Genetische Stoffwechselerkrankungen mit neuropathologischer Bedeutung

Harzer, K.; Breitbach, N.

doi:10.1007/978-3-642-85179-7_20

Cited by 1 publication

(2 citation statements)

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“…Nevertheless, in patients with Wilson's disease the cerebellar loop must be taken into account. This has been demonstrated by neuroimaging methods [24,29,36], by neuropathological cerebellar findings [7,13,24] and by changes of glucose utilisation [8,16,29]. Furthermore, abnormalities in the descending corticospinal motor system must be considered to be in part responsible for precision movement disorders.…”

Section: Discussionmentioning

confidence: 94%

“…Disturbances in precision movements remaining as residual symptoms after longterm therapy are signs of irreversible basal gangliar and cerebellar lesions [11]. Neuropathological changes can be demonstrated especially in basal ganglia including cellular necrosis predominantly in the putamen and in the cautate nucleus, less often in the globus pallidus, the subthalamic nucleus, the red nucleus, dentate nucleus, the substantia nigra and in the thalamic nuclei [7,13,24]. Correspondingly signal changes can be found in basal ganglia and in the cerebellum by cranial computed tomography as well as by magnetic resonance imaging [9,24,28,29,[35][36][37].…”

Section: Introductionmentioning

confidence: 98%

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Correlation between automated writing movements and striatal dopaminergic innervation in patients with Wilson's disease

Hermann

Eggers

Barthel³

et al. 2002

Journal of Neurology

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Handwriting defects are an early sign of motor impairment in patients with Wilson's disease. The basal ganglia being the primary site of copper accumulation in the brain suggests a correlation with lesions in the nigrostiatal dopaminergic system. We have analysed and correlated striatal dopaminergic innervation using [(123)I]beta-CIT-SPECT and automated handwriting movements in 37 patients with Wilson's disease. There was a significant correlation of putaminal dopaminergic innervation with fine motor ability (p < 0,05 for NIV [number of inversion in velocity], NIA [number of inversion in acceleration], frequency). These data suggest that loss of dorsolateral striatal dopaminergic innervation has a pathophysiological function for decreased automated motor control in Wilson's disease. Furthermore analysis of automated handwriting movements could be useful for therapy monitoring and evaluation of striatal dopaminergic innervation.

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