-transparent.png){kind=logo}
Long-chain polyisoprenol alcohol (dolichols) levels are significantly increased in the urinary sediment of patients with infantile, late-infantile, and juvenile forms of neuronal ceroid-lipofuscinosis (NCL). The values in obligate heterozygotes for these diseases are similar to those in patients with other neurological diseases and in healthy controls. Antioxidant treatment of patients with juvenile NCL has no effect on dolichol values. The rate of false-negative results is 13.9% in infantile, 7.5% in late-infantile, and 15.0% in juvenile NCL. False-positive results were found in 8.2 to 14.3% of patients with other neurological diseases and in 15.4% of healthy controls. The test is of considerable value in the diagnosis of NCL and in decisions on whether to perform a biopsy. It is not useful in the screening of random samples, however.
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
June 1975,
The New England journal of medicine,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
December 1978,
The New England journal of medicine,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
February 1997,
Neuropediatrics,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
March 1984,
The New England journal of medicine,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
January 1999,
Annals of Saudi medicine,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
January 1999,
Ryoikibetsu shokogun shirizu,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
January 2001,
Ryoikibetsu shokogun shirizu,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
November 2007,
Expert opinion on medical diagnostics,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
September 2000,
Indian journal of pediatrics,
L S Wolfe, and
J Palo, and
P Santavuori, and
F Andermann, and
E Andermann, and
J C Jacob, and
E Kolodny
January 1989,
Clinical neuropathology,
