1.313 Unified Wilson's Disease Rating Scale (UWDRS) – a proposal for the neurological scoring of Wilson's disease patients

Członkowska, Anna; Tarnacka, Beata; Möller, J. Carsten; Leinweber, Barbara; Oertel, WH; Bandmann, Oliver; Woimant, F.

doi:10.1016/s1353-8020(08)70548-0

Cited by 63 publications

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“…Severity of neurological impairment may be scored using Unified Wilson's disease Rating Score (UWDRS) which consist 3 parts including: 1. conciousness, 2. changes in activities of daily life and 3. the results of the clinical neurological examination. 26,27 Contrary to HE, disturbed consciousness, pyramidal signs, myoclonus and/or asterixis in neurological cases of WD are very rarely observed. Another possibility to quantify the symptoms is the Global Assessment Scale (GAS) which includes global disability (tier 1: liver, cognition and behavior, motor, osseomuscular) and neurological assessment (tier 2: detailed neuropsychiatric).…”

Section: Neurological Manifestation Of Wilson Diseasementioning

confidence: 99%

Encephalopathy in Wilson Disease: Copper Toxicity or Liver Failure?

Ferenci

Litwin

Seniów

et al. 2015

Journal of Clinical and Experimental Hepatology

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Section: Neurological Manifestation Of Wilson Diseasementioning

confidence: 99%

Encephalopathy in Wilson Disease: Copper Toxicity or Liver Failure?

Ferenci

Litwin

Seniów

et al. 2015

Journal of Clinical and Experimental Hepatology

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“…[24][25][26][27] The other common neurological disturbances are hypokinesia, dystonia, dysarthria, gait apraxia, and further cerebellar-extrapyramidal disturbances. 4,27 There is no single reliable, noninvasive diagnostic test for WD. 21 Diagnosis relies mostly on the clinical, laboratory, and radiological fi ndings.…”

Section: Discussionmentioning

confidence: 99%

Structural and neurochemical evaluation of the brain and pons in patients with Wilson’s disease

Algın¹,

Taşkapılıoğlu

Hakyemez

et al. 2010

Jpn J Radiol

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“…Patients were evaluated before and after LT using both the modified Rankin scale and the more specific Unified Wilson's Disease Rating Scale (UWDRS) [6][7][8] . Items of bradykinesia, rigidity and dystonia were scored for left and right sides.…”

Section: Methodsmentioning

confidence: 99%

Liver Transplantation in Wilson's Disease with Neurological Impairment: Evaluation in 4 Patients

Laurencin¹,

Brunet

Dumortier

et al. 2016

Eur Neurol

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Background: The aim of this work is to report our early experiences about the benefits of liver transplantation (LT) in the treatment of persistent neurological symptoms in Wilson's disease (WD) patients. Methods: We describe our findings in 4 WD patients with neurological impairment or symptoms treated by LT: 2 patients had transplants due to worsening of neurological symptoms despite long-term appropriate medical treatment. The other 2 required LT because of symptoms associated with liver failure. Patients were evaluated using the modified Rankin scale and the Unified Wilson's Disease Rating Scale (UWDRS). Results: The 4 patients experienced neurological improvement after LT. The pre-LT Rankin score of the 2 patients transplanted due to neurological impairment was 4 compared to 3 and 2, respectively, post LT. The pre-LT Rankin scores of the 2 WD cases transplanted because of hepatic failure were 1 and 2, respectively, compared to 0 in both cases post LT. UWDRS score improved in 2 cases and remained stable in 1 less severely impaired case. Brain MRI abnormalities proved partially reversible in 3 patients and remained stable for 1 patient. Conclusions: These results suggest that LT could be envisaged for neurologically impaired WD patients.

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1.313 Unified Wilson's Disease Rating Scale (UWDRS) – a proposal for the neurological scoring of Wilson's disease patients

Cited by 63 publications

References 0 publications

Encephalopathy in Wilson Disease: Copper Toxicity or Liver Failure?

Encephalopathy in Wilson Disease: Copper Toxicity or Liver Failure?

Structural and neurochemical evaluation of the brain and pons in patients with Wilson’s disease

Liver Transplantation in Wilson's Disease with Neurological Impairment: Evaluation in 4 Patients

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