Society Proceedings / Clinical Neurophysiology 120 (2009) e9–e88
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Conclusion: In MG patients, who are clinically not stable under standard immunosuppressive drug treatment, long-term IVIG therapy may result in tapering steroids and clinical improvement of myasthenic signs. The data give reason to conduct a controlled clinical trial.
121. Focal cortical dysplasia: Improvements of SHE scores and cognitve parameters in patients with epilepsy after surgery—A. Kühn, K. Wagner, B. Metternich, S. Fauser, R. Krämer, J. Zentner, A. Schulze-Bonhage (Universitätsklinikum Freiburg, Epileptologie, Freiburg, Germany)
doi:10.1016/j.clinph.2008.07.118
Background: In the first instance the aim of epilepsy surgery is the control of seizures, but the improvement of quality of life and cognitive functions is also of big importance. In order to quantitatively analyze post-surgical outcomes of quality of life, attention and memory functions in patients with epilepsy caused by focal cortical dysplasia (FCD), we compared pre- and post-surgical scores using standardized methods. The more precise analyses for special patient groups are, the better the surgery outcome prediction for individual cases may be. Methods: The sample of 17 patients with a FCD (pre-surgical age range: 15 and 57 years, M = 33.58, SD = 13.66) was recruited with an pre-post surgery interval between 12 and 35 months (M = 16.35, SD = 6.11). The mean age at epilepsy onset was 9.88 years (SD = 12.84) and mean disease duration was 23.71 years (SD = 15.45). In 12 patients the FCD was located right hemispherically (5 frontal, 7 temporal) and in 5 patients left hemispherically (1 frontal, 4 temporal). Histologically (classification according to Palmini) 13 patients had a FCD type 1 and 4 patients a FCD type 2. An additional hippocampal sclerosis was diagnosed in 7 patients (three left hemispherical). Eleven of the 17 patients (65%) were postoperatively completely seizure free (Engel class 1a).On the one hand we analyzed data of the self-rating questionnaire of subjective handicap of epilepsy (SHE) by Donoghue et al. (Brain, 1998) and on the other hand we compared objectively measured cognitive parameters such as selective attention (d2 Test of Attention), verbal and nonverbal declarative memory (German version of CVLT and DCS – a visual learning and memory Test for Neuropsychological Assessment). Statistical analyses were made using the nonparametric Wilcoxon test or Mann–Whitney-U-test. Tests with p < 0.05 were regarded as significant. Results: Patients post-surgically scored higher in domains like working and activity, social and personal life, feelings about oneself, physical scale and change during the last year. The only post-surgical score of SHE which did not show significant improvement was lifesatisfaction, even in the 11 post-surgical seizure free patients. Contrary to our expectations improvements in SHE scores of seizure free patients were not higher than improvements of the not seizure free patients. Neuropsychological assessment showed that selective attention as a basic cognitive function also improved after surgery. The patients’ post-surgical processing speed was significantly higher, while they were not more prone to mistakes than pre-surgically. Neither patients with left hemispherical FCD, nor patients with right hemispherical FCD improved in declarative memory functions; postsurgical memory results were unchanged in both the groups. Conclusion: Summarizing, nonparametric analyses showed, that a surgical treatment of FCD in patients with epilepsy leads to improvements in most domains of quality of life even though the life-satisfaction did not improve. Further analyses of socioeconomic factors may help to understand the missing life-satisfaction improvement. Further, the surgical treatment of FCD elicits an improvement of selective attention and did not contribute to deterioration in declarative memory.
120. Vergence deficits in cerebellar lesions—T. Sander 1, A. Sprenger 1, G. Neumann 1, S. Gottschalk 2, B. Machner 1, C. Helmchen 1, H. Rambold 3 (1 Universitätsklinikum Schleswig-Holstein, Neurologie, Lübeck, Germany, 2 Universitätsklinikum Schleswig-Holstein, Neuroradiologie, Lübeck, Germany, 3 The National Eye Institute, Bethesda, USA) The cerebellum is part of the cortico-ponto-cerebellar circuit for conjugate eye movements. Accordingly, cerebellar lesions cause smooth pursuit deficits and saccadic dysmetria. In contrast, the role of the cerebellum for disconjugate, i.e. vergence eye movements in humans are still unknown. The aim of this study was to investigate whether cerebellar lesions affect vergence eye movements. Similar to conjugate eye movements, vergence can be separated into two different components: vergence to step targets (fast vergence) and vergence to ramp and sinusoidal targets (slow vergence). Eye movements (fast and slow vergence, smooth pursuit, saccades) were binocularly recorded with the scleral search coil system in 20 patients with acute cerebellar lesions (ischemic strokes) and agematched healthy controls. Small dim laser stimuli were presented on an earth horizontal platform at the level of the subject’s nose. Smooth pursuit always had a constant vergence angle (isovergence). The majority of patients had unilateral lesions in the territory of the posterior inferior cerebellar artery (3 patients with infarctions in the superior cerebellar artery territory, one cavernoma patient). Group analysis revealed impairment of both conjugate and disconjugate eye movements as well as reduced saccadic gain (Table 1). Table 1 Smooth pursuit (gain) All patients Vermal lesion Non-vermal lesion
+ +
Fast vergence (acceleration)
Slow vergence (gain)
Saccades (gain)
+ +
+ + +
The gain of slow vergence to sinusoidal targets was reduced in the patients. Patients with vermal lesions (subgroup, n = 10) had a significant lower gain compared to controls while the other patients did not differ. In contrast, fast vergence to step targets remained unimpaired (acceleration). Smooth pursuit to step-ramp targets was impaired, with increased latency, decreased initial acceleration and reduced gain. Smooth pursuit eye movement gain to sinusoidal targets was significantly reduced. Similar to vergence eye movements to sinusoidal targets, patients with vermal lesions had a lower smooth pursuit gain while the other patients did not differ from controls. We propose that (1) the cerebellum is involved in the processing of vergence eye movements, (2) the cerebellar vermis plays an important role in executing vergence eye movements and (3) neural control of fast and slow vergence appears to be separated in the cerebellum. doi:10.1016/j.clinph.2008.07.119
doi:10.1016/j.clinph.2008.07.120
122. Cerebral and extracranial neurodegeneration are strongly coupled in Parkinson’s disease—J. Spiegel 1, D. Hellwig 2, W.H. Jost 3, G. Farmakis 2, S. Samnick 2, K. Fassbender 1, C.M. Kirsch 2,