Patient characteristics and the effects of intravenous immunoglobulin in patients with Guillain-Barre syndrome.

Purpose - The purpose of this study is to determine the diagnosis- and treatment-related characteristics in Guillain-Barré syndrome (GBS) and to evaluate the effects of early intravenous immunoglobulin (IVIg) treatment on disability, mortality and prognosis. Materials and methods - Adult patients who were diagnosed with GBS in our clinic between January 2000 and January 2014 were retrospectively scanned. While the patients undergoing IVIg treatment were included in the study, the other neuropathic diseases were excluded. Patients were divided into two groups based on the administration time of the IVIg treatment; Group 1 (<7 days) and Group 2 (≥ 7 days) Group 1 consisted of patients undergoing IVIg treatment within 7 days after presentation of symptoms and Group 2 consisted of patients undergoing IVIg treatment on and after 7th day following presentation of symptoms. The scores from Hughes Functional Grading Scale (HFGS) on admission and one month laterwere recorded in all patients in order to evaluate the disability and prognosis in terms of demographic and clinical laboratory characteristics. Results - In this study, 49 GBS patients were included (31 patients in Group 1 and 18 patients in Group 2). Demyelinating form of GBS was determined in 22 (44.8%) patients.). While there was no difference between both groups (p: 0.288, p: 0.762, p: 0.693 respectively) in terms of intensive care and rehabilitation requirement and progression, only 2 patients in Group 1 died. While HFGS mean score on admission in all the patient groups was 3.27±0.974, their HFGS mean score at month 1 was 2.53±1.226. There was no difference between the groups in terms of HFGS mean scores on admission and at month 1. Within each groups, there was a significant improvement between initial (on admission) HFGS scores and HFGS scores acquired at month 1. Conclusion - In this study, demyelinating form was more frequent than axonal form. A total of 2 g/kg dose of IVIg treatment administered for 5 days as a standard in GBS patients ensured a significant improvement on both disability and early and late administration and early administration of the treatment does not lead to any difference in intensive care unit and rehabilitation requirements.

Correlation of cumulative corticosteroid treatment with magnetic resonance imaging assessment of avascular femoral head necrosis in patients with multiple sclerosis.

Increased risk of osteoporosis, fractures, and avascular necrosis (AVN) has been suggested in multiple sclerosis (MS). Patients with MS are often exposed to corticosteroid treatment (CST) during the disease course and conflicting reports exist regarding complications of CST. Our study aims to investigate the association between cumulative doses of CST and radiographic evaluation of AVN of the femoral head in MS. Twenty-six MS patients (mean age, 38.4±10 yr) were enrolled and prospectively evaluated for AVN by magnetic resonance imaging (MRI). The mean disease duration was 11.5±8.5 years and mean expanded disability status scale (EDSS) score was 3±2. The cumulative dosage of CST varied between 20 g and 60 g; patients were grouped into two categories: 1) CST between 20-40 g, 17 (65%) patients; 2) CST ≥40 g; 9 (35%) patients. The relationship between cumulative CST dosage and MRI diagnosis of AVN was statistically insignificant (P>0.9). Clarification of the cumulative effect of CST in the development of AVN is of great importance for future long-term steroid treatment strategies.

The presentation of headache in neuro-Behçet's disease: a case-series.

INTRODUCTION: Behçet's disease (BD) is a chronic, relapsing, multisystemic, inflammatory disorder with unknown etiology. Neurological involvement is observed in about 5% of the patients with BD and headache is a frequently reported symptom with or without neurological involvement. In this case-series, we aim to demonstrate the secondary headaches associated with neuro-Behçet's disease in consecutive BD patients who had been referred for neurologic evaluation. METHODS: The case-series included 17 patients (11 males [65%]), mean age 41 +/- 11.4, who met the criteria for BD established by the International Study Group for BD and developed headache during the disease course. RESULTS: Seventeen BD patients were identified, the neurologic presentations included in parenchymal involvement 12 (70%), extraparenchymal involvement/venous thrombosis in 5 (30%), seizure disorder in 2 (12%), and psychiatric problems/depression or anxiety in 5 (30%) patients, respectively. The characteristics of the headache according to the patients' reports included subacute, moderate to severe headache with unilateral localization and throbbing quality accompanied by nausea, vomiting, and aggravation upon awakening. CONCLUSION: Because neurological involvement is one of the most devastating aspects of BD, the need to treat neurological involvement as early as possible, the possible role of headache onset as an early indicator for neurological involvement in this relatively uncommon disease should be kept in mind.

Assessment of autonomic nervous system dysfunction in multiple sclerosis and association with clinical disability.

Recent studies have reported autonomic dysfunction (AD) in multiple sclerosis (MS), and bladder and/or bowel dysfunction, orthostatic hypotension, and cardiac adaptation disorders have been observed in a wide range of patients (15-80%). The primary aim of this study is to investigate the frequency and association of AD in MS patients, assessed by sympathetic skin response (SSR) and a symptoms questionnaire. The secondary aims are to study the association of AD and disease disability assessed by expanded disability status scale (EDSS), as well as disease duration. One hundred clinically definite MS (CDMS) patients were evaluated for autonomic nervous system (ANS) dysfunction by use of an autonomic symptoms questionnaire and SSR testing. The relationship between these methods, AD and disease-related parameters, such as the expanded disability status scale (EDSS) and disease duration were all evaluated. Sixty-five per cent of the patients presented with AD and 29% of these patients had abnormal SSR results. MS patients with high EDSS values (EDSS>4) and longer disease duration were more likely to have ANS dysfunction (p<0.0001). ANS dysfunction is not uncommon in CDMS patients and thus non-invasive investigations of AD are warranted to optimize AD evaluation and disease management.

Clustering of organ-specific autoimmunity: a case presentation of multiple sclerosis and connective tissue disorders.

Multiple sclerosis (MS) is the most common demyelinating disease caused by an autoimmune inflammatory process in the central nervous system (CNS) and is associated with aberrant immune response to myelin selfantigens. Coexistence of MS with other autoimmune disorders, including connective tissue disorders including systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome and scleroderma have been reported previously. In the present article we report the coexistence of MS, familial mediterranean fever and ankylosing spondylitis in a patient and review the clinical presentation, neurologic findings, cerebrospinal fluid and radiologic characteristics and treatment options. We further discuss the immunopathogenetic mechanisms for a possible association between MS and autoimmune disorders.