Long-term Course Of Disease Research Articles

Prevalence, Characteristics, and Long-Term Prognosis of Epilepsy Associated with Pediatric Brain Tumors.

We investigated the prevalence, onset, characteristics, and long-term course of epilepsy disease in children who underwent surgical intervention for diagnosed brain tumors. We reviewed the medical records of children with diagnosed brain tumors who underwent surgery during 2004-2014 at the Hadassah Medical Center. All patients with epilepsy were invited to a clinical visit that included a neurologic examination. The primary outcome measures were neurologic status according to the Glasgow outcome score (GOS) and postoperative seizure outcome according to the Engel system. We compared clinical characteristics according to the timing of epilepsy onset. The mean follow-up was 49 months. Of 128 patients included in the study, 44 (34%) had seizures; 23 (18%) developed epilepsy after surgery. Of the 30 patients with epilepsy who survived, 21 (70%) are in Engel class I and 13% Engel are in class II. Forty-five percent of the children are classified as GOS 5. Children who developed epilepsy after surgery were more likely to be in GOS 1-2 than were those who had seizures before surgery (P=0.0173). Children with seizures were more likely to have cortical tumors and less likely to have tumors of the posterior fossa (P < 0.001). Children who underwent gross total resection were less likely to have epilepsy (P < 0.001). We show a high incidence of epilepsy in the late course of pediatric brain tumor disease. In the long term, seizure outcome was excellent. However, postsurgical onset of epilepsy was associated with a less favorable neurologic outcome.

Survival and clinical events related to end-stage liver disease associated with HCV prior to the era of all oral direct-acting antiviral treatments

ABSTRACTBackground: The aim of this study was to describe the natural long-term course of end-stage liver disease associated with chronic hepatitis C (HCV) infection by measuring survival and complication rates in the era prior to the arrival of new direct-acting antiviral (DAA) drugs.Methods: A retrospective population-based cohort study was designed to establish the follow-up of patients hospitalized for a decompensated cirrhotic event or hepatocellular carcinoma using electronic records from hospital discharge databases from 2009 to 2015. Their survival was compared with a sex, age and non-liver mortality excess matched simulation of the general Spanish population.Results: A total of 253 patients were included in the study. Among those with decompensated cirrhosis (n = 151) the hospital admission rate was 1.88 per patient-year with a mortality rate of 0.16 per patient-year. Mean survival was 4.10 years for patients with decompensated cirrhosis, and 1.75 for non-transplanted hepatocellular carcinoma, compared to 18.39 years for the general population.Conclusion: Our results show the complexity and rapid progression of end-stage liver disease associated with HCV infection. The considerable loss of life expectancy associated with the development of decompensated cirrhosis in patients with chronic HCV infection in the absence of viral clearance through treatment is acutely evident.

Schizophrenia or Atypical Lupus Erythematosus with Predominant Psychiatric Manifestations over 25 Years: Case Analysis and Review

We observed a case over 25 years of relapsing–remitting schizophrenic spectrum disorder, varying regarding the main symptomatology between more depressive or more schizoaffective or rather typical schizophrenic syndrome. Diseased phases were repeatedly accompanied by minor skin lesions, which were initially classified as mixed tissue disorder. Psychotic phases were waxing–waning over years. During one later relapse, skin involvement was severe, classified to likely represent an allergic reaction to psychopharmaca; this generalized exanthema remitted rapidly with cortisone treatment and azathioprine. Under continued azathioprine and low dose neuroleptics, the patient remitted completely, appearing psychiatrically healthy for 16 years. When azathioprine was set off due to pregnancy, an extraordinary severe relapse of schizophrenia like psychosis accompanied by most severe skin lesions developed within a few weeks, then requiring 2 years of psychiatric inpatient treatment. Finally, a diagnosis of systemic lupus erythematodes plus neuropsychiatric lupus was made. A single CSF sample in 2013 showed suspicious biomarkers, matching with CSF cytokine profiling in schizophrenic and affective spectrum disorder patients and indicated mild neuroinflammation. Complex immune suppressive treatment was reinitiated short after relapse, but was only partially successful. However, surprisingly the psychosis and skin lesions remitted (in parallel) when belimumab was given (add-on). The very details of this complicated, long-term disease course are discussed also with regard to general ideas, in particular with respect to the question if this case of seemingly comorbid schizophrenia with minor autoimmunity signs represented a case of one emerging autoimmune disorder with variant manifestations systemically and within the CNS, though atypically with predominant appearance as a schizophrenia spectrum disorder.

Typology of urination disorders in patients with idiopathic overactive bladder

The aim of the study was to determine the frequency and clinical significance of individual dysuric symptoms found according to the result of the monitoring of the clinical course of idiopathic overactive bladder (IOAB). The examination and treatment of 362 patients (41 men and 321 women) with IOAB have been carried out. It has been proved that the clinical course of IOAB may be manifested by different combinations of symptoms and disorders of accumulative and voiding function of the bladder with symptoms of pollakiuria (88,8%); nocturnal pollakiuria (83,2%); urgency (39%); incontinence (56%); difficulty urinating (30%). The patients with IOAB revealed a significant correlation with the «satellite» urinary symptoms, which are dominated by: a feeling of incomplete emptying of the bladder 64,9%, discomfort in the urethra 58,8% cystalgia 44,7%, 30,9% nocturnal polyuria. The presence of heavy dysuric symptoms, long-term course of the disease and emotional exhaustion can provoke behavioral symptoms that occur in 30,1% of the patients with restricted fluid intake, 26.5% of the patients using feminine hygiene products (pads, diapers), 20,1% of the patients in the control micturition «on track» in 12,7% of the patients with drawing maps of lavatories, in 14.3% – «protective» urination.

The natural history of Crohn's disease in children: a review of population-based studies.

The incidence of Crohn's disease (CD) has been reported to increase. The aim of this review is to perform a comprehensive literature search of population-based studies focused on the natural history of paediatric-onset CD. A literature search of English and non-English language publications listed in the electronic database of MEDLINE (source PUBMED) and EMBASE from 1935 to 2016 was performed. Population-based studies or national cohorts reporting data on the short-term or long-term disease course of paediatric CD were included. Forty-nine paediatric and 15 nonpaediatric studies on CD have been identified. Up to one-third of children with inflammatory behaviour developed bowel complications more than 5 years after diagnosis. From 48 to 88% of children have experienced at least one corticosteroid course irrespective of the period of diagnosis and up to one-third became steroid dependent. Immunosuppressive preparations were used earlier and more frequently in newer than older cohorts (68 vs. 32% at 5 years) and more than one-third of children have received biological treatment early in the disease course. A decline in the surgery rate might be observed in more recent compared with older unselected populations. The relative risk of cancer in childhood-onset CD as well as the risk of death seem to have increased. Childhood-onset CD seems to be an aggressive phenotype of the disease. Compared with older cohorts, a trend towards decreasing surgical rate can be observed in newer cohorts paralleled by an increase in immunomodulator use and biologicals. Nevertheless, the causative role has yet to be investigated.

Timing of Future Remyelination Therapies and Their Potential to Stop Multiple Sclerosis Progression.

Prior to the onset of demyelination in multiple sclerosis (MS), early oligodendrocyte injury, axonal degeneration and astroglial scarring occur. The irreversible progressive phase of MS begins when the axonal loss threshold is reached. Progressive disease onset has the highest impact on a poor prognosis in MS. Conversion to progressive disease is essentially an age-dependent process independent of disease duration and initial disease course. Although prevention of relapses has been the primary approach in the disease management, incomplete recovery from even the first relapse correlates with the long-term neurodegenerative phenotype of progressive MS onset. Therefore, the provider should review each patient's potential for relapse-related disability and start DMDs with the goal of preventing relapses. Existing immunomodulatory medications used to prevent MS relapses do not prevent long-term disability, which requires agents focused on remyelination and axonal repair. If applied immediately after a relapse rather than during the progressive phase of MS, remyelination-stimulating strategies may result in full recovery and prevention of long-term neurodegeneration and progressive disease course.

Commentary: Tumefactive Demyelinating Lesions as a First Clinical Event: Clinical, Imaging, and Follow-up Observations.

We read with great interest the paper recently published by Jeong and colleagues exploring the long-term evolution and disease course of patients initially presenting with tumefactive demyelinating lesions (TDLs) and describing their clinical and radiographic characteristics. They found that most of TDLs evolve into multiple sclerosis (MS) or neuromyelitis optica spectrum disorders (NMOSD), albeit a minority of patients remains without a definite diagnosis, even after a careful and extensive diagnostic workup (1). The authors underlined that the current understanding of the etiology of TDLs should be revaluated upon appraisal of NMOSD because extensive brain lesions have been frequently reported as a first manifestation in NMOSD, especially in Asian populations (1).

Web-based medical and health information in multiple sclerosis: for patients and physicians.

Neurodegenerative Disease ManagementVol. 6, No. 6s Symposium PaperWeb-based medical and health information in multiple sclerosis: for patients and physiciansAlessandra SolariAlessandra Solari*Author for correspondence: E-mail Address: Alessandra.Solari@istituto-besta.it Unit of Neuroepidemiology, Foundation IRCCS Neurological Institute C. Besta, Milan, ItalySearch for more papers by this authorPublished Online:22 Nov 2016https://doi.org/10.2217/nmt-2016-0050AboutSectionsView ArticleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinkedInReddit View articleKeywords: decision aidsmultiple sclerosispatientsself-managementshared decision makingweb resourcesReferences1 Lejbkowicz I, Paperna T, Stein N, Dishon S, Miller A. Internet usage by patients with multiple sclerosis: implications to participatory medicine and personalized healthcare. Mult. Scler. Int. 2010, 640749 (2010).Medline, Google Scholar2 Solari A, Giordano A, Kasper J et al. Role preferences of people with multiple sclerosis: image-revised, computerized self-administered version of the Control Preference Scale. PLoS ONE 8, e66127 (2013).Crossref, Medline, CAS, Google Scholar3 Pietrolongo E, Giordano A, Kleinefeld M et al. Decision-making in multiple sclerosis consultations in Italy: third observer and patient assessments. PLoS ONE 8, e60721 (2013).Crossref, Medline, CAS, Google Scholar4 Holmes-Rovner M. International Patient Decision Aid Standards (IPDAS): beyond decision aids to usual design of patient education materials. Health Expect. 10, 103–107 (2007).Crossref, Medline, Google Scholar5 International patient decision aid standards (IPDAS). www.ipdas.ohri.ca.Google Scholar6 Stacey D, Bennett CL, Barry MJ et al. Decision aids for people facing health treatment or screening decisions. Cochrane Database Syst. Rev. 10, CD001431 (2011).Google Scholar7 Köpke S, Solari A, Khan F, Heesen C, Giordano A. Information provision for people with multiple sclerosis. Cochrane Database Syst. Rev. 4, CD008757 (2014).Google Scholar8 Solari A, Martinelli V, Trojano M et al. An information aid for newly diagnosed multiple sclerosis patients improves disease knowledge and satisfaction with care. Mult. Scler. 16, 1393–1405 (2010).Crossref, Medline, CAS, Google Scholar9 Giordano A, Lugaresi A, Confalonieri P et al. Implementation of the ‘Sapere Migliora’ information aid for newly diagnosed people with multiple sclerosis in routine clinical practice: a late-phase controlled trial. Mult. Scler. 20, 1234–1243 (2014).Crossref, Medline, CAS, Google Scholar10 Galea I, Lederer C, Neuhaus A et al. A web-based tool for personalized prediction of long-term disease course in patients with multiple sclerosis. Eur. J. Neurol. 20, 1107–1109 (2013).Crossref, Medline, CAS, Google Scholar11 Colombo C, Filippini G, Synnot A et al. Development and assessment of a website presenting evidence-based information for people with multiple sclerosis: the IN-DEEP project. BMC Neurol. 16, 30 (2016).Crossref, Medline, Google Scholar12 MS Decisions. www.msdecisions.org.uk.Google Scholar13 Healthwise: Health Content and Patient Education Solutions. www.healthwise.org.Google Scholar14 PatientsLikeMe: Live better, together! www.patientslikeme.com.Google Scholar15 Khan F, Amatya B, Kesselring J, Galea M. Telerehabilitation for persons with multiple sclerosis. Cochrane Database Syst. Rev. (4), CD010508 (2015).Medline, Google ScholarFiguresReferencesRelatedDetailsCited ByAdvance Care Planning in Neurodegenerative Disorders: A Scoping Review12 January 2022 | International Journal of Environmental Research and Public Health, Vol. 19, No. 2RebiQoL: A randomized trial of telemedicine patient support program for health-related quality of life and adherence in people with MS treated with Rebif5 July 2019 | PLOS ONE, Vol. 14, No. 7 Vol. 6, No. 6s Follow us on social media for the latest updates Metrics Downloaded 69 times History Published online 22 November 2016 Published in print December 2016 Information© Future Medicine LtdKeywordsdecision aidsmultiple sclerosispatientsself-managementshared decision makingweb resourcesFinancial & competing interests disclosureA Solari is an advisory board member for Genzyme, Merck Serono and Novartis; and has received honoraria for lecturing from Almirall, Biogen, Excemed, Genzyme, Novartis and Teva. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.Writing assistance was utilized in the production of this manuscript. Writing assistance was provided by Content Ed Net (Madrid, Spain), with funding from Almirall SA (Barcelona, Spain).PDF download

Long-Term Disease Course and Pregnancy Outcomes in Women with Inflammatory Bowel Disease: An Indian Cohort Study.

The literature on interaction between pregnancy and inflammatory bowel disease (IBD) is inconsistent, and there are no reports on this aspect from Asia. This study evaluated the impact both IBD and pregnancy have on each other in a large cohort of Indian patients. In total, 514 females with ulcerative colitis (UC) or Crohn's disease (CD) aged between 18 and 45years attending IBD clinic, at our institute, from July 2004 to July 2013 were screened, and patients with data on pregnancy status were included (n=406). Pregnancies were categorized as either before, after or coinciding with disease onset. Long-term disease course was ascertained from prospectively maintained records. Pregnancy and fetal outcomes were recorded from antenatal records or individual interviews. Of 406 patients (UC: 336, CD: 70), 310 became pregnant (UC: 256, CD: 54), with a total of 597 pregnancies (UC: 524, CD: 73). More UC patients with pregnancies were in long-term remission than non-pregnant patients (56.7 vs. 43.4%, p=0.04). Long-term remission was less frequent in UC patients in whom pregnancy coincided with disease onset than patients with pregnancies before and after/pregnancy after the disease onset (41.4 vs. 62.5%, p=0.023). Pregnancies after the disease onset were associated with more cesarean sections and adverse fetal outcomes than pregnancies before disease onset in both UC and CD patients. Long-term disease course in UC patients was better in pregnant as compared to non-pregnant patients. Among pregnant UC patients, disease course was worst when pregnancy coincided with disease onset. Pregnancy and fetal outcomes were worse in pregnancy after disease onset than pregnancy before disease onset.

Multiple sclerosis: clinical profiling and data collection as prerequisite for personalized medicine approach.

Multiple sclerosis (MS) is a highly heterogeneous disease as it can present inter-individually as well as intra-individually, with different disease phenotypes emerging during different stages in the long-term disease course. In addition to advanced immunological, genetic and magnetic resonance imaging (MRI) profiling of the patient, the clinical profiling of MS patients needs to be widely implemented in clinical practice and improved by including a greater range of relevant parameters as patient-reported outcomes. It is crucial to implement a high standard of clinical characterization of individual patients as this is key to effective long-term observation and evaluation.To generate reliable real-world data, individual clinical data should be collected in specific MS registries and/or using intelligent software instruments as the Multiple Sclerosis Documentation System 3D. Computational analysis of biological processes will play a key role in the transition to personalized MS treatment. Major breakthroughs in the areas of bioinformatics and computational systems biology will be required to process this complex information to enable improved personalization of treatment for MS patients.

Autoimmune hepatitis and long-term disease course in children in Turkey, a single-center experience

The aim of this study is to determine clinic and laboratory features, treatment protocols, treatment responses, and long term follow-up of children with autoimmune hepatitis (AIH) in a region of Turkey followed at Ege University. The records of 47 children with AIH between 1998 and 2012 were retrospectively analyzed for clinical profiles, treatment response, relapse rate, and long-term side effects. The median age of the children was 10±4.1 years (55.3% females). A total of 29 patients presented with chronic hepatitis (61.7%). According to the autoantibody profiles, 40 (85.1%) and seven (14.9%) cases were classified as type 1 and type 2, respectively. Presentation with acute hepatitis and chronic hepatitis was significantly higher in type 1 disease. Laboratory findings at presentation was found similar among races as well as AIH types (P>0.05). The prednisolone was used for remission induction in 37 patients; 86.4% (n: 32) achieved a complete response, 2.7% (n: 1) achieved a partial response, and four patients (10.8%) showed no response. Maintenance was attained by low-dose steroid plus thiopurine and relapse in steroid responders (n: 32) was 9.4% (n: 3) at 8, 12, and 48 months. A total of 36% (n: 24) had neither acute nor chronic treatment side effects. Bone marrow suppression was observed in five patients and hyperglycemia was observed in one patient (10.6 and 2.1%), respectively. AIH type 1 prevails in children in a region of Turkey during the second decade of life. Low-dose corticosteroids combined with azathioprine are found.

THE RISK FACTORS AND SPECIFIC FEATURES OF OSTEOPENIC SYNDROME IN PATIENTS WITH CHRONIC NON-INFECTIOUS DISEASES

Objective: to analyze the risk factors (RFs) of osteoporosis (OP), the risk of OP-related fractures, the specific features of osteopenic syndrome in patients with chronic non-infectious diseases (CNID) (coronary heart disease (CHD), hypertension, chronic obstructive pulmonary disease (COPD), and asthma). Materials and methods. The investigation enrolled 377 patients (mean age 55.3 ± 1.6 years) with CNID and 221 persons (mean age, 53.2 ± 1.3 years) who formed a control group. According to the nosological entity, the patients were divided as follows: Group 1 included 84 patients with CHD and hypertension; Group 2 comprised 99 hypertensive patients; Group 3 consisted of 70 patients with COPD; and Group 4 included 124 asthmatic patients. The examinees of all the groups were matched for age, gender, and body mass index. The investigation excluded patients with functional class IV chronic heart failure, continuous atrial fibrillation, heart valve disease, or myocardial noncoronarogenic diseases and those with other diseases and conditions that could have an independent impact on bone metabolism. Prior to the examination, the patients had received no specific therapy for the prevention and treatment of OP. RFs for OP were assessed using the one-minute test recommended by the International OP Foundation (2008); 10-year risk for OP-related fractures were calculated applying the FRAX computer program in accordance with the guidelines of the International OP Association and the World Health Organization (WHO, 2008). To investigate bone mineral density (BMD), bioenergy X-ray densitometry of the lumbar spine and proximal femur was carried out by means of a Lunar DPX apparatus (USA). The results were assessed using the t-test in standard deviations (SD) from the peak bone mass according to the WHO guidelines. Results. The RFs of OP were more frequently recorded in the patients with CNID than in the healthy individuals. RFs, such as smoking, low physical activity, and low-energy fractures, were most common in the patients with cardiovascular disease or COPD. The frequent use of glucocorticoid therapy was also an important RF in the patients with COPD. CHID considerably increased the risk of fractures in the succeeding 10 years after disease onset. The high risk of fractures, those of the proximal femur in particular, provides a rationale for the need for timely antiosteoporotic therapy in the majority of patients with CNID. The performed investigation demonstrated that the BMD values in the patients with CNID corresponded, on the average, to the osteopenia criteria; the lowest BMD values were recorded in the patients with COPD and associated cardiovascular disease. The severe course of osteopenic syndrome (a BMD decrease that was diagnostically significant for OP concurrent with fractures was observed in one-third of patients with CNID. The patients with cardiovascular disease or COPD showed a high incidence and degree of OP, which allows these diseases to be considered as a RF for decreased BMD. The long-term uncontrolled course of disease, the degree of organ and functional disorders in the patients with CNID, and concomitant use of glucocorticoid therapy contribute to a reduction in BMD. Conclusion. RFs for OP were identified in the majority of patients with CNID. The high risk of fractures due to an obvious BMD decrease in patients with CNID requires timely diagnosis, treatment, and prevention of osteopenia.

Influenza infection modulate the extent and duration of inflammation in the central nervous system of EAE pathogenesis

Abstract Multiple sclerosis (MS) starts with increased migration of auto-reactive lymphocytes across the blood-brain barrier, resulting in persistent neurodegeneration. Evidence from clinical and epidemiological studies indicated that viral infections are important in the induction of autoimmunity including MS. Using the experimental autoimmune encephalomyelitis (EAE) as a model for MS, we demonstrated that influenza infection can modulate the development of the central nervous system (CNS) inflammation. It was shown that mice with influenza infection were unable to recover from EAE, exhibiting a long-term chronic disease course. We found that the exacerbated disease after influenza virus infection was due to elevated Th1 cells infiltration into CNS, and this enhancement in the early stage of EAE disease is accompanied by increased CCR5+CD4+, CXCR3+CD4+ T cell and MOG35–55 specific CD4+ T cells localized in the lung and its draining lymph node in influenza virus-infected mice. We also observed much more CD45highCD4+CD44high effector T cell and CCR5, CXCR3, IFNγ expressing Th1 type cells entering the CNS at both acute phase and later stage of EAE disease in influenza virus-infected mice. Finally, the administration of a CCR5 and CXCR3 antagonist inhitibs the Th1 cells infiltration and significantly decreases the difference of clinical disease course between mock and influenza virus-infected mice. Taken together, our data suggested that prior influenza infection may affect subsequent EAE pathogenesis by selectively recruit CCR5 and CXCR3 bearing CD4 T cells into CNS.

P154. Temporal relationship of pregnancy to long-term disease course and pregnancy and foetal outcomes: North Indian inflammatory bowel disease cohort study

P154. Temporal relationship of pregnancy to long-term disease course and pregnancy and foetal outcomes: North Indian inflammatory bowel disease cohort study

Pregnancy course and outcomes in women with arrhythmogenic right ventricular cardiomyopathy

ObjectivesTo characterise pregnancy course and outcomes in women with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C).MethodsFrom a combined Johns Hopkins/Dutch ARVD/C registry, we identified 26 women affected with ARVD/C (by 2010 Task...

Long-term course of Crohn's disease in Japan: Incidence of complications, cumulative rate of initial surgery, and risk factors at diagnosis for initial surgery.

Intestinal complications of stenosis or fistula may occur during the course of Crohn's disease (CD), and surgery is performed in a fair number of patients. The risk factors for initial surgery in a Japanese hospital-based cohort of CD patients were evaluated. This study was a single-center, retrospective, cohort study. The subjects were 520 patients who underwent inpatient and outpatient treatment at our hospital, had a definitive diagnosis of CD, and no previous surgery. Three parameters were investigated: (i) cumulative incidence of stenosis and fistula; (ii) cumulative rate of initial surgery for each disease type; and (iii) risk factors at diagnosis for initial surgery. (i) Stenosis and fistula increased with time, with stenosis or fistula appearing in about half of the patients after 5 years. (ii) The cumulative rate of initial surgery was about 50% after 10 years. (iii) The patient factors at diagnosis of current smoker, upper gastrointestinal disease, stricturing, penetrating, moderate to severe stenosis of the jejunum, moderate to severe stenosis of the ileum, and moderate to severe stenosis of the terminal ileum were risk factors for initial surgery. Stenosis or fistula appeared in about half of the patients after 5 years from diagnosis. When upper gastrointestinal disease or complicated small intestinal lesions are seen at the time of diagnosis, the cumulative rate of initial surgery is significantly higher.

Juvenile chronic non-bacterial osteomyelitis (CNO): Long term course of disease and response to treatment in a large institutional cohort

Chronic non-bacterial osteomyelitis (CNO) is an inflammatory disorder of the skeletal system of unknown etiology. Long-term follow-up and response to treatment data have rarely been reported. The aim of the study was to characterize the clinical, radiological, histological and laboratory data at juvenile CNO onset, and to analyze the long term treatment response.

An overview of pregnancy-related issues in patients with multiple sclerosis.

Although pregnancy in women with multiple sclerosis (MS) is not generally considered high risk, there are some associated therapeutic challenges. The pregnancy-associated reduction in the relapse rate, especially in the third trimester, is followed by a sharp increase in the first few months postpartum. Nevertheless, retrospective evidence for pregnant women with and without MS followed for up to 10years indicates that pregnancy has no perceptible effect on long-term disease course or disability progression. Likewise, MS has noapparent effects on the pregnancy course or fetal outcomes. All disease-modifying therapies (DMTs) have potential adverse effects on fertility and pregnancy outcomes, but the level of risk varies amongst agents. There is some support for continued use of interferon-β and glatiramer acetate throughout pregnancy to reduce the risk of relapse. Use of DMTs during breastfeeding is best avoided if possible. Close evaluation of drug safety information is imperative when managing women with MS who are pregnant or wish to become pregnant. Decision-making should be a shared experience between patient and physician, and the approach must be individualized for each patient.

Tumefactive demyelinating lesions as a first clinical event: Clinical, imaging, and follow-up observations

BackgroundTumefactive demyelinating lesions (TDLs) are associated with a variety of demyelinating diseases in the central nervous system (CNS). However, there are no current guidelines describing how to classify and treat patients with this rare phenotype. Thus, the present study aimed to determine the long-term evolution and disease course of patients initially presenting with TDLs and to describe their clinical and radiographic characteristics. MethodsFrom the National Cancer Center registry of inflammatory diseases of the CNS, 31 patients initially presenting with TDLs with follow-up for at least 12months were enrolled and their demographic, clinical, and radiographic characteristics were evaluated. ResultsThe median follow-up duration was 37.6months, during which time 11 patients were diagnosed with neuromyelitis optica spectrum disorder (NMOSD), seven with multiple sclerosis (MS), and 11 remained idiopathic; six did not experience any further clinical events (isolated demyelinating syndrome), and five patients experienced recurrent demyelinating events that were not consistent with either MS or NMOSD. Of the remaining two patients, one was diagnosed with hyperthyroidism-associated demyelination and one with tacrolimus-induced demyelination. ConclusionsThe majority of TDLs evolve into MS or NMOSD. However, despite extensive diagnostic work-ups and long-term follow-ups, the etiology of TDLs was unknown for some patients.

Are extraintestinal manifestations associated with disease outcomes in inflammatory bowel diseases? Results from the Veszprem population based inception cohort database

Background and Aims: Association between extraintestinal manifestations (EIM) and disease activity suggest a common pathogenic link. Limited data are available on the effect of EIMs on the long-term disease course in inflammatory bowel diseases (IBD). The aim of this study was to analyze the association between the presence of EIMs (joint, skin, eyes) and treatment steps and long term disease outcomes in the population-based inception cohort in the Veszprem province database after 1977. Methods: A well-characterized Hungarian cohort of 506 incident cases with Crohn's disease (male/female: 251/255, age at diagnosis: 31.5 years, SD 13.8 years) diagnosed from January 1, 1977 were included and data of 347 incident UC patients diagnosed from January 1, 2002 were analyzed (m/f: 200/147, median age at diagnosis: 36, IQR: 26 – 50 years, duration: 7, IQR 4 – 10 years). Follow-up data were collected until December 31, 2012. Both in- and outpatient records were collected and comprehensively reviewed. Results: EIMs (Joint, skin and eyes) were present in 32.2% of the CD and 17.3% of the UC patients. Presence of EIMs was associated in both CD and UC with female gender (pCD = 0.01, pUC = 0.07), with ileocolonic and extensive location (pCD = 0.009, pUC = 0.003). In CD, there was an association with smoking (p < 0.001), but not in UC. No association was found in both CD and UC between the presence of EIMs and need for surgery/colectomy. Presence of EIMs was associated with the need for azathioprine (pCD< 0.001; pUC = 0.004) and need for steroids (pCD < 0.001, pUC < 0.001) in both diseases, but anti-TNF therapy was only associated in CD (p < 0.001). In Kaplan-Meier analysis there was an association between the presence of EIMs and time to first UC-related hospitalization (p = 0.002). Conclusions: In IBD, the presence of EIM was associated higher maximum treatment steps during the disease course including need for steroids, azathioprine. In CD, there was an association with anti-TNF therapy while in UC with the need for hospitalization.