Neurological deterioration in late infantile neuronal ceroid lipofuscinosis.

  title={Neurological deterioration in late infantile neuronal ceroid lipofuscinosis.},
  author={Stefan Worgall and Minal Vikram Kekatpure and Linda A. Heier and Douglas J. Ballon and Jonathan P. Dyke and Dikoma C. Shungu and Xiangling Mao and B Kosofsky and Michael G. Kaplitt and M M Souweidane and D Sondhi and Neil R. Hackett and C Hollmann and Ronald G. Crystal},
  volume={69 6},
BACKGROUND Late infantile neuronal ceroid lipofuscinosis (LINCL) is associated with progressive degeneration of the brain and retina starting in early childhood. METHODS Thirty-two individual neurologic, ophthalmologic, and CNS imaging (MRI and MRS) assessments of 18 children with LINCL were analyzed. Disease severity was followed by two rating scales, one previously established but modified to solely assess the brain and exclude the retinal disease (modified Hamburg LINCL scale), and a newly… CONTINUE READING


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