Evoked potentials in assessment and follow-up of patients with Wilson's disease.
Treatment of 9 patients with Wilson's disease was prospectively studied with evoked potentials and magnetic resonance imaging (MRI). Oral penicillamine therapy led to a decrease in auditory brainstem (ABP) and somatosensory (SEP) conduction times in 6 and 4 neurologically symptomatic patients, respectively. ABP and SEP were normal in 3 other symptom-free patients. MRI showed cerebral lesions in 4 of 7 patients. Quantified indices of brain atrophy were unaffected by treatment. ABP and SEP may reveal a reversible component of the disease that cannot be detected by MRI, and may be a more sensitive measure of treatment efficacy.
Grimm
G
G
Department of Medicine, University of Vienna, Austria.
Oder
W
W
Prayer
L
L
Ferenci
P
P
Madl
C
C
D000284:Administration, Oral; D000328:Adult; D001923:Brain Chemistry; D002648:Child; D003300:Copper / Q000032:analysis; D005069:Evaluation Studies as Topic; D016057:Evoked Potentials, Auditory, Brain Stem / Q000187:drug effects*; D005073:Evoked Potentials, Somatosensory / Q000187:drug effects*; D005260:Female; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008991:Monitoring, Physiologic; D010396:Penicillamine / Q000008:administration & dosage / Q000627:therapeutic use; D011446:Prospective Studies; D013997:Time Factors
D003300:Copper; D010396:Penicillamine