Rapidly progressive moyamoya syndrome associated with bartonella henselae infection and factor V leiden mutation— A. Hahn 1 , R. Siekmann 2 , B. Neubauer 1 ( 1 Abteilung fu ¨r Neuropa ¨diatrie, Universita ¨tsklinik Gießen, 2 Abteilung fu ¨r Neuroradiologie, Universita ¨tsklinik Gießen) Introduction: Moyamoya syndrome (MMS) is characterized by progressive stenosis of the distal internal carotid artery (ICA) and proximal cerebral arteries accompanied by an extensive collateral vascular network at the base of the brain. Case: We report on a now 24-month-old Caucasian girl who developed right-sided hemiparesis at age 19 months. An MRI revealed an infarction in the territory of the left median cerebral artery (MCA) also involving the border zones of the anterior and posterior cerebral artery. Evaluation for prothrombotic dis- orders disclosed a heterozygous factor V Leiden mutation (FVLM). Ig-M- and Ig-G-antibodies against Bartonella henselae (Bh) were positive in blood. Nine weeks later, she experienced a transient paresis of her left arm and face. Four weeks later, the girl was referred to our department because of decreased vigi- lance, recurrent vomiting, and visual disturbances. A new MRI displayed an infarction in the right hemisphere. MR-angiography suggested an almost complete occlusion of both ICA, stenotic narrowing of the proximal parts of all their branches, and an extensive collateral network of small vessels. Now, Ig-G-antibod- ies against Bh only were positive in blood. The girl underwent bilateral encepalo-duro-arterio-myosynangiosis and right-sided anastomosis of the superficial temporal artery and MCA at age 23 months. Since then, the girl has experienced a further infarc- tion of the right hemisphere with left-sided hemiparesis that has almost completely resolved. Conclusion: Cat scratch disease as a cause of cerebral arteritis has been described in one patient, and Bh-infection presenting as systemic leukencephaloclastic vasculitis has also been reported. Moreover, a high rate of antibodies against Bh has been found in a cohort of patients with Henoch-Scho ¨ nlein purpura, indicating a significant association of antecedent Bh-infection and vasculitis. It has been suggested that FVLM contributes significantly to stroke occurrence in childhood, and a prospective study revealed prothrombotic anomalies in four of 10 children with MMS. Because of these data, it seems tempting to speculate that FVLM and concomitant Bh-infection contributed to the rapidly progres- sive course in this peculiar patient. doi:10.1016/j.clinph.2006.11.099 Correlation between metabolic alterations in 1 H-MR spec- troscopy and epileptic activity in patients with temporal lobe epilepsy—T. Hammen 1 , M. Do ¨lken 2 , M. Schwarz 1 , F. Kerling 1 , T. Engelhorn 2 , A. Stadlbauer 4 , O. Ganslandt 3 , C. Nimsky 3 , M. Buchfelder 3 , A. Doerfler 4 , H. Stefan 1 ( 1 Epilepsiezentrum Erlangen, Klinik und Poliklinik fu ¨r Neurologie Universita ¨tsklinik Erlangen, 2 Klinik fu ¨r Neuro- radiologie Universita ¨tsklinik Erlangen, 3 Klinik fu ¨r Neuro- chirurgie Universita ¨tsklinik Erlangen, 4 Landesklinikum St. Po ¨lten) Introduction: In the following study, the degree of metabolite alterations in 1 H-MR spectroscopy ( 1 H-MRS) were correlated to findings of intensive video-EEG monitoring and duration of clinical features in patients with temporal lobe epilepsy (TLE). Patients and methods: Fourteen TLE patients investigated by 1 H-MRS were analysed by: number of clinical seizures, duration of clinical symptoms, frequency of interictal epileptiform dis- charges (IEDs) and ictal activity. Single voxel spectroscopy was carried out in temporomesial structures (Abb. 1). Spectral post processing was carried out by LCModel. Pearson correlation coefficient (PCC) was calculated between alterations of tNAA, Creatin, Cholin, my-Ins and NAA/(Cr + Cho) ratio and frequen- cy of IEDs, ictal activity, number of clinical seizures, duration of clinical symptoms, ictal activity and IEDs. Statistical significance was accepted at p < 0.05. Results: A significant negative correlation was found between tNAA values and degree of IEDs in 24 h intensive video-EEG monitoring (p = 0.048) (Abb. 2). Duration of clinical features indicating temporomesial seizure activity registered by video mon- Fig. 1. e40 Society Proceedings / Clinical Neurophysiology 118 (2007) e9–e116