsevier BV
          1474-4422
          1474-4422
          Oct 2008
          : 7
          : 10
          Affiliations
          [1 ] Department of Neurology, Erasmus Medical Center, Rotterdam, Netherlands. j.c.vanswieten@erasmusmc.nl
          Article
          S1474-4422(08)70194-7
          10.1016/S1474-4422(08)70194-7
          18771956
          f57fe2dc-4dfb-4d94-9ca1-e290919e542d
          History

          Comments

          Comment on this article