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An EEG study of Wilson’s disease
Society Proceedings / Clinical Neurophysiology 118 (2007) e9–e116
tion studies. We examined the usefulness of HVS in 19 patients with plexus lesions of different etiologies especially to detect the site of acute demyelinating lesion. Methods: 19 patients (17–82 years, mean 53.7) with unilateral plexus lesions (1–56 days from onset, mean 21.6) were examined amongst others clinically and radiologically (mostly with MRI). Serum- and/or CSF-analysis were performed in cases suspect of inflammatory etiology. HVS was undertaken to deltoideus, biceps brachii, or abductor digiti minimi muscle to examine the brachial plexus. Vastus medialis or tibialis anterior muscle were used for the lumbosacral plexus. Muscles were chosen regarding the main focus of the paresis. The measurements were performed according to a standardized protocol and the contralateral, healthy side was always used as a reference concerning latencies. Analysis was done in the usual way for motor conduction studies with the exception of the conduction velocity which cannot be determined in proximal sites. Results: Partial conduction block (PCB) could be found in 9 patients (47.4%), axonal lesion (AL) in 5 (26.3%), 4 patients (21.1%) showed combined PCB and AL. One patient had normal results (5.3%). As to be expected older lesions (2 or 3 weeks and more) tend to result in AL, younger (especially within the first 2 or 3 weeks) in PCB in our collective. Within the different etiologies no ‘‘typical’’ lesion pattern could be discerned. Conclusions: We were able to detect the site of the lesion with HVS in 68.5% of our patients. In our experience HVS is a very useful electrodiagnostic tool especially in early stages of plexus lesions. doi:10.1016/j.clinph.2006.11.083
Delayed feedback of somato-motor cortex activations modulates finger tapping results in real-time functional MR imaging—J. Gawehn 1, G. Vucurevic 1, P. Dellani 1, P. Urban 2, P. Stoeter 1 (1 Institut fu¨r Neuroradiologie, Universita¨t Mainz, 2 Klinik fu¨r Neurologie, Universita¨t Mainz) The objective of this study was to examine the effect of a slightly delayed feedback projection of real-time fMRI motor activations into the scanner on the performance of the subsequent tasks during the same session and/or on the performance during a second session one week later. All subjects were informed about the aim of the study: to modulate their cortical activation in accordance to the demand of the task. The paradigm consisted out of three 112.5-s-runs of self-paced finger tapping of the right hand with increasing demand (low, medium and high) for the modalities: strength, complexity and velocity. All measurements were performed in a 1.5 T MR scanner (Sonata, Siemens) using an eight channel head coil and the real-time fMRI software supplied by the manufacturer. Activation results were recorded online from the contralateral and ipsilateral senso-motor cortices and the supplementary motor area (SMA). In the examination group (8 volunteers), the results were projected as a column graph into the scanner with a delay of 2–3 s after the end of each run. In the control group (5 volunteers), no back-projection was performed. For evaluation, we determined the number of activated voxels and the ‘‘correctness of performance’’, i.e. the number of runs showing increasing activations with increasing demand of task. In comparison to the control group, the subjects of the feedback group showed a significantly better correlation between task
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demand and activation in the contralateral senso-motor cortex and the SMA during the second session and between the sessions. This applied as well to the number of activated voxels achieved between the runs and sessions (p < 0.05) as to the number of ‘‘correctly performed’’ runs in the sense of increasing activation from low to high task demand (p < 0.001). This improvement of modulation of cortical activation may be due to increased attention and/or a learning effect induced by delayed feedback and in future could be helpful in rehabilitation training programs. doi:10.1016/j.clinph.2006.11.084
An EEG study of Wilson’s disease—P. Gu¨nther 1, W. Hermann 2, A. Wagner 1 (1 Klinik und Poliklinik fu¨r Neurologie, Universita¨t Leipzig, 2 Paracelsus-Klinik Zwickau) Introduction: Wilson’s disease is a rare autosomal recessive disorder of copper metabolism. In addition to hepatic and extrapyramidal motor clinical symptoms, patients with Wilson’s disease also exhibit subclinical disorders of other central nervous pathways. However, abnormalities of electroencephalography are reported inconsistently and infrequently. Methods: In this study, electroencephalography was performed in 38 patients with Wilson’s disease (26 patients with the neurological form, 12 patients with the non-neurological form) undergoing long-term medicamentous therapy. Attention was paid to general slowing, focal abnormalities and epileptiform activity. Results of EEG were correlated to clinical manifestation. Results: The results of electroencephalography show normal findings in almost 80% of all investigated patients with a long course of the disease. A mild continuous slow activity occurred in 5 patients and intermitted rhythmic delta activity was seen in 2 patients with a neurologic form of hepatolenticular degeneration. A difference in the median frequency of a-rhythm between neurological form (9.1/s) and non-neurological form (9.7/s) was not significant. Epileptiform activity was shown in only one patient with coincident epilepsy. Conclusions: Patients with Wilson’s disease show normalized electroencephalographic findings in the course of the disease. Abnormal changes seem to be due to additional disturbances, respectively, secondary complications. In that regard electroencephalography is helpful in the differential diagnostic in the course of the disease. doi:10.1016/j.clinph.2006.11.085
Variants and differential diagnosis of peripheral nerve hyperexcitability—H.J. Gdynia, A.C. Ludolph, A.D. Sperfeld (Neurologische Universita¨tsklinik Ulm) Aims: Peripheral nerve hyperexcitability syndromes (PNHS) are a heterogeneous group of diseases characterized by spontaneous muscle fiber activity, e.g. myokymias and fasciculations. Acquired, hereditary and idiopathic forms are known. Antibodies to voltage-gated potassium channels (VGKCs) appear likely to be the main effector mechanism in many patients with acquired PNHS. Neuromyotonia (Isaacs’ syndrome when acquired),
tion studies. We examined the usefulness of HVS in 19 patients with plexus lesions of different etiologies especially to detect the site of acute demyelinating lesion. Methods: 19 patients (17–82 years, mean 53.7) with unilateral plexus lesions (1–56 days from onset, mean 21.6) were examined amongst others clinically and radiologically (mostly with MRI). Serum- and/or CSF-analysis were performed in cases suspect of inflammatory etiology. HVS was undertaken to deltoideus, biceps brachii, or abductor digiti minimi muscle to examine the brachial plexus. Vastus medialis or tibialis anterior muscle were used for the lumbosacral plexus. Muscles were chosen regarding the main focus of the paresis. The measurements were performed according to a standardized protocol and the contralateral, healthy side was always used as a reference concerning latencies. Analysis was done in the usual way for motor conduction studies with the exception of the conduction velocity which cannot be determined in proximal sites. Results: Partial conduction block (PCB) could be found in 9 patients (47.4%), axonal lesion (AL) in 5 (26.3%), 4 patients (21.1%) showed combined PCB and AL. One patient had normal results (5.3%). As to be expected older lesions (2 or 3 weeks and more) tend to result in AL, younger (especially within the first 2 or 3 weeks) in PCB in our collective. Within the different etiologies no ‘‘typical’’ lesion pattern could be discerned. Conclusions: We were able to detect the site of the lesion with HVS in 68.5% of our patients. In our experience HVS is a very useful electrodiagnostic tool especially in early stages of plexus lesions. doi:10.1016/j.clinph.2006.11.083
Delayed feedback of somato-motor cortex activations modulates finger tapping results in real-time functional MR imaging—J. Gawehn 1, G. Vucurevic 1, P. Dellani 1, P. Urban 2, P. Stoeter 1 (1 Institut fu¨r Neuroradiologie, Universita¨t Mainz, 2 Klinik fu¨r Neurologie, Universita¨t Mainz) The objective of this study was to examine the effect of a slightly delayed feedback projection of real-time fMRI motor activations into the scanner on the performance of the subsequent tasks during the same session and/or on the performance during a second session one week later. All subjects were informed about the aim of the study: to modulate their cortical activation in accordance to the demand of the task. The paradigm consisted out of three 112.5-s-runs of self-paced finger tapping of the right hand with increasing demand (low, medium and high) for the modalities: strength, complexity and velocity. All measurements were performed in a 1.5 T MR scanner (Sonata, Siemens) using an eight channel head coil and the real-time fMRI software supplied by the manufacturer. Activation results were recorded online from the contralateral and ipsilateral senso-motor cortices and the supplementary motor area (SMA). In the examination group (8 volunteers), the results were projected as a column graph into the scanner with a delay of 2–3 s after the end of each run. In the control group (5 volunteers), no back-projection was performed. For evaluation, we determined the number of activated voxels and the ‘‘correctness of performance’’, i.e. the number of runs showing increasing activations with increasing demand of task. In comparison to the control group, the subjects of the feedback group showed a significantly better correlation between task
e33
demand and activation in the contralateral senso-motor cortex and the SMA during the second session and between the sessions. This applied as well to the number of activated voxels achieved between the runs and sessions (p < 0.05) as to the number of ‘‘correctly performed’’ runs in the sense of increasing activation from low to high task demand (p < 0.001). This improvement of modulation of cortical activation may be due to increased attention and/or a learning effect induced by delayed feedback and in future could be helpful in rehabilitation training programs. doi:10.1016/j.clinph.2006.11.084
An EEG study of Wilson’s disease—P. Gu¨nther 1, W. Hermann 2, A. Wagner 1 (1 Klinik und Poliklinik fu¨r Neurologie, Universita¨t Leipzig, 2 Paracelsus-Klinik Zwickau) Introduction: Wilson’s disease is a rare autosomal recessive disorder of copper metabolism. In addition to hepatic and extrapyramidal motor clinical symptoms, patients with Wilson’s disease also exhibit subclinical disorders of other central nervous pathways. However, abnormalities of electroencephalography are reported inconsistently and infrequently. Methods: In this study, electroencephalography was performed in 38 patients with Wilson’s disease (26 patients with the neurological form, 12 patients with the non-neurological form) undergoing long-term medicamentous therapy. Attention was paid to general slowing, focal abnormalities and epileptiform activity. Results of EEG were correlated to clinical manifestation. Results: The results of electroencephalography show normal findings in almost 80% of all investigated patients with a long course of the disease. A mild continuous slow activity occurred in 5 patients and intermitted rhythmic delta activity was seen in 2 patients with a neurologic form of hepatolenticular degeneration. A difference in the median frequency of a-rhythm between neurological form (9.1/s) and non-neurological form (9.7/s) was not significant. Epileptiform activity was shown in only one patient with coincident epilepsy. Conclusions: Patients with Wilson’s disease show normalized electroencephalographic findings in the course of the disease. Abnormal changes seem to be due to additional disturbances, respectively, secondary complications. In that regard electroencephalography is helpful in the differential diagnostic in the course of the disease. doi:10.1016/j.clinph.2006.11.085
Variants and differential diagnosis of peripheral nerve hyperexcitability—H.J. Gdynia, A.C. Ludolph, A.D. Sperfeld (Neurologische Universita¨tsklinik Ulm) Aims: Peripheral nerve hyperexcitability syndromes (PNHS) are a heterogeneous group of diseases characterized by spontaneous muscle fiber activity, e.g. myokymias and fasciculations. Acquired, hereditary and idiopathic forms are known. Antibodies to voltage-gated potassium channels (VGKCs) appear likely to be the main effector mechanism in many patients with acquired PNHS. Neuromyotonia (Isaacs’ syndrome when acquired),