Wagdi P, Meier C
Medizinische und Neurologische Universitätskliniken, Inselspital Bern.
Schweiz Med Wochenschr. 1990 Sep 1;120(35):1247-52.
Clinically, X-chromosomal adrenoleukodystrophy is characterized by a variable neurological symptomatology and non-obligatory adrenocortical insufficiency. The underlying pathogenetic mechanism consists of a disturbance of the peroxisomal metabolism of fatty acids with consecutive accumulation of very long chain fatty acids (VLCFA) in the blood. The diagnosis of this rare condition is important in view of the possibility of special dietary treatment and genetic counseling of the family.