Atypical neurological presentation of GSS: Case report

WCN 2013 No: 2196 Topic: 6 — MS & Demyelinating Diseases Atypical neurological presentation of GSS: Case report O.H. Daoud, O. Hdiji, I. Bouchhima, E. Turki, A. Boukhris, M. Damak, M.I. Miladi, I. Feki, C. Mhiri. Habib Bourguiba Hospital, Sfax's Medecine University, Sfax, Tunisia Introduction: Gougerot-Sjogren Syndrome (GSS) is an auto-immune exocrinopathy characterized by xerophthalmia and xerostomia. Neurological complications occur between 8.5 and 70%. Data concerning central nervous system (CNS) symptoms have been rarely described. We analyze the clinical, radiological and therapeutic aspects of a patient with CNS signs revealing primitive GSS and we discuss some particularities. Case report: A 52-year-old woman was hospitalized for recurrent clinical focal signs of CNS involvement. It consisted on sudden left hemiparesis on 2009 and two sudden regressed episodes of aphasia on 2011 and 2013. Brain MRI revealed micro nodular lesions appearing as hyper-signal in T2 on periventricular white matter and frontal cortex. Both lacrymal and salivary gland secretions were affected. A high level of antinuclear antibodies to SSA was associated with inflammatory lesions in minor salivary glands biopsy. The diagnosis of GSS was established. An oral corticosteroid therapy (1 mg/kg/day) was prescribed during 6 weeks followed by progressive degression, with good outcome. Discussion: CNS damages are less frequently associated with GSS's systemic damages, and are often inaugural. The pathophysiology remains unknown. Some authors suggest the pathologic role of the anti-SSA based on their higher frequency in patients with CNS manifestation. Neurological focal manifestations are the most observed, but deficit signs are polymorphic. Aphasia is rarely described. The onset may be sudden as stroke. The MRI can reveal T2 hyperintensities on periventricular white matter or juxtacortical, more rarely on the cerebral cortex. Treatment of GSS's neurological manifestations is usually based an oral corticosteroid with high dose. doi:10.1016/j.jns.2013.07.1398 Abstract — WCN 2013 No: 2001 Topic: 6 — MS & Demyelinating Diseases Onset of secondary progression and long-term prognosis in multiple sclerosis A. Scalfari, A. Neuhaus, M. Daumer, P. Muraro, G. Ebers. Centre of Neuroscience, Department of Medicine, Imperial College, London, UK; Sylvia Lawry Centre, Munich, Germany; Oxford University, Oxford, UK Background: Prognosis in multiple sclerosis (MS) remains largely unpredictable. Objectives and methods: Among 806 relapsing-remitting (RR) patients from the London Ontario database, we assessed: 1) the effect of baseline features on the risk of secondary progression (SP); 2) the risk of becoming disabled (DSS 6–8) according to latency to SP. Results: Longer latency to SP predicted lower risk of reaching DSS 6 (OR= 0.76 for 5 years and OR= 0.44 for 15 years of latency); same results were observed in patients matched for early (year-1 + year-2) relapses number. However, RR phase duration did not influence SP evolution. The latency to SP conversely related to the number of total relapses (1–2 = 8.8 years; 3–4 = 11.3 years; ≥5 = 12.2 years). Predictors of the risk of SP were male sex (HR= 1.41), older age at onset (age N 30years vs ≤20years, 21–30years HR= 0.52, 0.65, respectively) and high early relapse frequency (≥3 vs 1, 2 attacks HR= 0.63, 0.75, respectively). Disease duration affected the probability of becoming SP (OR= 1.07), increasing by 9% every 5 years. At baseline, OR for risk of SP yielded by 3 relapses were 3.6, 5.8, 9.5 in patients aged 20, 30 or 40 at onset respectively, and increased by 2-fold at 10 years (OR 7.5, 12.1, 19.8, respectively) and by 5-fold at 20 years (OR 15.6, 25.5, 41.4, respectively). Conclusions: Prevention/delay of SP represents a potential therapeutic target. Early relapse number and age at onset can be used for selecting groups at higher risk of developing severe disability and requiring more aggressive treatments. doi:10.1016/j.jns.2013.07.1399 Abstract — WCN 2013 No: 2105 Topic: 6 — MS & Demyelinating Diseases Pediatric and adult multiple sclerosis — Comparison of magnetic resonance imaging characteristics at disease onset WCN 2013 No: 2105 Topic: 6 — MS & Demyelinating Diseases Pediatric and adult multiple sclerosis — Comparison of magnetic resonance imaging characteristics at disease onset R.-I. Milos, M. Szimacsek, M. Pritsch, J. Penzien, M. Karenfort, A. Blaschek, R. Seidl, K. Rostasy, A. Bayas, K. Storm van's Gravesande, M. Weber, D. Prayer, B. Kornek. Department of Radiology, Medical University Vienna, Vienna, Austria; DRK-Children’s Hospital Siegen, Siegen, Germany; Children’s Hospital Klinikum Augsburg, Augsburg, Germany; Department of General Pediatrics, Pediatric Neurology, Heinrich Heine University, Dusseldorf, Germany; Department of Pediatric Neurology and Developmental Medicine, Dr. von Hauner's Children's Hospital, Ludwig-Maximilians University, Munich, Germany; Department of Pediatrics, Medical University of Vienna, Vienna, Austria; Department of Pediatrics IV, Division of Pediatric Neurology, InnsbruckMedical University, Innsbruck, Austria; Department of Neurology, Klinikum Augsburg, Augsburg, Germany; Gottfried von Preyer Children Hospital, Medical University of Vienna, Vienna, Austria; Department of Neurology, Medical University of Vienna, Vienna, Austria Background: Only few studies have compared MRI findings at disease onset between patients with pediatric(POMS) and adultonset multiple sclerosis (AOMS). Abstracts / Journal of the Neurological Sciences 333 (2013) e358–e421 e385