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Multiple Sclerosis is a neurodegenerative disease which shows different phenotypes making difficult for clinicians to make short-term decisions related with treatment and prognosis. Diagnosis is usually retrospective. Learning Healthcare Systems (LHS) can support clinical practice as they are devised as constantly improving modules. LHS can identify insights which allow evidence-based clinical decisions and more accurate prognosis. We are developing a LHS with the aim of reducing uncertainty. We are using ReDCAP to collect patients’ data, both from Clinical Reported Outcomes (CRO) and from Patients Reported Outcomes (PRO). Once analyzed, this data will serve as a foundation to our LHS. We conducted bibliographical research to select those CRO and PRO collected in clinical practice or identified as possible risk factors. We designed a data collection and management protocol based on using ReDCAP. We are following a cohort of 300 patients for 18 months. At the moment, we have included 93 patients and received 64 complete responses and 1 partial response. This data will be used to develop a LHS, able to accurate prognosis as well as to automatically include new data and improve its algorithm. [ABSTRACT FROM AUTHOR]

Objective: To describe the profile of patients with multiple sclerosis (MS) treated with fingolimod in Spain and to assess the effectiveness and safety of fingolimod after 4 years of inclusion in the Spanish Gilenya Registry., Methods: An observational, retrospective/prospective, multicenter case registry, including all patients with relapsing-remitting MS (RRMS) starting treatment with fingolimod in 43 centers in Spain. Analyses were performed in the overall population and in subgroups according to prior disease-modifying therapy (DMT): glatiramer acetate/interferon beta-1 (BRACE), natalizumab, other treatment, or naïve., Results: Six hundred and sixty-six evaluable patients were included (91.1% previously treated with at least one DMT). The mean annualized relapse rate (ARR) prior to fingolimod was 1.12, and the mean EDSS at fingolimod initiation was 3.03. Fingolimod reduced the ARR by 71.4%, 75%, 75.5%, and 80.3%, after 1, 2, 3 and 4 years, respectively (p<0.001). This significant reduction in the ARR continued to be observed in all subgroups. After 4 years, the EDSS showed a minimal deterioration, with the EDSS scores from year 1 to year 4 remaining mostly stable. The percentage of patients without T1 Gd+ lesions progressively increased from 45.6% during the year prior to fingolimod initiation to 88.2% at year 4. The proportion of patients free from new/enlarged T2 lesions after 4 years of fingolimod treatment was 80.3%. This trend in both radiological measures was also observed in the subgroups. Adverse events (AEs) were experienced by up to 41.6% of patients (most commonly: lymphopenia [12.5%] and urinary tract infection [3.7%]). Most AEs were mild in severity, 3.6% of patients had serious AEs., Conclusions: The patient profile was similar to other observational studies. The results obtained from the long-term use of fingolimod showed that it was effective, regardless of prior DMT, and it had adequate safety results, with a positive benefit-risk balance., Competing Interests: Meca‐Lallana [Biogen, Genzyme, Merck Serono, Novartis, Roche and TEVA], C. Oreja‐Guevara [Biogen‐Idec, Novartis, Merck‐Serono, Almirall, Teva and Genzyme], D. Muñoz [Merck,Biogen, Teva, Novartis and Genzyme], J. Olascoaga [Biogen Idec, Bayer‐Schering, Genzyme, Merck‐Serono, Novartis and Teva], A. Pato [Novartis, Biogen and Genzyme Almirall], L. Ramió [Biogen Idec, Novartis, Bayer, Merck‐Serono, Genzyme, Teva PharmaceuticalIndustries Ltd.], V. Meca‐Lallana [Almirall, Biogen Idec,Genzyme, Merck Serono, Novartis, Roche, Teva and Terumo], M.A. Hernández [Merck Serono, Novartis, Biogen, Genzyme, TEVA and Almirall ], M.E. Marzo [nothing to disclose ], J.C. Álvarez‐Cermeño [Bayer Schering Pharma, Merk Serono, Biogen‐Idec, Teva Pharmaceuticals, Sanofi‐Aventis, Genzyme, and Novartis ], A. Rodríguez‐Antigüedad [Biogen‐Idec, Novartis, Merck‐Serono, Teva and Genzyme. ], X. Montalbán [Biogen‐Idec, Bayer‐ Schering, Merck‐Serono, Teva, Novartis, and Sanofi‐Genzyme, Bayer‐Schering, Genzyme, Merck‐Serono, Teva, Novartis, Actelion, Almirall, Allergan and Roche.] Ó. Fernández [Biogen‐Idec, Bayer‐ Schering, Genzyme, Merck‐Serono, Teva, Novartis, Actelion, Almirall, Allergan and Roche.] The rest of investigators do not declare any competing interests related to this article.

Background: To establish the early risk of multiple sclerosis (MS) following isolated demyelinating syndromes., Patients and Method: 36 patients with optic neuritis, 24 with brainstem syndromes, 27 with spinal cord syndromes and 8 patients with a poliregional syndrome were included in a prospective protocol: including clinical data, immunological determinations, oligoclonal bands, visual, somestesic and brainstem evoked potentials and a magnetic resonance imaging (MRI) study., Results: MRI showed the presence of signal abnormalities in 70% of the patients. The conversion rate (CR) was 18.5%. In patients with a normal MRI, CR was 4% compared to 26% in patients with an abnormal MRI (p = 0.05)., Conclusions: The early conversion rate to MS is significantly increased when MRI is abnormal.

Background: Interferon beta 1b (IFN beta-1b) has showed a reduction of exacerbation rate and a decrease in multiple sclerosis activity as evidenced by MRI. After the approval of IFN beta-1b in our country more than 1,000 patients are under treatment, however the experience is limited. The purpose of this study is to describe the clinical results and the tolerance of IFN beta-1b during the postmarketing period in our country., Material and Methods: We studied 95 patients treated with IFN beta-1b. An exhaustive follow-up has been performed in order to assess the tolerance and the efficacy of the drug. We registered the haematological and biochemical abnormalities, secondary effects, relapses, clinical evolution and drop-outs., Results: Mean age was 32.5 +/- 9 years, and the mean follow-up was 13.2 months. Seventy patients (74%) have been followed for more than one year. Haematological abnormalities are frequent, lymphopenia being the most common finding (37%). Flulike symptoms appear in 90% of the patients and skin reaction in the 70%. We have observed a drop-out rate of 7%. One patient developed depressive symptoms and the treatment was temporally discontinued. We have observed a significant decrease in relapses of the disease, however disability has not changed in the first year after treatment., Conclusions: IFN beta-1b has been well tolerated in the postmarketing period. The profile of secondary effects is similar, although not identical to that reported. The patient awareness of secondary effects and the realistic expectations of the drug are important in order to decrease the drop-out rate.

Introduction: Ocular myasthenia gravis is a localized form of myasthenia in which only the extra-ocular muscles are clinically affected, namely the levator palpebrae superioris and orbicularis oculi. Two years after onset of the ocular condition, it became generalized in 44-53% of the patients., Objective: 1. To describe the clinical features, diagnostic characteristics and clinical course of seven patients who fulfilled the criteria for diagnosis of ocular myasthenia and in whom the condition did not become generalized: 2. Review recent papers on this. Material and methods. We studied seven patients (two men and two women) diagnosed as having ocular myasthenia gravis, and followed them up for at least three years., Results: The average age was 56.5. The clinical findings were of ptosis of the eyelids and diplopia. All seven patients were treated with pyridostigmine. In six cases prednisone was also given and in one patient thymectomy was done. There was a satisfactory result in all cases., Conclusions: The basic treatment of ocular myasthenia is with anticholinesterases and corticosteroids. Occasionally other immunosuppressives may be required. Prednisone seems to reduce the number of patients who go on to develop the generalized form.

Introduction: Hypoglycemia can cause a diffuse brain malfunction and sometimes a focal neurological deficiency, that could lead to a mistaken diagnosis of cerebrovascular disease., Clinical Case: We describe the case of a 67 year old man, with diabetes mellitus type II treated with glibenclamide, that was referred to our hospital due to worsening of his chronic obstructive pulmonary disease. On the fifth day following admission he developed acute weakness in the right extremities and experienced difficulty in talking: six hours later he was conscious, with normal eye movements, but there was an absence of spontaneous facial motility and to pain; he showed complete cuadraplegia and bilateral Babinski. A determination of glycemia was made with the result of 24 mg/dl; after immediate treatment with glucose solution intravenously the patient recovered in a few minutes., Conclusion: This clinical observation reminds us of the importance of determining blood glucose in the assessment of any acute neurological dysfunction.

Introduction: Adrenoleukodystrophy is a hereditary recessive sex-linked disorder with very variable phenotype expression, including classical infantil ALD, adrenomyeloneuropathy (AMN) in adults and sex-linked Addison's disease. Clinical observations. Three affected patients are presented. The first showed signs of myeloneuropathy from the age of 38 and diagnosis was made by showing raised serum and fibroblasts levels of very long chain fatty acids (C26:0). In the second case symptoms started at the age of 13 and cerebral and peripheral nervous system changes developed progressively. This patient's brother was the third case, showing symptoms when he was 21 and developing cerebral, medullary and peripheral nervous system involvement. In the latter two cases, diagnosis was made by showing intracytoplasmatic trilaminary inclusions in the nervous system., Conclusions: It is important to recognize the different varieties of this disease in view of the possibilities of genetic counselling and of the therapeutic implications which are currently being evaluated.

Introduction: Arteriovascular malformations are anomalies of the embryonic development of cerebral vessels. They usually appear at between 10 and 30 years of age, being infrequent in infants., Objective: Describe our experience of angiomas in small children., Material and Method: The histories of all children under 10 years of age with the diagnosis of arteriovenous malformation admitted to our department between November 1984 and May 1995 were reviewed., Results: Nine patients, aged between 3 months and 10 years; seven cases presented as intracranial haemorrhage, one with epileptic crises and another with general clumsiness and a hemicerebellar syndrome. The diagnosis was confirmed by angiography and/or study by the pathologist in all cases. Six patients were treated surgically and the other three by embolization. Of the latter, two also received radiosurgical treatment. The complications seen were; two children died, one during the acute phase and the other four years later as direct consequence of the haemorrhage. Six patients had residual neurological defects. One child had no complications., Conclusions: Intra-parenchymatous haemorrhage is the commonest manifestation of angiomas in children under 10 years of age. The high risk and serious consequences of bleeding make it necessary for treatment to be as radical as possible.