Cognitive function in adult adrenoleukodystrophy: comparison with leukoaraiosis and multiple sclerosis.
Cognitive evaluation of 6 cases of adult adrenoleukodystrophy (ALD) included in a brain magnetic resonance (MR) study are reported: 2 males with adrenomyeloneuropathy and 4 women heterozygous for ALD. Cognition was normal in 4 and MR scan in 2 of them. In the 2 others, there were mild modifications of the white matter. One patient suffered of visual retention disturbances with abnormalities of the white matter in MR scan. In the last, cognitive decline was observed; MR scan showed atrophy of cortex and corpus callosum and periventricular high signal areas. Comparison with leukoaraiosis in healthy adults and with multiple sclerosis suggests that there is probably a relationship between cognition and extension of brain MR abnormalities. Time of appearance and frequency of cognitive dysfunction might be explained by the natural history of each of these diseases.
Lyon-Caen
O
O
Service de Neurologie et de Neuropsychologie, Hôpital de la Salpêtrière, Paris, France.
Benoit
N
N
Carreau
V
V
Montreuil
M
M
Menage
P
P
Lubetzki
C
C
Cabanis
E
E
Iba-Zizen
M T
MT
Tourbah
A
A
Baumann
N
N
D000326:Adrenoleukodystrophy / Q000150:complications / Q000473:pathology / Q000523:psychology*; D000328:Adult; D001921:Brain / Q000473:pathology*; D003071:Cognition; D003072:Cognition Disorders / Q000209:etiology / Q000473:pathology / Q000523:psychology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000150:complications / Q000473:pathology / Q000523:psychology*; D009413:Nerve Fibers, Myelinated / Q000473:pathology*