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41.
Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures. 总被引:1,自引:0,他引:1
Martin Südmeyer Andreas Saleh Lars Wojtecki Mathias Cohnen Joachim Gross Markus Ploner Harald Hefter Lars Timmermann Alfons Schnitzler 《Movement disorders》2006,21(12):2134-2139
Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra. 相似文献
42.
Prof. Dr. Bernhard Schmitt Prof. Dr. Michael Albani Priv.-Doz. Dr. Thomas Bast Prof. Dr. Ulrich Brandl Prof. Dr. Rudolf Korinthenberg Prof. Dr. Gerhard Kurlemann Prof. Dr. Bernd Neubauer Prof. Dr. Ulrich Stephani Dr. Markus Wolff 《Zeitschrift für Epileptologie》2007,20(3):113-119
Zusammenfassung
Der richtige Zeitpunkt für das Absetzen der Antiepileptika (AE) im Kindesalter ist unbekannt. Anl?sslich ihrer Jahrestagung
haben die Mitglieder des K?nigsteiner Arbeitskreises (KA) eigene und publizierte Absetzstrategien diskutiert. Da Studien zu
diesem Thema rar und widersprüchlich sind, wurde beschlossen, die Diskussionsergebnisse im Sinne einer Meinungs?u?erung zu
publizieren.
Bei Neugeborenen besteht übereinstimmung, AE innerhalb von 2 bis 12 Wochen nach dem letzten Anfall abzusetzen. Bei BNS-Epilepsie
wird Vigabatrin nach 6 bis 12 und Sultiam nach 6 bis 36 Monaten abgesetzt. Nach erfolgreicher Steroidtherapie setzt die Mehrheit
des KA die AE-Therapie für zwei Jahre fort. Für die Rolando-Epilepsie sind 1 bis 3 Jahre Anfallsfreiheit ausreichend, auch
wenn fokale Spike-Waves persistieren. Im Falle einer symptomatisch fokalen Epilepsie ist die Grunderkrankung mitentscheidend
für das Absetzen. Die Behandlung der Absencen-Epilepsie kann nach zwei Jahren beendet werden, w?hrend bei myoklonisch- astatischer
Epilepsie meist eine 2- bis 5-j?hrige Anfallsfreiheit vorausgesetzt wird. Konsens besteht darüber, dass die Juvenile- Myoklonus-Epilepsie
ein sehr hohes Rückfallrisiko birgt. Dennoch ziehen einzelne neurop?diatrische Mitglieder einen Absetzversuch nach 2- bis
3-j?hriger Anfallsfreiheit in Betracht. Die überwiegende Mehrheit des KA führt aber bei gesicherter Diagnose keinen Absetzversuch
durch. Bezüglich der Absetzgeschwindigkeit wird ein langsames (3 bis 12 Monate) Ausschleichen favorisiert. Nur zwei Mitglieder
praktizieren ein rascheres Absetzen (<3 Monaten). Das EEG spielt für die Entscheidung eine untergeordnete Rolle und bleibt
auf bestimmte Epilepsieformen (z. B. Absencen-Epilepsie) beschr?nkt.
Das vorliegende Papier gibt die Meinung des KA wieder und eignet sich nicht im Sinne einer Leitlinie. Für die Entscheidung
AE abzusetzen, ist immer eine individuelle Abw?gung von Grunderkrankung, Epilepsieform und psychosozialen Umst?nde erforderlich.
相似文献
43.
We evaluated the effect of different manipulations on the performance of a standardized counting task in 7 patients with idiopathic jaw-opening dystonia. Patients used a small stick as sensory stimulus. Following conditions were examined: stick placed between teeth and cheek (CHEEK), biting on stick (TEETH), voluntary jaw occlusion without stick (OCCLUSION). Articulation was rated by patients and experimenters and surface electromyographic activity (EMG) was recorded. Patient-rating (CHEEK - 36.6%, TEETH - 48.1%) and EMG (-18.1%; -17.3%) were significantly improved for conditions using the stick, whereas experimenter-rating showed a trend for TEETH (-16.2%). Although jaw occlusion during speaking deteriorates articulation in healthy subjects, there was no further deterioration in patients and EMG was even significantly reduced (-18.6%). Comparable results were obtained in 1 patient using a special dental device. We conclude that sensory tricks significantly improve subjective and objective parameters. Besides tactile stimulation, altered proprioceptive feedback and antagonist activation may modulate hyperactive dystonic networks. 相似文献
44.
Markus Thalmann Heinrich Schima Georg Wieselthaler Ernst Wolner 《The Journal of heart and lung transplantation》2005,24(3):237-245
The beating heart and the resultant pulse wave have been a symbol of life for centuries. The development history of roller pumps for cardiopulmonary bypass shows that the human body tolerates non-pulsatile blood flow, at least for short-term support. Over the last few years, many types of rotary blood pumps have been developed for clinical use in patients requiring mid- to long-term support. Although early clinical experiences in patients with long-term support have been promising, the matter of whether pulsatile flow is needed or not remains controversial. Therefore, this review summarizes the observed clinical consequences of continuous blood flow in patients supported by rotary blood pumps and relates these consequences to underlying experimental studies. 相似文献
45.
Erkki Lotspeich Markus Schoene Heinz Gerngroß Roland Schmidt Reinhard Steinmann Marco Ramadani Susanne Gansauge 《Langenbeck's archives of surgery / Deutsche Gesellschaft fur Chirurgie》2007,392(5):559-566
Introduction Postoperative treatment for colorectal cancer depends on tumor stage as defined by the International Union Against Cancer
(UICC). Adjuvant chemotherapy is not recommended in patients without lymph node involvement (UICC stages I and II). As many
as 20–30% of these patients, however, will develop recurrence.
Aims and objectives We conducted this study to determine the presence of disseminated tumor cells in the lymph nodes by quantitative real-time
polymerase chain reaction (QRT-PCR) for cytokeratin 20 (CK20) in an attempt to provide supplementary information compared
to histopathological findings.
Materials and methods Using a standard QRT-PCR assay, we examined primary tumors and 391 lymph nodes from 31 patients with completely resected colorectal
cancer.
Results Of the 31 primary tumors, 29 were positive for CK20 by QRT-PCR.
Discussion An examination of the lymph nodes from the 29 patients with CK20-positive primary tumors revealed that 35 (92.1% sensitivity)
of the 38 histopathologically positive lymph nodes and 54 (16.7%) of the 324 histopathologically negative lymph nodes were
positive by molecular analysis. CK20 expression was detected in 10 (100%) of 10 patients with a histopathologically positive
lymph node status (pN1). In 9 (47.4%) of 19 patients with negative histopathological results (pN0), we detected a CK20 mRNA
signal in at least one lymph node. Whereas eight patients with histopathologically negative lymph nodes could be upstaged
on the basis of the molecular findings, no patient would be downstaged.
Conclusion Our results suggest that QRT-PCR for CK20 is a useful tool for the quantitative detection of micrometastases in the regional
lymph nodes. We introduce a standardized procedure that integrates a molecular diagnostic technique in the clinical staging. 相似文献
46.
Evaluation of myocardial function in patients with end-stage heart failure during support with the Jarvik 2000 left ventricular assist device. 总被引:2,自引:0,他引:2
Markus Ferrari Kamuran A Kadipasaoglu Mihai Croitoru Jeff Conger Tim Myers Igor Gregoric Branislav Radovancevic George V Letsou O H Frazier 《The Journal of heart and lung transplantation》2005,24(2):226-228
In 2 patients with the Jarvik 2000 left ventricular assist device (LVAD), we assessed left ventricular systolic function through pressure-volume loops and E(max) at the beginning and end of the support period to potentially predict the possibility of pump removal without transplantation. Immediately before LVAD implantation and explantation, pressure and volume measurements were made with catheters and echocardiography, respectively, the E(max) being calculated from the slope of the pressure-volume loops, and the left ventricular ejection fraction (LVEF) being estimated by echocardiography. Transplantation was performed after 14 and 62 days, respectively, during which the LVEF increased by 75% (from 12% to 21%) in Patient 1 and remained unchanged (from 16% to 18%) in Patient 2, whereas the E(max) increased from 0.63 and 0.42 mm Hg/ml, respectively, to 1.31 and 1.07 mm Hg/ml, reflecting a 107% and 155% improvement. In these 2 cases, the E(max) was a more reliable indicator of intrinsic myocardial contractility than was the LVEF. 相似文献
47.
48.
49.
Expression and distribution of vascular endothelial growth factor protein in human brain tumors 总被引:9,自引:0,他引:9
T. Pietsch Markus M. Valter Helmut K. Wolf A. von Deimling H.-J. Su Huang Webster K. Cavenee Otmar D. Wiestler 《Acta neuropathologica》1997,93(2):109-117
Marked neovascularization is a hallmark of many neoplasms in the nervous system. Recent reports indicate that the endothelial
mitogen vascular endothelial growth factor (VEGF) may play a critical role in the regulation of vascular endothelial proliferation
in malignant gliomas. Using novel monoclonal antibodies to the VEGF polypeptide we have determined the expression and cellular
distribution of VEGF protein in a representative series of 171 human central nervous system (CNS) tumors by immunohistochemistry
and immunoblotting. In agreement with previous in situ hybridization data, 19 out of 20 glioblastomas (95%) showed immunoreactivity
for VEGF, whereas both the percentage of immunoreactive tumors and the extent of immunoreactivity for VEGF were significantly
lower in astrocytomas. Of the pilocytic astrocytomas (WHO grade I) 44% were immunoreactive for VEGF, but we observed several
cases with pronounced vascular proliferates in the absence of VEGF. In ependymomas, meningiomas, hemangioblastomas, and primitive
neuroectodermal tumors, there was no correlation between VEGF expression, vascular endothelial proliferation and the grade
of malignancy. Oligodendrogliomas and the oligodendroglial component of mixed gliomas lacked immunoreactive VEGF, indicating
that endothelial growth factors other than VEGF may regulate tumor angiogenesis in these neoplasms. Western blot analysis
showed a predominant VEGF protein species of 23 kDa and confirmed the immunohistochemical data in all cases. Our findings
demonstrate that VEGF is expressed in a wide spectrum of brain tumors in which it may induce neovascularization. However,
other angiogenic factors also appear to contribute to the vascularization of CNS neoplasms.
Received: 18 April 1996 / Revised, accepted: 20 August 1996 相似文献
50.