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Paraneoplastic neurological syndromes (PNS) are mostly immune-mediated, tumor-associated disorders that can develop years before detection of any malignancy. Their prevalence varies among different tumor types. Diagnostic criteria for PNS include the presence of a classical or nonclassical clinical syndrome in association with antineuronal antibodies and the presence of any malignancy [1]. We report the case of a 32-year-old male, who presented with a 2-year history of progressing neurological deficits starting with headaches, muscle pain and weight loss. Nine months later, the patient had developed two generalized epileptic seizures, optic and acoustic hallucinations, oculomotor disturbances, gait ataxia and signs of polyneuropathy. Over time, investigations in external hospitals had led to the assumption of an autoimmune encephalitis according to the following findings: a T2-sequence hyperintense alteration of the right hippocampus in the magnetic resonance imaging (MRI), positive oligoclonal bands but normal cell count in the cerebrospinal fluid, and positive serum tests for Anti-Huand N-Methyl-D-aspartic acid-receptor (NMDA-R) antibodies. Serum antibodies against acetycholine receptors and repeated tumor search remained negative. Treatments with prednisolone, intravenous immunglobuline and plasmapheresis were not effective. On clinical examination we saw a 32-year-old, cachectic and wheelchair bound patient with complex oculomotor disturbances including an anisocoric, converged right pupil with disturbed abduction and a dysfunction of horizontal movements of both eyes. There were signs of dysphagia and dysphonia. Severe generalized muscle atrophy and muscle pain were accompanied by pareses focused on the muscles of the neck causing a dropped head, but pareses were also found in the muscles of the proximal upper limb region and the distal lower limbs. Severe gait and limb ataxia and signs of polyneuropathy predominantly of the lower limbs including pallhypaesthesia and arreflexia made it impossible for the patient to stand or walk. Considering the fast progression of symptoms several diagnostics were repeated: cerebral MRI (1.5 T) showed an unchanged T2-hyperintense, non-contrast enhancing signal alteration of the right hippocampal area (Fig. 1). The patient’s serum was again tested positive (Indirect immunofluorescence test [2], Euroimmun Medical Laboratory Diagnostics, Luebeck, Germany) for anti-Hu-antibodies (titre 1:1,000, reference \1:10) and NMDA-R antibodies (titre 1:320, reference\1:10). The patient denied repetition of lumbar puncture at this point. Electroneurography demonstrated a mostly demyelinating sensorimotor I. Pohley M. Wittstock R. Benecke A. Wolters Department of Neurology, University of Rostock, Rostock, Germany

We conducted an open-labeled clinical trial of interferon beta-1b (IFNB) treatment in 20 patients with primary progressive multiple sclerosis (PPMS) and longitudinally monitored autoantibodies against double-stranded DNA (dsDNA), thyroid peroxidase (TPO),myelin basic protein (MBP), myelin oligodendrocyte glycoprotein (MOG), synapsin and S-100B. Before treatment, one patient had elevated TPO antibodies, four patients had elevated antibodies against S-100B, two patients against MOG or synapsin and one patient against MBP. In two patients we observed a continuous increase of dsDNA or TPO antibodies above the normal range. This rise paralleled IFNB treatment. In addition, 11 of 20 patients developed neutralizing antibodies against IFNB. There was no increase of autoantibodies directed against central nervous system antigens. Like patients with relapsing remitting or secondary progressive multiple sclerosis, PPMS patients may be at risk of an autoimmune response during IFNB treatment.

We performed a cross-sectional, “bottom-up” observational study of resource use, costs, and quality of life in patients with multiple sclerosis (MS) in Germany. Six centers participated in the study. Patients were asked to complete a questionnaire, and a total of 737 patients returned it (response rate 66%). The questionnaire provided information on all resource consumption, medical, and nonmedical, work absence, informal care related to their MS, and quality of life (EuroQol). Simultaneously, medical charts were also abstracted for a subsample of 202 patients for comparison between answers in the questionnaires and registered data. Levels of disability were assessed using the Expanded Disability Status Scale. The mean age of the cohort was 41.9±14.1 years (disease onset 33.4), mean EDSS score 4.4 (range 1.0–9.5), and mean utility measured by EQ-5D 0.552±0.331). Mean total cost per patient and year was 65,400 DM, adjusted for use of interferons, which was higher in this sample than the current average use in Germany. When this cost is extrapolated to an estimated patient population of 120,000, total costs to society are estimated at 7.85 billion DM. Direct costs represented 57.5%, informal care accounted for 12.1% and indirect costs amounted to 42.5%. Public payers pay for an estimated 24,800 DM per patient or 38% of total costs. All types of costs (direct, informal care, indirect) increased with increasing disability, while utilities decreased.