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

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.

scholarly journals Analysis of Running in Wilson’s Disease

Sports ◽  
2022 ◽  
Vol 10 (1) ◽  
pp. 11
Author(s):  
Sara Samadzadeh ◽  
Harald Hefter ◽  
Osman Tezayak ◽  
Dietmar Rosenthal
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Control Group ◽  
Minor Importance ◽  
Laboratory Findings ◽  
Walking Aids ◽  
Reaction Forces ◽  
Analysis System ◽  
Vertical Ground Reaction Forces

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.

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

Toxins ◽  
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.

scholarly journals Effects of Long-Term Hydrogen Intervention on Plasma Metabolites and Gut Microbiome of Rats in Health Status

2021 ◽  
Author(s):  
Fei Xie ◽  
Xue Jiang ◽  
Yang Yi ◽  
Zi-Jia Liu ◽  
Chen Ma ◽  
...  
Keyword(s):  
Health Status ◽  
Gut Microbiota ◽  
Biological Effects ◽  
Enrichment Analysis ◽  
The Body ◽  
Plasma Metabolites ◽  
Control Group ◽  
Pathway Enrichment Analysis ◽  
Rrna Gene

Abstract The potential for preventive and therapeutic applications of H2 have now been confirmed in various disease. However, the effects of H2 on health status have not been fully elucidated. Our previous study reported changes in the body weight and 13 serum biochemical parameters during the six-month hydrogen intervention. To obtain a more comprehensive understanding of the effects of long-term hydrogen consumption, the plasma metabolome and gut microbiota were investigated in this study. Compared with the control group, 14 and 10 differential metabolites (DMs) were identified in hydrogen-rich water (HRW) and hydrogen inhalation (HI) group, respectively. Pathway enrichment analysis showed that HRW intake mainly affected starch and sucrose metabolism, and DMs in HI group were mainly enriched in arginine biosynthesis. 16S rRNA gene sequencing showed that HRW intake induced significant changes in the structure of gut microbiota, while no marked bacterial community differences was observed in HI group. HRW intake mainly induced significant increase in the abundance of Lactobacillus, Ruminococcus, Clostridium XI, and decrease in Bacteroides. HI mainly induced decreased abundances of Blautia and Paraprevotella. The results of this study provide basic data for further research on hydrogen medicine. Determination of the effects of hydrogen intervention on microbiota profiles could also shed light on identification of mechanism underlying the biological effects of molecular hydrogen.

Wilson's Disease

1984 ◽  
Vol 5 (7) ◽  
pp. 217-222
Author(s):  
Caroline A. Riely
Keyword(s):  
Wilson’S Disease ◽  
Acute Phase Reactant ◽  
Wilson's Disease ◽  
Clinical Findings ◽  
The Body ◽  
Hepatolenticular Degeneration ◽  
Ferric Ions ◽  
Pediatric Age Group ◽  
Life Threatening ◽  
Response To Stress

Wilson's disease (hepatolenticular degeneration) is a relatively rare cause of illness in the pediatric age group. But, as a chronic life-threatening disease that is treatable, even "curable," its investigation should be thoroughly pursued by the pediatrician. The recognition of Wilson's disease provides the opportunity to prevent this illness in presymptomatic family members. Therefore, Wilson's disease should be included in the differential diagnosis of all forms of liver disease in pediatrics and appropriately excluded. The clinical findings that are compatible with a diagnosis of Wilson's disease are protean, and confirming or denying this diagnosis is often difficult. In order to appreciate these problems, it is necessary first to understand the normal physiology of copper in the body and the derangements in this homeostasis that characterize Wilson's disease (Fig 1). COPPER HOMEOSTASIS Normal Copper is absorbed from the diet in excess of the body's requirement for it. Many foods contain copper, but chocolate, nuts, mushrooms, liver, and shellfish are particularly rich sources. Once absorbed, copper is transported free in the blood to the liver. Here, a certain fraction is incorporated in an irreversible fashion into ceruloplasmin. This copper-containing protein separates in the α2-globulins, can be an acute-phase reactant, increasing in concentration in response to stress. Alternatively, in the presence of hepatic failure with decrerased protein synthesis, its concentration in blood decreases. Ceruloplasmin is an oxidative enzyme used in a variety of pathways, including the oxidation of ferrous to ferric ions.

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

1989 ◽  
Vol 29 (6) ◽  
pp. 301-305 ◽  
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

Long-term outcome of neurological Wilson's disease

2018 ◽  
Vol 49 ◽  
pp. 48-53 ◽  
Author(s):  
Harald Hefter ◽  
Osman Tezayak ◽  
Dietmar Rosenthal
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Term Outcome ◽  
Long Term Outcome

Focal Ballismus (Right Arm) in a Boy with Wilson's Disease

2020 ◽  
Author(s):  
Roshan Koul ◽  
Priti Vijay ◽  
Rajeev Khanna ◽  
Seema Alam
Keyword(s):  
Basal Ganglia ◽  
Subthalamic Nucleus ◽  
Wilson’S Disease ◽  
Involuntary Movement ◽  
Wilson's Disease ◽  
Vascular Trauma ◽  
The Body ◽  
Lower Limbs ◽  
Rare Entity ◽  
The Right

AbstractBallismus is defined as rapid, forceful, shaking or throwing involuntary movement of the extremities, mainly proximal parts. When it affects half of the body (upper and lower limbs on one side), it is called hemiballismus. If it affects one limb, it could be labeled as focal or one limb ballismus. Any lesion (vascular, trauma, tumor, deposition, and demyelination) in the subthalamic nucleus of the basal ganglia results in ballismus. A rare entity such as copper deposition in Wilson's disease can manifest as ballismus. We describe a boy with Wilson's disease with focal ballismus of the right arm.

scholarly journals BCI-Based Rehabilitation on the Stroke in Sequela Stage

2020 ◽  
Vol 2020 ◽  
pp. 1-10
Author(s):  
Yangyang Miao ◽  
Shugeng Chen ◽  
Xinru Zhang ◽  
Jing Jin ◽  
Ren Xu ◽  
...  
Keyword(s):  
Motor Function ◽  
The Body ◽  
Control Group ◽  
Stroke Patients ◽  
Time Period ◽  
Average Improvement ◽  
Study Results ◽  
Electroencephalogram Eeg ◽  
Positive Effect

Background. Stroke is the leading cause of serious and long-term disability worldwide. Survivors may recover some motor functions after rehabilitation therapy. However, many stroke patients missed the best time period for recovery and entered into the sequela stage of chronic stroke. Method. Studies have shown that motor imagery- (MI-) based brain-computer interface (BCI) has a positive effect on poststroke rehabilitation. This study used both virtual limbs and functional electrical stimulation (FES) as feedback to provide patients with a closed-loop sensorimotor integration for motor rehabilitation. An MI-based BCI system acquired, analyzed, and classified motor attempts from electroencephalogram (EEG) signals. The FES system would be activated if the BCI detected that the user was imagining wrist dorsiflexion on the instructed side of the body. Sixteen stroke patients in the sequela stage were randomly assigned to a BCI group and a control group. All of them participated in rehabilitation training for four weeks and were assessed by the Fugl-Meyer Assessment (FMA) of motor function. Results. The average improvement score of the BCI group was 3.5, which was higher than that of the control group (0.9). The active EEG patterns of the four patients in the BCI group whose FMA scores increased gradually became centralized and shifted to sensorimotor areas and premotor areas throughout the study. Conclusions. Study results showed evidence that patients in the BCI group achieved larger functional improvements than those in the control group and that the BCI-FES system is effective in restoring motor function to upper extremities in stroke patients. This study provides a more autonomous approach than traditional treatments used in stroke rehabilitation.

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

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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