BACKGROUND: The prevalence of eosinophilic esophagitis (EE) is increasing rapidly in Western countries. Several case series of EE have also been reported in Japan. However, the prevalence of EE in Japanese patients as investigated by upper gastrointestinal endoscopy is unknown. Therefore, we carried out a prospective multicenter study to address ...

A case series of 15 patients. To report a series of patients with coexisting multiple sclerosis (MS) and progressive myeloradiculopathy who were successfully treated with surgical decompression, fusion, and fixation. MS and cervical myeloradiculopathy share clinical signs, including gait dysfunction, motor weakness, and hyperreflexia. Distinguishing between these 2 entities may ...

Objective:  To investigate possible peripheral nervous system (PNS) affection in patients with multiple sclerosis (MS) by standard nerve conduction velocity (SNC) and pain-related evoked potentials (PREP), a novel electrophysiological method for diagnosing small fibre neuropathy. Background:  Former neuropathological and electrophysiological studies demonstrated subtle PNS affection in MS, but routine measurements ...

Burman J, Raininko R, Fagius J. Bilateral and recurrent optic neuritis in multiple sclerosis. Acta Neurol Scand: 2011: 123: 207-210. © 2010 John Wiley & Sons A/S. Objective -  To assess the frequency of bilateral and recurrent optic neuritis (ON) in multiple sclerosis (MS) and to compare these results with epidemiological ...

Purpose The pharmacology, pharmacokinetics, clinical efficacy, and safety of febuxostat are reviewed. Summary Febuxostat is a novel non-purine selective inhibitor of xanthine oxidase for the management of hyperuricemia in patients with gout. The ability of febuxostat to decrease serum uric acid production through selective inhibition of enzyme xanthine oxidase has ...

Background:  Patients with acute disseminated encephalomyelitis (ADEM) may relapse and some may ultimately convert to multiple sclerosis (MS); however, no criteria that can predict MS conversion are available to date. Our aim was to describe the clinical and magnetic resonance imaging (MRI) features of patients with an initial ADEM attack ...

Natalizumab has been demonstrated to be highly effective in reducing measures of disease activity, such as clinical relapse rate, and gadolinium (Gd)-enhancing and new or enlarging T2 lesions appearance in patients with relapsing remitting multiple sclerosis (RRMS). Up to date, no data on the effect of natalizumab on cortical pathology ...

BACKGROUND: The efficacy of granulocyte transfusions in patients with HLA alloimmunization is uncertain. A flow cytometric assay using dihydrorhodamine 123 (DHR), a marker for cellular NADPH oxidase activity, was used to monitor the differential survival of transfused oxidase-positive granulocytes in alloimmunized patients with chronic granulomatous disease (CGD). STUDY DESIGN AND ...

To assess the predictive value of T2 lesions on the rate of progression of disability in multiple sclerosis (MS). We reanalyzed T2 lesion number and load on brain MRI scans, performed before 1997, of 186 MS patients, who were clinically followed. There were 90 patients with progressive MS (35 secondary ...

Background and purpose:  Several previous studies have employed optical coherence tomography (OCT) of the optic disc and 'white-on-white' automated perimetry to evaluate optic neuritis (ON) associated with multiple sclerosis (MS). This study employed OCT, white-on-white automated perimetry as well as 'blue-on-yellow' automated perimetry to evaluate MS patients with or without ...

Gait impairment and fatigue are common and disabling problems in multiple sclerosis (MS). Characterisation of abnormal gait in MS patients has been done mainly using observational studies and simple walking tests providing only limited quantitative and no qualitative data, or using intricate and time-consuming assessment procedures. In addition, the correlation ...

We aimed to evaluate the frequency and determinants of restless legs syndrome (RLS) in a group of 76 patients with amyotrophic lateral sclerosis (ALS) and 100 control subjects. A diagnosis of RLS was made according to the criteria of the International RLS Study Group, and severity was assessed by the ...

Background: There is increasing evidence on cerebrospinal fluid (CSF) oligoclonal IgM (OCIgM) predicting a more aggressive disease course in relapsing-remitting Multiple Sclerosis (MS), while there is a scarcity of data for primary progressive MS (PPMS). Objective: Our aim was to investigate the presence and possible prognostic value of CSF OCIgM ...

Neuromyelitis optica (NMO) is a rare demyelinating disease, affecting selectively the optic nerve and the spinal cord. It was previously considered to be a severe variant of multiple sclerosis (MS) due to the similar pathological features and its resemblance to optico-spinal, or Japanese, MS, typical of Asian populations. The finding ...

The associations between vitamin D and MRI measures of brain tissue injury have not been previously investigated in multiple sclerosis (MS). This research evaluates the significance of vitamin D and its active metabolites in brain tissue injury and clinical disability in MS patients. The study population consisted of 193 MS ...

The motor unit number index (MUNIX) refers to an electrophysiologic technique that measures the approximate number of motor units using the surface electromyographic interference pattern (SIP) recorded during voluntary contraction. This study was done to assess the reproducibility of MUNIX performed on hypothenar muscles in 62 normal controls and 22 ...

Extracranial venous stenosis (EVS) has recently been implicated as the primary cause of multiple sclerosis (MS). Objective: The aim of this study was to determine the presence of EVS in MS patients. We performed selective extracranial venography on 42 patients with early MS (EMS): clinically isolated syndrome (CIS) or relapsing-remitting ...

To examine how coping styles among patients with multiple sclerosis (MS) change over time and how patients' coping styles after 5 years are associated with disability pension. Seventy-six MS patients and 94 healthy controls were included in this study. The patients were examined at baseline and 5 years later. This ...

TNFRSF6B and TNFRSF14 genes were recently associated with Crohn's disease and rheumatoid arthritis. TNFRSF14 is known as herpes virus entry mediator (HVEM), and herpes viruses have been involved in the aetiology of multiple sclerosis (MS). MS patients present human herpes virus 6 (HHV6) in active plaques and increased antibody responses ...

Many studies have observed atrophy and abnormal diffusion within the CC in MS. However, few studies have addressed whether such abnormalities appear at the earliest stage of MS, especially in CIS. In this study, we aimed to investigate the CC integrity and patterns of CC abnormalities in CIS with diffusion ...

Neuro-axonal damage is a well known sequelae of MS pathogeneses. Consequently, our aim was to test whether the ∼20% of patients with MS exhibiting a clinically benign disease course also have minimal neural dysfunction as reflected by the global concentration of their MR imaging marker NAA. Q(NAA) was obtained with ...

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late onset neurodegenerative disorder that affects carriers of the fragile X premutation, typically after age 50. Common symptoms include intention tremor, ataxia, neuropathy, autonomic dysfunction, cognitive decline, and dementia. The objectives of this study were to determine if patients with FXTAS have altered ...

Brain derived neurotrophic factor (BDNF) regulates several CNS physiological and pathological processes. To investigate in multiple sclerosis (MS) patients, the relationship between the Val66Met polymorphism of BDNF and clinical markers of disease activity and MRI markers of focal and diffuse brain pathologies. 45 MS patients and 34 healthy controls (HCs) ...

Background  Tuberous sclerosis complex is a multisystem inherited disorder characterized by the development of tumour-like growths in brain, skin and other organs. Although cutaneous vascular anomalies are not considered a common manifestation, we have encountered co-occurrence of port wine stains and tuberous sclerosis. Objective  To assess the prevalence of port ...

To monitor increases or decreases in cardiovascular disease (CVD)-related proteins that will be released from the deposits or plaque on the inner wall of blood vessels. Protein profiles of sera from healthy subjects and CVD patients were determined via 2-DE. Differentially expressed spots in CVD patients were identified by ESI-Q-TOF ...

Anti-aquaporin-4 antibodies are believed to have a central pathogenetic role in neuromyelitis optica (NMO). B-cell activating factor (BAFF) is one of the crucial factors that determines the fate and survival of B cells and may play a role in induction of antibody-mediated autoimmunity. To evaluate the blood and cerebrospinal fluid ...

Several lines of evidence exist which suggest that changes in the expression of circulating cytokines are linked to the development or reoccurrence of multiple sclerosis (MS). This study aimed to evaluate the serum levels of relevant cytokines after therapy with IFN-β formulations in MS patients. In this study, blood samples ...

A new amyotrophic lateral sclerosis (ALS) category named 'UMN-dominant ALS' and defined as 'due predominantly to UMN signs but with minor electromyogram (EMG) denervation or LMN signs on examination' has been proposed. The clinical and laboratory features of 20 patients with UMN-dominant ALS are described here, their disease course is ...

OBJECTIVE: To determine the reproducibility of 24-hour monitoring of motor activity in patients with multiple sclerosis (MS). DESIGN: Test-retest design; 6 research assistants visited the participants twice within 1 week in the home situation. SETTING: General community. PARTICIPANTS: A convenience sample of ambulatory patients (N=43; mean age ± SD, 48.7±7.0y; ...

Chronic cerebrospinal venous insufficiency (CCSVI) has been suggested to be a possible cause of multiple sclerosis (MS). If the presumed mechanism of venous stasis-related parenchymal iron deposition and neurodegeneration were true, then upregulation of intrathecal iron transport proteins may be expected. This was a cross-sectional (n = 1,408) and longitudinal ...

The objective of the study was to assess the natalizumab effect on the course of cognitive impairment in patients with relapsing-remitting multiple sclerosis (MS). Patients with active relapsing-remitting MS (n = 17) were treated with natalizumab for 1 year. The quasi control group included patients (n = 7) with clinically stable MS. Assessment of disease ...

Prevalence of metabolic syndrome (MS) in obstructive sleep apnea (OSA) patients is high. The effect of autoadjusting positive airway pressure (APAP) on MS remains unclear. This study aimed to determine the prevalence of MS in OSA patients before and 6 months after APAP, and to identify potential determinants of metabolic status ...

The association between epilepsy and multiple sclerosis (MS) is not a coincidence. Our objective was to compare MS patients with or without history of seizures. In a population of 5041 MS patients, we identified 102 (2%) patients with epileptic seizures. In 67 patients (1.3%), epileptic seizure could not be explained ...

Despite similarities, neuromyelitis optica (NMO) can be distinguished from multiple sclerosis (MS) by clinical, radiological and serological findings. This case-control study aimed to determine whether patients with NMO or with MS in an Afro-Caribbean population originating from French West Indies shared the same or different HLA class I and II ...

Our objective was to characterize the clinical and radiologic features of Korean pediatric patients with relapsing central nervous system (CNS) demyelination disease. Twenty-one patients with relapsing CNS demyelinating events were classified as having multiple sclerosis (MS, 18 patients) or neuromyelitis optica (NMO, three patients) according to the international consensus definitions. ...

The causes of incomplete remyelination in progressive multiple sclerosis are unknown, as are the pathological correlates of the different clinical characteristics of patients with primary and secondary progressive disease. We analysed brains and spinal cords from 51 patients with progressive multiple sclerosis by planimetry. Thirteen patients with primary progressive disease ...

Antibodies to aquaporin-4 (AQP4-Ab) are found in 60-80% of patients with neuromyelitis optica (NMO), a severely disabling inflammatory CNS disorder of putative autoimmune aetiology, which predominantly affects the optic nerves and spinal cord. To assess the frequency of AQP4-Ab in patients with optic neuritis (ON), and to investigate the prognostic ...

Epidermal nevi are hamartomas arising from the embryonic ectoderm during early embryonic stages, resulting in cutaneous mosaicism. We describe a patient with diffuse epidermal nevi and multiple large pilomatricomas arising near or within epidermal nevi.

Background: Mitoxantrone (MTX) is an immunosuppressive drug approved for multiple sclerosis (MS) treatment. Objective: The aim of this study is to evaluate and to compare the clinical and neuroradiological responses to MTX in relapsing-remitting (RR) and secondary progressive (SP) MS patients. Methods: We conducted a retrospective, non-randomized, open-label, observational study ...

To evaluate antibodies to myelin oligodendrocyte glycoprotein (MOG) in the serum and cerebrospinal fluid (CSF) of patients with multiple sclerosis (MS) and control individuals. Prospective case-control series. Academic referral center. Twenty-six controls with noninflammatory neurologic disease and 35 patients with MS donated serum and CSF for recombinant MOG (rMOG) antibody ...

Meige syndrome (MS) is an adult-onset segmental dystonia characterized by the combination of upper and lower cranial involvement. Its treatment is challenging and the use of oral medication or of botulinum neurotoxin treatment is not decisive. Deep brain stimulation of the globus pallidus internum (GPi DBS) has been used occasionally ...

Annunziata P, De Santi L, Di Rezze S, Millefiorini E, Capello E, Mancardi G, De Riz M, Scarpini E, Vecchio R, Patti F. Clinical features of Sjogren's syndrome in patients with multiple sclerosis. Acta Neurol Scand: 2011: 124: 109-114. © 2010 John Wiley & Sons A/S. Objectives -  To assess the ...

Objective: To investigate whether cognitive impairment in multiple sclerosis (MS) patients is associated to different patterns of gray matter (GM) atrophy and T2-visible lesion distribution according to the clinical phenotype. Experimental Design: Twenty-two relapsing remitting (RR), 29 secondary progressive (SP), and 22 primary progressive (PP) MS patients, and 39 healthy ...

BACKGROUND: The presence of cortical lesions (CLs) and their topographic distribution in the brains of patients with multiple sclerosis (MS) have been clearly shown by recent histopathologic studies. CLs can also be assessed in vivo, with less sensitivity, by using specific MRI sequences. MRI-based lesion probability maps (LPMs) may partially ...

Short echo time localized proton magnetic resonance spectroscopy provides quantification of brain metabolites, including N-acetyl-aspartate, myo-inositol, creatine/phosphocreatine and choline-containing compounds, which may be useful biomarkers for monitoring Alzheimer's disease. We aimed to quantify the rate of metabolite change in Alzheimer's disease, to assess factors influencing changes and to investigate the ...

Acquired neuromyotonia encompasses a group of inflammatory disorders characterized by symptoms reflecting peripheral nerve hyperexcitability, which may be clinically confused in the early stages with amyotrophic lateral sclerosis. Despite a clear peripheral nerve focus, it remains unclear whether the ectopic activity in acquired neuromyotonia receives a central contribution. To clarify ...

Objective:  To evaluate the efficacy of botulinum neurotoxin type A (BoNT/A) for severe oro-pharyngeal dysphagia associated with multiple sclerosis (MS). Patients and methods:  BoNT/A was injected percutaneously into the hyperactive cricopharyngeal muscle of 14 dysphagic MS patients under electromyographic control. Patients were evaluated by videofluoroscopic and electromyographic examinations and by ...

We explore the safety, and therapeutic benefit of intrathecal injection of ex-vivo expanded autologous bone marrow derived mesenchymal stem cells (BM-MSCs) in 10 patients with advanced multiple sclerosis (MS). Patients were assessed at 3, 6 and 12 months. Assessment at 3-6 months revealed Expanded Disability Scale Score (EDSS) improvement in ...

Objective: To assess longitudinally cognitive functioning in relapsing-remitting multiple sclerosis (RRMS) patients and its relationship with clinical and MRI variables. Methods: Early RRMS patients and matched healthy controls were assessed in parallel in three testing sessions over 3 years, using the Rao's Brief Repeatable Battery of Neuropsychological Tests. Patients also ...

Chemokines are involved in the migration of inflammatory cells to the central nervous system in multiple sclerosis (MS). The aim of our study was to estimate the concentrations of CCL5, CXCL10 and CXCL11 in serum and cerebrospinal fluid (CSF) samples of relapsing-remitting MS (RRMS) patients during both relapse and stable ...