Klinische und feinmotorische Therapiekontrolle bei Morbus Wilson

Hermann, W.; Tietze, F.; Villmann, Thomas; Grahmann, F.; Wagner, A.

doi:10.1007/s001150050694

Cited by 3 publications

(1 citation statement)

References 5 publications

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“…According to this system each patient was judged before (score A) and after long term therapy (score B), (see Tab. 4), [11]. This system comprises the assessment of resting and holding tremor, dysdiadochokinesia, gate disturbances, brady-and hypokinesia as well as rigor while only dysarthrophonia yields the value 1.…”

Section: Methods S Normal Volunteers and Patientsmentioning

confidence: 99%

Comparison of clinical types of Wilson's disease and glucose metabolism in extrapyramidal motor brain regions

Hermann

Barthel

Hesse

et al. 2002

Journal of Neurology

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In Wilson's disease a disturbed glucose metabolism especially in striatal and cerebellar areas has been reported. This is correlated with the severity of extrapyramidal motor symptoms (EPS). These findings are only based on a small number of patients. Up to now it is unknown whether EPS are caused by various patterns of disturbed basal ganglia glucose metabolism. We investigated 37 patients and 9 normal volunteers to characterize the disturbed glucose metabolism in Wilson's disease more precisely. The glucose metabolism was determined in 5 cerebellar and cerebral areas (putamen, caput nuclei caudati, cerebellum, midbrain and thalamic area) by using (18)F-Fluorodesoxyglucose-Positron-Emission-Tomography ( [(18)F]FDG-PET). The database was evaluated by a cluster analysis. Additionally, the severity extrapyramidal motor symptoms were judged by a clinical score system. Three characteristic patterns of glucose metabolism in basal ganglia were obtained. Two of them may be assigned to patients with neurological symptoms whereas the third cluster corresponds to most patients without EPS or normal volunteers. The clusters can be identified by characteristic consumption rates in this 5 brain areas. The severity of EPS can not clearly be assigned to one of the clusters with disturbed glucose metabolism. However, the most severe cases are characterized by the lowest consumption in the striatal area. When there is marked improvement of EPS impaired glucose consumption reveals a persistent brain lesion. Finally, the neurological symptoms in Wilson's disease are caused by (at least) two different patterns of disturbed glucose metabolism in basal ganglia and cerebellum. The severity of EPS seems to be determined by a disturbed consumption in the striatal area.

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Section: Methods S Normal Volunteers and Patientsmentioning

confidence: 99%

Comparison of clinical types of Wilson's disease and glucose metabolism in extrapyramidal motor brain regions

Hermann

Barthel

Hesse

et al. 2002

Journal of Neurology

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Regional serotonin transporter availability and depression are correlated in Wilson?s disease

Hesse

Barthel

Hermann

et al. 2003

Journal of Neural Transmission

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In patients with Wilson's disease (WD), depression is a frequent psychiatric symptom. In vivo neuroimaging studies suggest that depression and other neuropsychiatric disorders are associated with central serotonergic deficits. However, in vivo measurements of serotonergic neurotransmission have not until now been performed in patients with this copper deposition disorder. The present prospective study revealed that depressive symptomatology is related to an alteration of presynaptic serotonin transporters (SERT) availability as measured by [123I]-2beta-carbomethoxy-3beta-(iodophenyl)tropane ([123I]beta-CIT) and high-resolution single-photon emission computed tomography (SPECT). SERT imaging with [123I]beta-CIT-SPECT could therefore become a useful tool for diagnosis and therapy monitoring in depressed WD patients.

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Classification of fine-motoric disturbances in Wilson's disease using artificial neural networks

et al. 2005

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Patients suffering from Wilson's disease are divided into several types according clinical symptoms only at time of manifestation. Thereby two main subgroups exist: neurologic and non-neurologic types. After long-term therapy the neurological symptoms occurring in hepatolenticular degeneration may be improved but frequently with remaining fine-motoric disturbances which should be used for evaluation of the actual patient state. These disturbances are difficult to assess in an exact and objective manner by clinical examination. Therefore we measured fine-motoric passive and active abilities based on a standardized test set using the VSCOPE-system. The parallel evaluation of all fine-motoric data using an artificial neural network leads to a reclassification of these patients based on actual fine-motoric abilities but not reflecting the clinical classification at time of manifestation.

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Klinische und feinmotorische Therapiekontrolle bei Morbus Wilson

Cited by 3 publications

References 5 publications

Comparison of clinical types of Wilson's disease and glucose metabolism in extrapyramidal motor brain regions

Comparison of clinical types of Wilson's disease and glucose metabolism in extrapyramidal motor brain regions

Regional serotonin transporter availability and depression are correlated in Wilson?s disease

Classification of fine-motoric disturbances in Wilson's disease using artificial neural networks

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