Evoked potentials in children with Wilson's disease

Topçu, Meral; Topçuoğlu, Mehmet Akif; Köse, Gülşen; Nurlu, Gülay; Turanlı, Güzide

doi:10.1016/s0387-7604(02)00055-4

Cited by 10 publications

(8 citation statements)

References 22 publications

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“…Our study revealed EP abnormality in 61% patients with neurologic WD; abnormal MEP in 35.7%, ABER in 61.5%, VEP in 57% and SEP in 30.7%. In earlier studies, MEP abnormality has been reported in 39-50%, SEP in 15-69%, VEP in 5.3-75% and ABER in 8.3-92% [8][9][10][11][12][13][14][15][16][17][18][19][20]. MEP abnormality in our study correlated with MRI involvement of motor pathway in four patients.…”

Section: Discussionsupporting

confidence: 80%

“…The varying frequency of EP abnormalities in different studies may be due to inclusion of mixed population of hepatic and neurological WD and selection of upper or lower limb MEP and SEP. The frequency of EP abnormality is higher in neurologic WD compared with hepatic [11,13,15].…”

Section: Discussionmentioning

confidence: 99%

“…Moreover, WD presents with predominant extrapyramidal features, pyramidal dysfunction may be overshadowed by rigidity and movement disorders which can be evaluated by motor evoked potential (MEP). On Medline search with key words evoked potentials and WD, we could retrieve 45 articles; out of which multimodality evoked potentials were performed in 14 studies; majority included both hepatic and neurologic WD, six correlated evoked potential changes with magnetic resonance imaging findings and only two conducted MEP [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]. In this communication, we report multimodality evoked potential changes in patients with neurologic WD and correlate these with their clinical severity and MRI findings.…”

Section: Introductionmentioning

confidence: 99%

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A study of clinical, MRI and multimodality evoked potentials in neurologic Wilson disease

Das

Misra

2007

Euro J of Neurology

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The aims of this study were evaluate motor, somatosensory, visual and auditory brainstem evoked potential (MEP, SEP, VEP, ABER) changes in Wilson disease (WD) and correlate these with magnetic resonance imaging (MRI) and clinical findings. Neurologic WD diagnosed on the basis of clinical, ceruloplasmin and Kayser-Fleischer ring were evaluated including pedigree charting, hepatic, renal, hematologic and osteoarticular manifestations. Blood counts, serum chemistry, MRI, MEP to tibialis anterior, tibial SEP, VEP and ABER were performed. Evoked potential (EP) changes were correlated with clinical and MRI findings. Eighteen WD patients were recruited from 17 families whose mean age was 16 years. Movement disorders were present in 14, cognitive decline in 12 and pyramidal signs in 12 patients. MRI revealed involvement of basal ganglia in 80%, thalamus in 40%, brain stem in 46.7% and subcortical white matter in 53.3%. MEP was abnormal in 35.7%, SEP in 30.8%, VEP in 57% and ABER in 61.5% patients; the latter three EP changes were subclinical. Frequency and number of EP abnormalities were higher with increasing severity of illness. SEP, VEP and ABER reveals subclinical abnormality and MEP helps in documenting both clinical and subclinical abnormalities. Number of EP abnormalities increases with increasing clinical severity of WD.

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Section: Discussionsupporting

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A study of clinical, MRI and multimodality evoked potentials in neurologic Wilson disease

Das

Misra

2007

Euro J of Neurology

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“…Existing research suggests that neurodegeneration in WD may be reflected in measurable retinal and PVEP findings (Topcu et al 2002 ; Albrecht et al 2012 ). Significant retinal thinning was previously observed in WD patients via OCT (Albrecht et al 2012 ).…”

Section: Discussionmentioning

confidence: 99%

Optical coherence tomography and electrophysiology of retinal and visual pathways in Wilson’s disease

et al. 2015

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We evaluated correlations between positive findings of changes on brain magnetic resonance imaging (MRI) and selected morphological and electrophysiological parameters of the retinal and visual systems in Wilson’s disease. Fifty-eight Wilson’s disease symptomatic patients were divided according to whether they displayed brain changes on MRI (positive, n = 39; negative, n = 19). All participants and healthy control group (n = 30), underwent retinal optical coherence tomography to assess the thickness of macula and the total retinal nerve fiber layer. Visual evoked potentials were measured and electroretinography was performed. Macular and retinal nerve fibers were thinner in participants with changes on MRI than in participants without changes. Electrophysiological parameters were markedly different in the MRI positive group compared with the negative group and 30 healthy controls; however, some abnormalities were evident in cases without visible brain pathology. Morphological and electrophysiological changes of retinal and visual pathways are associated with MRI visualized brain injury in Wilson’s disease and may be useful for detecting the degree of neurodegeneration.

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“…Histological studies have reported necrosis, gliosis and cystic changes in the brainstem, thalamus, cerebellum and cerebral cortex of Wilson’s disease patients [4]. The functional consequences of these structural changes have been demonstrated in the acoustic, sensory, motor and visual systems and are reflected by disordered multimodality evoked potentials [10]–[13]. Visual evoked potentials (VEPs) have been reported to be abnormal in approximately 50% of symptomatic Wilson’s disease patients [10], [11], [14]–[16].…”

Section: Introductionmentioning

confidence: 99%

Retinal Neurodegeneration in Wilson’s Disease Revealed by Spectral Domain Optical Coherence Tomography

et al. 2012

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Background/ObjectiveIn addition to cirrhosis of the liver, Wilson’s disease leads to copper accumulation and widespread degeneration of the nervous system. Delayed visual evoked potentials (VEPs) suggest changes to the visual system and potential structural changes of the retina.MethodsWe used the latest generation of spectral domain optical coherence tomography to assess the retinal morphology of 42 patients with Wilson’s disease and 76 age- and sex-matched controls. We measured peripapillary retinal nerve fiber layer (RNFL) thickness and total macular thickness and manually segmented all retinal layers in foveal scans of 42 patients with Wilson’s disease and 76 age- and sex-matched controls. The results were compared with VEPs and clinical parameters.ResultsThe mean thickness of the RNFL, paramacular region, retinal ganglion cell/inner plexiform layer and inner nuclear layer was reduced in Wilson’s disease. VEPs were altered with delayed N75 and P100 latencies, but the N140 latency and amplitude was unchanged. An analysis of the laboratory parameters indicated that the serum concentrations of copper and caeruloplasmin positively correlated with the thickness of the outer plexiform layer and with N75 and P100 VEP latencies.ConclusionNeuronal degeneration in Wilson’s disease involves the retina and changes can be quantified by optical coherence tomography. While the VEPs and the thickness of the outer plexiform layer appear to reflect the current copper metabolism, the thicknesses of the RNFL, ganglion cell/inner plexiform layer, inner nuclear layer and the total paramacular thickness may be the best indicators of chronic neuronal degeneration.

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Evoked potentials in children with Wilson's disease

Cited by 10 publications

References 22 publications

A study of clinical, MRI and multimodality evoked potentials in neurologic Wilson disease

A study of clinical, MRI and multimodality evoked potentials in neurologic Wilson disease

Optical coherence tomography and electrophysiology of retinal and visual pathways in Wilson’s disease

Retinal Neurodegeneration in Wilson’s Disease Revealed by Spectral Domain Optical Coherence Tomography

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