Comparison of MRI, EEG, EPs and ECD-SPECT in Wilson's disease.
OBJECTIVE
The purpose of this study is to evaluate the efficiency of a few methodologies in detecting anatomo-functional brain abnormalities in patients with Wilson's disease.


METHODS
Twenty-three patients with Wilson's disease underwent almost simultaneously brain magnetic resonance imaging (MRI), computerized electroencephalography (EEG), multimodal evoked potentials (EPs) and ECD single photon computerized tomography (SPECT) evaluation. The clinical picture was of the neurologic type in 8 patients and of the hepatic type in 15.


RESULTS
MRI was abnormal in 7 patients with neurological manifestations. The EPs proved pathologic in 7 neurologically symptomatic patients and in 4 cases with hepatic form. These results agree with those reported in other case studies. The EEG records were abnormal only in 3 cases. Nevertheless, the most interesting finding of this study is the particular frequency (86%) of diffuse or focal decrease of ECD uptake shown by brain SPECT.


CONCLUSIONS
We highlight the use of this interesting procedure in the therapeutic monitoring of this disease.
Clinica Neurologica, Policlinico Universitario, Universita degli Studi di Cagliari, Italy.
M
M
Giagheddu
G
G
Tamburini
M
M
Piga
P
P
Tacconi
A
A
Giagheddu
A
A
Serra
P
P
Siotto
L
L
Satta
L
L
Demelia
F
F
Marrosu
D000328:Adult; D000368:Aged; D001921:Brain / Q000098:blood supply / Q000473:pathology / Q000503:physiopathology; D002536:Cerebral Arteries / Q000473:pathology; D002560:Cerebrovascular Circulation / Q000502:physiology; D004569:Electroencephalography; D004576:Electromyography; D005071:Evoked Potentials / Q000502:physiology*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D007839:Functional Laterality / Q000502:physiology; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D006984:Hypertrophy / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000502:physiology; D018482:Muscle, Skeletal / Q000294:innervation; D012680:Sensitivity and Specificity; D015899:Tomography, Emission-Computed, Single-Photon