Comparison of MRI, EEG, EPs and ECD-SPECT in Wilson's disease

M, Giagheddu; Tamburini, Giorgio; Piga, M.; Tacconi, Paolo; Giagheddu, Antonio; Serra, Alessandra; Siotto, Paolo; Satta, L; Demelia, Luigi; Marrosu, Francesco

doi:10.1034/j.1600-0404.2001.103002071.x

Cited by 42 publications

(25 citation statements)

References 58 publications

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“…The hyperintense signal on MRI is often reversible with treatment [32][33][34]. Variably abnormal findings have been reported in other imaging studies, including single photo emission computed tomography (SPECT) and positron emission tomography (PET), in patients with WD [35,36].…”

Section: Diagnosismentioning

confidence: 91%

Diagnosis and treatment of Wilson’s disease

Subramanian

Vanek

Bronstein

2002

Curr Neurol Neurosci Rep

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Wilson's disease (WD) is an autosomal recessive disease that causes increased copper deposition in the liver and basal ganglia with resultant hepatic and neurologic sequelae. In the past few years, dramatic new discoveries have changed our understanding of the pathophysiology of WD. Although there are potentially life-saving therapies for WD, there is much controversy surrounding the optimal treatments of patients in the various stages of the disease. Specifically, the relative roles of penicillamine, trientene, and tetrathiomolybdate in the initial treatment of the symptomatic patient with WD remain to be defined. Zinc monotherapy for maintenance treatment and in the treatment of asymptomatic patients with WD is still controversial. It is also unclear whether neurologic status alone is an indication for liver transplantation in WD. This paper reviews the pathogenesis, genetics, clinical presentation, and diagnosis, with a special emphasis on the treatment controversies that arise in the care of the WD patient.

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Section: Diagnosismentioning

confidence: 91%

Diagnosis and treatment of Wilson’s disease

Subramanian

Vanek

Bronstein

2002

Curr Neurol Neurosci Rep

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“…Ver schie de ne Ar beits grup pen zeig ten, dass nicht alle Wil son-Pa ti en ten iden tische pa tho lo gi sche Be fun de für sen si ble, sen so ri sche und mo to ri sche Bahn sys teme auf wei sen [6,7,10,15,31]. Hin sicht lich des Auf tre tens pa tho lo gi scher Be fun de sind die FAEP mit 60-65 am häu figs ten be trof fen [15,18,21,25].…”

Section: Elek Tro Phy Sio Lo Gi Sches Schä DI Gungs Pro Filunclassified

“…Die zentral mo to ri sche Leit zeit (CMCT) der korti ko spi na len Lei tungs bahn wur de nach der F-Wel len-Me tho de[10] mit dem System "Ma gne tic Sti mu la tor TM und Counter point TM" der Fir ma Dan tec (Dä nemark) be stimmt. Im Er geb nis die ser Ablei tun gen wer den pro Pa ti ent/Pro band 7 La ten zen (Wel le V, IPL III-V, N 20, P 40, P 100, CMCT-Arm, CMTC-Bein) je weils rechts und links er fasst.…”

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Klassifikation des Morbus Wilson auf der Basis neurophysiologischer Parameter

et al. 2005

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In addition to hepatolenticular degeneration in Wilson's disease, sensory and extrapyramidal motoric systems are also disturbed. In this study a classification on the basis of neurophysiological parameters (EAEP, VEP, MSEP, TSEP and MEP) was established according to subclinical disturbances of these pathways in patients with Wilson's disease on long-term treatment. A cluster analysis of latencies of these evoked potentials was performed. The results of cluster analysis revealed three types of electrophysiological profiles: type I with normal latencies and types II and III showing different patterns of prolonged latencies. However, there was no correlation between clinical classification and the results of cluster analysis based on the electrophysiological data. The neurophysiological based classification provides additional information about central manifestations and aids in characterizing the progress of the disease.

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“…A análise visual do exame de SPECT mostrou alteração em 70% dos casos, porcentagem semelhante àquela encontrada por Piga et al (2008), porém inferior à encontrada por Giagheddu et al (2001). No presente estudo, os locais mais acometidos foram os núcleos da base (50% dos pacientes), seguidos em igual porcentagem (10%) pelo cerebelo, tálamo, lobos temporais e frontais.…”

Section: Spectunclassified

“…Piga et al (2008) observaram, como padrão mais comum (em 53% de sua amostra), hipofluxo cortical difuso, seguido por hipofluxo em núcleos da base (38%), lobos temporal e frontal (21% cada), parietal (17%) e occipital (10%). O lobo temporal (41%) foi o mais acometido, segundo Giagheddu et al (2001), seguido por núcleos da base e lobo parietal (36% cada),…”

Section: Spectunclassified

Avaliação neuropsicológica de pacientes com doença de Wilson e correlação das alterações cognitivas com exames de neuroimagem estrutural e perfusão cerebral

Frota¹

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Comparison of MRI, EEG, EPs and ECD-SPECT in Wilson's disease

Abstract: We highlight the use of this interesting procedure in the therapeutic monitoring of this disease.

Cited by 42 publications

References 58 publications

Diagnosis and treatment of Wilson’s disease

Diagnosis and treatment of Wilson’s disease

Klassifikation des Morbus Wilson auf der Basis neurophysiologischer Parameter

Avaliação neuropsicológica de pacientes com doença de Wilson e correlação das alterações cognitivas com exames de neuroimagem estrutural e perfusão cerebral

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