Magnetic resonance spectroscopy in Niemann-Pick disease type C: correlation with diagnosis and clinical response to cholestyramine and lovastatin.

@article{Sylvain1994MagneticRS,
  title={Magnetic resonance spectroscopy in Niemann-Pick disease type C: correlation with diagnosis and clinical response to cholestyramine and lovastatin.},
  author={Michel Sylvain and Douglas L. Arnold and Charles R. Scriver and Roberto Schreiber and Michael Israel Shevell},
  journal={Pediatric neurology},
  year={1994},
  volume={10 3},
  pages={228-32}
}
Niemann-Pick type C is an autosomal-recessive, neurovisceral storage disorder that results from defective cholesterol esterification. Cholesterol-lowering agents have been demonstrated to decrease hepatic lipids in Niemann-Pick type C patients. The objective was to determine the effects of cholesterol-lowering agents on neurologic features and to develop a noninvasive method of monitoring clinical response. A 9-month-old boy with progressive hepatosplenomegaly and neurodevelopmental delay was… CONTINUE READING

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