Patients with a novel neurofilamentopathy: dementia with neurofilament inclusions.

  title={Patients with a novel neurofilamentopathy: dementia with neurofilament inclusions.},
  author={Nigel J. Cairns and Robert H. Perry and Evelyn Jaros and David Burn and Ian G. McKeith and James S. Lowe and J. Jerome Holton and Martin Rossor and Kari Skullerud and Charles Duyckaerts and Felix F. Cruz-S{\'a}nchez and Peter L. Lantos},
  journal={Neuroscience letters},
  volume={341 3},
We report a new disease, dementia with neurofilament inclusions, characterized clinically by early-onset dementia with frontal lobe signs, focal atrophy of the frontal and temporal lobes, and microscopically by the presence in many brain regions of intraneuronal, cytoplasmic, neurofilament inclusions. The neuronal inclusions are immunoreactive to all three molecular weight neurofilament subunits: heavy (NF-H), light, and medium subunits, including the phosphorylated and non-phosphorylated forms… CONTINUE READING
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