Neuropathology of primary adult-onset dystonia

Idiopathic adult-onset primary dystonia usually affects the upper body and remains focal

J. L. Holton

2008

Scholarcy highlights

  • Idiopathic adult-onset primary dystonia usually affects the upper body and remains focal
  • Ubiquitinated perinuclear inclusion bodies were found in the brainstem of patients with DYT1-related dystonia
  • In X-linked recessive dystonia-parkinsonism, neuronal loss in the striosome compartment of the striatum has been described. It was unclear whether these changes are characteristic of these particular disorders or an epiphenomenon of dystonic conditions in general
  • In contrast to early-onset dystonia, neuronal inclusions immunoreactive for torsinA, ubiquitin, and laminA/C were not present in the brainstem nuclei
  • Our findings suggest that the underlying mechanism in the adult-onset primary torsion dystonia is different from that of early-onset DYT1-related dystonia and DYT3 X-linked recessive dystonia-parkinsonism
  • Alternative mechanisms may underpin the pathophysiology of adult-onset primary dystonia
  • NOTE: All authors, besides the first/corresponding author, must complete a separate Disputes & Debates Submission Form and provide via email to the editorial office before comments can be posted

Need more features? Save interactive summary cards to your Scholarcy Library.