Batten disease in the west of Scotland 1974-1995 including five cases of the juvenile form with granular osmiophilic deposits.

@article{Crow1997BattenDI,
  title={Batten disease in the west of Scotland 1974-1995 including five cases of the juvenile form with granular osmiophilic deposits.},
  author={Yanick J Crow and J. Tolmie and Alan G. Howatson and William J. Patrick and John B.P. Stephenson},
  journal={Neuropediatrics},
  year={1997},
  volume={28 3},
  pages={140-4}
}
We report on 12 children with neuronal ceroid lipofuscinosis (NCL) diagnosed between 1974-1995 in the West of Scotland. Diagnosis was made on the basis of clinical, electrophysiological, radiological and pathological examination including electron microscopy (EM) in all cases. Incidence was calculated on the basis of the year of diagnosis and the live birth rate. Six cases were infantile and 6 juvenile NCL derived from a total of 10 families. No cases of late infantile or early juvenile NCL… CONTINUE READING