P.1.g.007 Cerebellar dysfunction in male schizophrenic patients: clinical and cognitive correlates of the unified Wilson's disease rating scale (ICARS)

2007 ◽  
Vol 17 ◽  
pp. S306
Author(s):  
S.Y. Kim ◽  
Y.J. Kwon ◽  
S.H. Shim ◽  
H.Y. Jung
Keyword(s):  
Wilson’S Disease ◽  
Rating Scale ◽  
Wilson's Disease ◽  
Cerebellar Dysfunction ◽  
Cognitive Correlates ◽  
Schizophrenic Patients ◽  
Disease Rating

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

2007 ◽  
Vol 13 ◽  
pp. S80 ◽  
Author(s):  
A. Czlonkowska ◽  
B. Tarnacka ◽  
J. Carsten Möller ◽  
B. Leinweber ◽  
W.H. Oertel ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Rating Scale ◽  
Wilson's Disease ◽  
Disease Rating

Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German patients with treated Wilson's disease

2008 ◽  
Vol 23 (1) ◽  
pp. 54-62 ◽  
Author(s):  
Barbara Leinweber ◽  
J. Carsten Möller ◽  
Andre Scherag ◽  
Ulrike Reuner ◽  
Peter Günther ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Rating Scale ◽  
Wilson's Disease ◽  
Disease Rating

FP04-MO-05 Neurological signs of Wilson' disease assessed by unified Wilson's disease rating scale (UWDRS)

2009 ◽  
Vol 285 ◽  
pp. S63
Author(s):  
A. Czlonkowska ◽  
C. Möller ◽  
G. Chabik ◽  
M. Lesniak ◽  
T. Litwin ◽  
...  
Keyword(s):  
Wilson Disease ◽  
Wilson’S Disease ◽  
Rating Scale ◽  
Wilson's Disease ◽  
Neurological Signs ◽  
Disease Rating

scholarly journals Which factors are associated with global cognitive impairment in Wilson's disease?

2016 ◽  
Vol 10 (4) ◽  
pp. 320-326 ◽  
Author(s):  
Norberto Anízio Ferreira Frota ◽  
Egberto Reis Barbosa ◽  
Claudia Sellitto Porto ◽  
Leandro Tavares Lucato ◽  
Carla Rachel Ono ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Globus Pallidus ◽  
Educational Level ◽  
Wilson’S Disease ◽  
Rating Scale ◽  
Poor Performance ◽  
Wisconsin Card Sorting Test ◽  
Wilson's Disease ◽  
Cognitive Test ◽  
Card Sorting

ABSTRACT Background: Patients with Wilson's disease (WD) present cognitive impairment, especially in executive functions. Which other factors might be associated with global cognitive decline in these patients remains unclear. Objective: To assess which factors are associated with worse performance on a global cognitive test in patients with WD. Methods: Twenty patients with WD underwent cognitive assessment with the following tests: the Mini-Mental State Examination (MMSE), Dementia Rating Scale (DRS), verbal fluency test, brief cognitive battery, clock drawing test, Frontal Assessment Battery, Stroop test, Wisconsin card sorting test, Hopper test, cubes (WAIS) and the Pfeffer questionnaire. MRI changes were quantified. Patients with poor performance on the DRS were compared to patients with normal performance. Results: Nine patients had a poor performance on the DRS. This group had lower educational level (9.11±3.58 × 12.82±3.06) and a greater number of changes on MRI (9.44±2.74 × 6.27±2.45). The presence of hyperintensity in the globus pallidus on MRI was more frequent in this group (66.6% vs 9.0%), with OR=5.38 (95% CI 0.85-33.86). Conclusion: Global cognitive impairment was prevalent in this sample of patients with WD and was associated with low educational level, number of changes on MRI and MRI hyperintensity in the globus pallidus.

scholarly journals Wilson’s Disease Presenting with Generalized Tonic-Clonic Seizure and Cerebellar Dysfunction

2021 ◽  
Vol Volume 14 ◽  
pp. 529-532
Author(s):  
Aziz Rahman Rasib ◽  
Aemal Aziz Jabarkhil ◽  
Mohammad Faiq Sediqi ◽  
Ahmad Irshad Mansoor ◽  
Abdullah Asady
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Clonic Seizure ◽  
Tonic Clonic Seizure ◽  
Cerebellar Dysfunction

scholarly journals Clinical significance of self-descriptive apathy assessment in patients with neurological form of Wilson’s disease

2021 ◽  
Author(s):  
Marcin Leśniak ◽  
Magdalena Roessler-Górecka ◽  
Anna Członkowska ◽  
Joanna Seniów
Keyword(s):  
Wilson’S Disease ◽  
Rating Scale ◽  
Neuropsychiatric Symptoms ◽  
Executive Dysfunction ◽  
Wilson's Disease ◽  
Clinical Syndrome ◽  
Assessment Tools ◽  
Self Awareness ◽  
Clinical Scales ◽  
Proxy Version

Abstract Background and aim Apathy is one of the neuropsychiatric symptoms of Wilson’s disease (WD) which typically affects the brain’s fronto-basal circuits. Lack of agreed diagnostic criteria and common use of self-description assessment tools lead to underestimation of this clinical phenomenon. The aim of this study was to investigate whether subjective and informant-based clinical features of apathy in patients with WD enable clinicians to make a valid diagnosis. Methods Multiple aspects of goal-oriented behavior were assessed in 30 patients with the neurological form of WD and 30 age-matched healthy participants using two questionnaires, the Lille Apathy Rating Scale (LARS) and the Dysexecutive Questionnaire (DEX). Both included a self-descriptive and a caregiver/proxy version. Cognitive functioning was estimated with the use of Addenbrooke’s Cognitive Examination-Revised. Results Patients obtained significantly worse scores on all clinical scales when more objective measures were considered. Features of apathy and executive dysfunction were revealed in patients’ caregiver versions of LARS and DEX, which may indicate poor self-awareness of patients with WD. Roughly 30% of participants were likely to present with clinically meaningful symptoms, independent of cognitive dysfunction. Conclusions Methods relying on self-description appear inferior to informant-based scales when diagnosing apathy. More objective criteria and measurement tools are needed to better understand this clinical syndrome.

scholarly journals Multimodal magnetic resonance imaging analysis in the characteristics of Wilson’s disease: A case report and literature review

2021 ◽  
Vol 16 (1) ◽  
pp. 793-799
Author(s):  
Yun Wang ◽  
Zejin Jia ◽  
Yuelei Lyu ◽  
Qian Dong ◽  
Shujuan Li ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Sanger Sequencing ◽  
Wilson’S Disease ◽  
Rating Scale ◽  
Wilson's Disease ◽  
Resonance Imaging ◽  
Conventional Mri ◽  
Lenticular Nucleus ◽  
Multimodal Mri

Abstract Wilson’s disease (WD) is an inherited disorder of copper metabolism. Multimodal magnetic resonance imaging (MRI) has been reported to provide evidence of the extent and severity of brain lesions. However, there are few studies related to the diagnosis of WD with multimodal MRI. Here, we reported a WD patient who was subjected to Sanger sequencing, conventional MRI, and multimodal MRI examinations, including susceptibility-weighted imaging (SWI) and arterial spin labeling (ASL). Sanger sequencing demonstrated two pathogenic mutations in exon 8 of the ATP7B gene. Slit-lamp examination revealed the presence of Kayser–Fleischer rings in both eyes, as well as low serum ceruloplasmin and high 24-h urinary copper excretion on admission. Although the substantia nigra, red nucleus, and lenticular nucleus on T1-weighted imaging and T2-weighted imaging were normal, SWI and ASL showed hypointensities in these regions. Besides, decreased cerebral blood flow was found in the lenticular nucleus and the head of caudate nucleus. The patient recovered well after 1 year and 9 months of follow-up, with only a Unified Wilson Disease Rating Scale score of 1 for neurological symptom. Brain multimodal MRI provided a thorough insight into the WD, which might make up for the deficiency of conventional MRI.

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