MRI Manifestations of Wilson's Disease and its Change in Response to Treatment

Chou, Mei-Chuan; Kuo, Yu‐Ting; Wang, C.-K.; Chen, C.-Y.; Liu, J.-S.

doi:10.1177/19714009980110s211

Cited by 3 publications

(3 citation statements)

References 5 publications

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“…Our remarks on MRI and on evoked potentials are still not absolute, since our cases, as well as the patients described in other studies, cannot be perfectly superimposable as regards age of subjects, severity of clinical form, duration of illness, and efficacy of therapy. In further detail, treatment with chelating agents and/or zinc sulphate may not only improve the clinical conditions in many patients, but may mitigate the anomalies observed with MRI (8, 11, 12, 16, 52, 53, 65–68) and with evoked potentials (47, 52, 53).…”

Section: Discussionmentioning

confidence: 99%

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

Tamburini

Piga³

et al. 2001

Acta Neurologica Scandinavica

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Section: Discussionmentioning

confidence: 99%

“…Brain MRI shows symmetrical lesions in the puta‐men, globus pallidus, caudate, thalamus, mesen‐cephalon, pons and cerebellum (7–16). White matter involvement, generally asymmetrical, is also present.…”

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confidence: 99%

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

Tamburini

Piga³

et al. 2001

Acta Neurologica Scandinavica

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“…Different parts of the central nervous system (including cerebellum, brainstem, thalamus, and subcortical white matter) can be affected, but the greatest damage usually occurs in the basal ganglia 1–4. The close relation between the involved brain structures and the clinical picture has been further strengthened by neuroimaging studies 2, 5–7. Various attempts have also been made to identify different subgroups of the disease, based on neurological, neuropsychological,8 psychiatric,9, 10 and neuroimaging5 data.…”

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confidence: 99%

Cognitive functioning in neurologically symptomatic and asymptomatic forms of Wilson's disease

Seniów

Bak

et al. 2002

Movement Disorders

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We sought to determine the pattern of cognitive deficits in patients with Wilson's disease (WD) with different type and degree of neurological involvement, and to interpret the findings in relation to the underlying pathology. A total of 67 WD patients were examined with a neuropsychological test battery assessing different aspects of cognitive processing. The patients were subdivided into three groups: neurologically asymptomatic, neurological with pure basal ganglia lesions, and neurological with more extensive pathology. The results were compared with 50 matched healthy controls. Patients with a neurological form of WD showed a mild but definitive impairment in all cognitive functions. In contrast, the neurologically asymptomatic patients showed no deficits when compared with normal controls. Multifocal pathology was associated with more severe cognitive deficits than selective basal ganglia lesions but did not contribute significantly to memory impairment. A range of cognitive functions, including frontal-executive ability, aspects of memory and visuospatial processing, are affected in the neurologically symptomatic WD patients. In contrast, no subliminal deficits were observed in the asymptomatic patients. The lesions of the basal ganglia seem to be of central importance in explaining the symptomatology.

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Malattia di Wilson: Reperti RM in 13 casi

D’Amico

Caranci

Briganti

et al. 2001

Rivista di Neuroradiologia

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RIASSUNTO -La malattia di Wilson è un disordine del metabolismo del rame a trasmissione autosomica recessiva. Sono colpiti i tessuti ove si accumula preferenzialmente il rame: fegato, cornea, reni e gangli della base.. La malattia può esordire con sintomatologia epatica o neurologica, quest'ultimo con sintomi extrapiramidali (75% dei casi) o cerebellari (25%, forma pseudosclerotica di Westphal-Strumpell).Dalla nostra esperienza, basata sullo studio con Risonanza Magnetica (RM) in 13 pazienti, emerge l'importanza della metodica in fase diagnostica.La malattia di Wilson mostra un quadro RM abbastanza polimorfo, con interessamento sia della sostanza grigia profonda che della bianca e del tronco encefalico.Frequente il coinvolgimento bilaterale e simmetrico dei nuclei profondi; aree di alterato segnale possono localizzarsi a livello della sostanza bianca, più frequentemente lungo le vie extrapiramidali rispetto al coinvolgimento delle vie piramidali. Ulteriori aree di coinvolgimento della sostanza bianca riscontrate nei nostri pazienti comprendono le regioni retrotrigonali, la lamina midollare interna del talamo, lo splenio del corpo calloso. Non sono state invece riscontrate le più frequenti localizzazioni alla sostanza bianca emisferica cerebellare. In 7 dei nostri pazienti era presente il caratteristico segno "del panda" (iperintensità nelle immagini a TR lungo del mesencefalo con preservata ipointensità dei peduncoli cerebrali e dei nuclei rossi, della porzione laterale della pars reticulata della sostanza nera e dei collicoli superiori); in alcuni casi era presente una alterazione di segnale dei nuclei rossi, in altri un quadro tipo "mielinolisi centrale", con differenti gradi di gravità.L'atrofia corticale cerebrale è più frequentemente riportata in sede sopratentoriale rispetto a quella sottotentoriale; l'atrofia del tronco, meno comune, è stata riscontrata più frequentemente nei soggetti con sintomatologia tipo Westphal-Strumpell.Non meno importante il ruolo della RM nel follow-up post-terapeutico: Ìa metodica può evidenziare regressione totale o parziale delle lesioni, con elevata correlazione con il quadro clinico.

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MRI Manifestations of Wilson's Disease and its Change in Response to Treatment

Cited by 3 publications

References 5 publications

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

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

Cognitive functioning in neurologically symptomatic and asymptomatic forms of Wilson's disease

Malattia di Wilson: Reperti RM in 13 casi

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