Analysis of Running in Wilson’s Disease

Aim of the study was to analyze the ability of long-term treated patients with Wilson’s disease (WD) to run a distance of 40 m. 30 WD-patients from a single center were consecutively recruited. All patients were able to walk a distance of 40 m without walking aids. Vertical ground reaction forces (GRF-curves) were analyzed by means of an Infotronic® gait analysis system (CDG®) and correlated with clinical and laboratory findings. Results of the WD-patients were compared to those of an age-and sex-matched control group. 25 of the 30 WD-patients were able to run. Patients being unable to run had a significantly (p < 0.03) higher non-motor score. In comparison to the controls speed of running was significantly (p < 0.02) reduced in WD-patients. Their duration of foot contact on the ground lasted significantly (p < 0.05) longer. Running was more irregular in WD and the variability of times to peak of the GRF-curves was significantly (p < 0.05) increased. All running parameters extracted from the GRF-curves of the CDG® did not correlate with severity of WD. Cadence of running was significantly (p < 0.03) negatively correlated with serum liver enzyme levels. Running appears to be rather unimpaired in long-term treated WD, only 16% of the 30 WD-patients were unable to run. This knowledge is highly relevant for the patient management, but because of the missing correlation with severity of WD, analysis of running is of minor importance for monitoring WD-therapy.

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Mildly Impaired Foot Control in Long-Term Treated Patients with Wilson’s Disease

Journal of Functional Morphology and Kinesiology ◽

10.3390/jfmk7010005 ◽

2021 ◽

Vol 7 (1) ◽

pp. 5

Author(s):

Sara Samadzadeh ◽

Harald Hefter ◽

Osman Tezayak ◽

Dietmar Rosenthal

Keyword(s):

Gait Speed ◽

Wilson’S Disease ◽

Wilson's Disease ◽

The Body ◽

Gait Disturbance ◽

Control Group ◽

Initial Symptom ◽

Laboratory Findings ◽

Functional Deficit

Abnormal gait is a common initial symptom of Wilson’s disease, which responds well to therapy, but has not been analyzed in detail so far. In a pilot study, a mild gait disturbance could be detected in long-term treated Wilson patients. The question still is what the underlying functional deficit of this gait disturbance is and how this functional deficit correlates with further clinical and laboratory findings. In 30 long-term treated Wilson patients, the vertical component of foot ground reaction forces (GRF-curves) was analyzed during free walking without aid at the preferred gait speed over a distance of 40 m. An Infotronic® gait analysis system, consisting of soft tissue shoes with solid, but flexible plates containing eight force transducers, was used to record the pressure of the feet on the floor. Parameters of the GRF-curves were correlated with clinical scores as well as laboratory findings. The results of Wilson patients were compared to those of an age- and sex-matched control group. In 24 out of 30 Wilson patients and all controls, two peaks could be distinguished: the first “heel-on” and the second “push-off” peak. The heights of these peaks above the midstance valley were significantly reduced in the patients (p < 0.05). The time differences between peaks 1 or 2 and midstance valley were significantly negatively correlated with the total impairment score (p < 0.05). Gait speed was significantly correlated with the height of the “push-off” peak above the midstance valley (p < 0.045). The GRF-curves of free walking, long-term treated patients with Wilson’s disease showed a reduced “push-off” peak as an underlying deficit to push the center of mass of the body to the contralateral side with the forefoot, explaining the reduction in gait speed during walking.

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Effective Treatment of Neurological Symptoms with Normal Doses of Botulinum Neurotoxin in Wilson’s Disease: Six Cases and Literature Review

Toxins ◽

10.3390/toxins13040241 ◽

2021 ◽

Vol 13 (4) ◽

pp. 241

Author(s):

Harald Hefter ◽

Sara Samadzadeh

Keyword(s):

Botulinum Neurotoxin ◽

Wilson’S Disease ◽

Animal Experiments ◽

Symptomatic Treatment ◽

Wilson's Disease ◽

University Hospital ◽

Laboratory Findings ◽

Dose Information ◽

High Doses ◽

The University

Background: Recent cell-based and animal experiments have demonstrated an effective reduction in botulinum neurotoxin A (BoNT/A) by copper. Aim: We aimed to analyze whether the successful symptomatic BoNT/A treatment of patients with Wilson’s disease (WD) corresponds with unusually high doses per session. Methods: Among the 156 WD patients regularly seen at the outpatient department of the university hospital in Düsseldorf (Germany), only 6 patients had been treated with BoNT/A during the past 5 years. The laboratory findings, indications for BoNT treatment, preparations, and doses per session were extracted retrospectively from the charts. These parameters were compared with those of 13 other patients described in the literature. Results: BoNT/A injection therapy is a rare (<4%) symptomatic treatment in WD, only necessary in exceptional cases, and is often applied only transiently. In those cases for which dose information was available, the dose per session and indication appear to be within usual limits. Conclusion: Despite the evidence that copper can interfere with the botulinum toxin in preclinical models, patients with WD do not require higher doses of the toxin than other patients with dystonia.

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The study of the impacts of ‘Running’ on the contact area of soles and maximal strength among elite middle distance runners

Cypriot Journal of Educational Sciences ◽

10.18844/cjes.v12i1.1190 ◽

2017 ◽

Vol 12 (1) ◽

pp. 23-31 ◽

Cited By ~ 1

Author(s):

Ahmet Uzun ◽

Latif Aydos ◽

Metin Kaya ◽

Mehmet Fatih Yuksel ◽

Hacı Ahmet Pekel

Keyword(s):

Contact Area ◽

Middle Part ◽

Control Group ◽

Maximal Strength ◽

Distance Runners ◽

Male Athletes ◽

Maximal Force ◽

Analysis System ◽

The Right

It is possible that running training for many years in athletics affects athletes' running patterns and sole structure. The main aim of this study is to examine the effect of maximal force applied to the floor area and contact area of the athletes with related to mid-distance training for athletics. 18 male athletes who represent Turkey on the International area and another 25 male volunteers who form the control group from the participants of the study. The sole pressure measurement of the participants was performed through the use of EMED-SF (Novel H, Munich, Germany) plantar pressure analysis system (pedobarographic analysis). In the study, significant differences were found in heel medial, heel lateral, foot middle, 2nd, 3rd and 4th metatars heads and left foot 3rd, 4th, 5th finger in the contact area values of the right and left foot averages. Besides, significant differences were found in heel medial, middle part of feet, 2nd and 3rd metatars heads and 2nd, 3rd, 4th and 5th fingers in maximal force values. Athletes have pressed on sole of the feet more as a result of long-term training and as a consequence this pressure caused a change of the soles contact area and maximal strength. Also it can be said that they have used forefront part of soles more actively during running and walking. Keywords: runner foot sole, contact area, middle distance, pedobarographic.

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Long-term survival in patients with Wilson's disease

Hepatology ◽

10.1016/0270-9139(93)92583-l ◽

1993 ◽

Vol 18 (4) ◽

pp. A265

Author(s):

V BALAN

Keyword(s):

Wilson’S Disease ◽

Wilson's Disease ◽

Term Survival ◽

Long Term Survival

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Long-Term Care and Management of Wilson’s Disease in the GDR

European Neurology ◽

10.1159/000116435 ◽

1989 ◽

Vol 29 (6) ◽

pp. 301-305 ◽

Cited By ~ 11

Author(s):

Hans Bachmann ◽

Joachim Lössner ◽

Hans-Jürgen Kühn ◽

Dietmar Biesold ◽

Roland Siegemund ◽

...

Keyword(s):

Long Term Care ◽

Wilson’S Disease ◽

Wilson's Disease ◽

Term Care

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Long-term outcome of neurological Wilson's disease

Parkinsonism & Related Disorders ◽

10.1016/j.parkreldis.2018.01.007 ◽

2018 ◽

Vol 49 ◽

pp. 48-53 ◽

Cited By ~ 2

Author(s):

Harald Hefter ◽

Osman Tezayak ◽

Dietmar Rosenthal

Keyword(s):

Wilson’S Disease ◽

Wilson's Disease ◽

Term Outcome ◽

Long Term Outcome

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Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

Cardiology in the Young ◽

10.1017/s104795111900180x ◽

2019 ◽

Vol 29 (09) ◽

pp. 1183-1188

Author(s):

Asuman N. Karhan ◽

Hayrettin H. Aykan ◽

Ersin Gümüş ◽

Yasemin Dönmez ◽

Dursun Alehan ◽

...

Keyword(s):

Patient Group ◽

Doppler Echocardiography ◽

Wilson’S Disease ◽

Tissue Doppler ◽

Wilson's Disease ◽

Left Ventricular ◽

P Wave ◽

Tissue Doppler Echocardiography ◽

Control Group ◽

P Wave Dispersion

AbstractBackground:This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson’s disease.Method:Asymptomatic patients with a diagnosis of Wilson’s disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched.Results:The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias.Conclusion:Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.

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