Clinical Features and Course in Pediatric Onset Multiple Sclerosis

WOS: 000312600000015Objective: Multiple Sclerosis is uncommon in children and adolescents. Two to 5 % of all patients with MS have onset before age 16. Although the clinical profile of MS appears similar to adults some features may differ. The disease may lead to significant disability at a younger age. Our aim is to determine the clinical features and, disease course in pediatric onset multiple sclerosis. Methods: In this study, we evaluated 21 patients with pediatric onset (before age 18). The records of patients were reviewed retrospectively. The following variables were evaluated; sex, age at onset, presenting symptoms, disease course, relapse rate, immunomodulatory treatment. Results: Twenty one patients were included to study. The mean age at onset was 15.2 (ranged 8-17). The female/male ratio was 1.3. The median follow up period was 5 years. The major presenting symptoms were pyramidal (9 patients), brainstem (8 patients) and optic neuritis (4 patients). The initial disease course was relapsing remitting in 19 patients but in 3 patients, secondary progression was observed before age 30. Two patients were with clinically isolated syndrome. Nineteen patients were treated with immunomodulatory drugs. In 4 patients Mitoxantrone was used because of progressive course and relapses. Conclusion: The clinical presentation of multiple sclerosis with pediatric onset is usually benign but in the long term, patients with pediatric onset can become disabled at a younger age

Assessment of Early Cognitive Impairment in Patients with Clinically Isolated Syndromes and Multiple Sclerosis

WOS: 000335016500001PubMed ID: 25944978Objective. The aim of our study was to investigate the frequency and pattern of cognitive impairment in patients with clinically isolated syndromes and definite diagnosis of multiple sclerosis within the last 2 years. Methods. We assessed the cognitive status of 46 patients aged 18-49 years with clinically isolated syndromes or definite diagnosis of multiple sclerosis who have onset of their symptoms within the last 2 years. Patients were matched with 40 healthy participants for age, sex, and educational level. Neuropsychological assessment was performed by stroop test, paced auditory serial addition test (PASAT), controlled oral word association test (COWAT), clock drawing test, trail making test (TMT), faces symbol test (FST). Hamilton Depression Scale and Modified Fatigue Impact Scale were used to quantify the severity of any depression and fatigue the subjects might suffer. Results. 19.6% of early MS/CIS group failed at 4 and more tests and had significant cognitive impairment focused on attention, executive functions, memory, and learning. No significant relationship was found between cognitive impairment and disability and fatigue scores. Discussion. Cognitive impairment can be present from the earliest stage of multiple sclerosis. It should be considered among the main manifestations of MS even in the earliest stages of the disease

Propuesta de reducción del retraso de productos terminados en el área de producción de una empresa metalmecánica mediante la Teoría de las Restricciones y herramientas Lean

La siguiente tesis está conformada por cuatro capítulos, donde el primero presenta el marco teórico investigado en fuentes académicas respaldadas por autores especializados en metodologías de administración de producción y manufactura. En el segundo capítulo se muestra el análisis y diagnóstico realizado a una empresa metalmecánica nacional donde explica su entorno, sus procesos y el problema que enfrenta. En el capítulo 3 se muestra la propuesta de solución en función al diagnóstico realizado y el marco teórico investigado, se aterrizará al entorno de la empresa las diferentes metodologías explicadas en el capítulo 1, además se presentará una análisis económico justificando la rentabilidad de la implementación de la solución. Para finalizar en el capítulo 4 brinda las principales conclusiones a partir de la propuesta de solución y recomendaciones futuras para la empresa en estudio.Tesi

Progressive Multifocal Leukoencephalopathy in a Case Without Clear Evidence of Immunodeficiency

WOS: 000309218800026Introduction: Progressive Multifocal Leukoencephalopathy(PML) is a rare demyelinating disease of the central nervous system due to reactivation of a latent infection with JC papovavirus. PML predominantly occurs in severely immunosupressed patients, mostly as a late complication of acquired immunodeficiency syndrome and lymphoproliferative disorders. Clinically it is manifested by motor and sensory deficits, ataxia, cognitive impairment and dementia, corresponding to central nervous system (CNS) lesions. It is difficult to distinguish PML clinically from immune-mediated diseases of the CNS, such as multiple sclerosis, neuropsychiatric sistemic lupus erythematosus and CNS vasculitis. Case Report: A 19-year-old woman presented with ataxia and slurred speech rapidly progressing to quadriparesis and confusion. The diagnosis of PML was made by detecting the JC virus in the cerebrospinal fluid by polymerase chain reaction. Discussion: PML predominantly occurs in severely immunosupressed patients. However, it must also be kept in mind in patients without an apparent immunodeficiency when the clinical and radiological features are typical

Nosocomial Infections in a Neurological Intensive Care Unit

WOS: 000270682900006Background: Nosocomial infections in intensive care units are common and associated with high morbidity, mortality. This study was planned to evaluate the occurance and causes of site specific nosocomial infections in a neurological intensive care unit (NICU) and their relevance to underlying diseases. Methods: Patients treated for more than 24 hours in Ege University Medical School Hospital NICU were evaluated by prospective daily surveillance for overall and site specific infections during the 12 months of the year 2005. Nosocomial infections incidence and their relation with underlying disease were investigated. Results: Three hundred and fifty six patients were included. A hundred and ten nosocomial infections were seen. The rate of infection per 100 patients was 30.8. Site-specific infections were; urinary tract infection (49.1%), pneumonia (30.9%), catheter related blood stream infections (18.1%) and primary bacteremia (1.8%). In univariate analysis, no differences were found between the patients with stroke and other NICU disorders in terms of risk factors for NICU-AI such as; age, gender, type of nosocomial infections, length of stay, and mortality. But the length of stay was significantly prolonged and mortality was significantly higher in patients with NICU-AI when compared with patients without NICU-AIs. Conclusion: The incidence of nosocomial infections in our NICU was found to be as high as in other critical care units and causes higher mortality. This was supposed to be associated with prolonged hospital stay and insufficient compliance to infection control measures instead of the underlying disease of the patients

Differences Between General Neurologists And Multiple Sclerosis Specialists In The Management Of Multiple Sclerosis Patients: A National Survey

Introduction: The management of multiple sclerosis (MS) has become more complicated after the introduction of new diagnostic and treatment options. Despite the abundance of guidelines, the experience of physicians still plays a major role in the management of patients. This study aimed to define differences in behavior patterns between general neurologists (GNs) and MS specialists (MSSs). Methods: We conducted a survey of 36 questions to 318 neurologists, including 33 MSSs. The survey covered topics including laboratory investigations, pregnancy, and treatment. Results: Our study found many differences between GNs and MSSs in terms of management, the most important being treatment initiation and switching. GNs had a tendency to initiate treatment later than MSSs however, they tended to switch treatment faster. Our study also showed that GNs ordered magnetic resonance imaging (MRI) more frequently than MSSs, even if patients were clinically stable. Moreover, although GNs more frequently relied on MRI, they did not consider brain atrophy as an important measure in the follow-up of their patients. Furthermore, GNs considered replacement therapy less often than MSSs, even in patients with vitamin D deficiency. Discussion: Our study revealed important discrepancies between the management patterns of GNs and MSSs in MS patients. These findings suggest the need for a national education program for GNs on MSSs.Wo