Response to eculizumab in patients with myasthenia gravis recently treated with chronic IVIg: a subgroup analysis of REGAIN and its open-label extension study.

Background: In the phase III eculizumab for refractory generalized myasthenia gravis REGAIN study [ClinicalTrials.gov identifier: NCT01997229] and its open-label extension (OLE) [ClinicalTrials.gov identifier: NCT02301624], patients with treatment-refractory antiacetylcholine receptor antibody-positive generalized myasthenia gravis had clinically meaningful improvements with eculizumab placebo.

'Minimal symptom expression' in patients with acetylcholine receptor antibody-positive refractory generalized myasthenia gravis treated with eculizumab.

Background: The efficacy and tolerability of eculizumab were assessed in REGAIN, a 26-week, phase 3, randomized, double-blind, placebo-controlled study in anti-acetylcholine receptor antibody-positive (AChR+) refractory generalized myasthenia gravis (gMG), and its open-label extension.

Methods: Attainment of 'minimal symptom expression' was evaluated using patient-reported outcome measures of gMG symptoms [MG activities of daily living scale (MG-ADL), 15-item MG quality of life questionnaire (MG-QOL15)] at the completion of REGAIN and during the open-label extension. 'Minimal symptom expression' was defined as MG-ADL total score of 0-1 or MG-QOL15 total score of 0-3.

Human hippocampal CA3 damage disrupts both recent and remote episodic memories.

Neocortical-hippocampal interactions support new episodic (event) memories, but there is conflicting evidence about the dependence of remote episodic memories on the hippocampus. In line with systems consolidation and computational theories of episodic memory, evidence from model organisms suggests that the cornu ammonis 3 (CA3) hippocampal subfield supports recent, but not remote, episodic retrieval. In this study, we demonstrated that recent and remote memories were susceptible to a loss of episodic detail in human participants with focal bilateral damage to CA3.

Long-term efficacy and safety of eculizumab in Japanese patients with generalized myasthenia gravis: A subgroup analysis of the REGAIN open-label extension study.

The terminal complement inhibitor eculizumab was shown to improve myasthenia gravis-related symptoms in the 26-week, phase 3, randomized, double-blind, placebo-controlled REGAIN study (NCT01997229). In this 52-week sub-analysis of the open-label extension of REGAIN (NCT02301624), eculizumab's efficacy and safety were assessed in 11 Japanese and 88 Caucasian patients with anti-acetylcholine receptor antibody-positive refractory generalized myasthenia gravis. For patients who had received placebo during REGAIN, treatment with open-label eculizumab resulted in generally similar outcomes in the Japanese and Caucasian populations.

Long-term safety and efficacy of eculizumab in generalized myasthenia gravis.

Introduction: Eculizumab is effective and well tolerated in patients with antiacetylcholine receptor antibody-positive refractory generalized myasthenia gravis (gMG; REGAIN; NCT01997229). We report an interim analysis of an open-label extension of REGAIN, evaluating eculizumab's long-term safety and efficacy.

Methods: Eculizumab (1,200 mg every 2 weeks for 22.

Distinct HLA associations of LGI1 and CASPR2-antibody diseases.

The recent biochemical distinction between antibodies against leucine-rich, glioma-inactivated-1 (LGI1), contactin-associated protein-2 (CASPR2) and intracellular epitopes of voltage-gated potassium-channels (VGKCs) demands aetiological explanations. Given established associations between human leucocyte antigen (HLA) alleles and adverse drug reactions, and our clinical observation of frequent adverse drugs reactions in patients with LGI1 antibodies, we compared HLA alleles between healthy controls (n = 5553) and 111 Caucasian patients with VGKC-complex autoantibodies. In patients with LGI1 antibodies (n = 68), HLA-DRB1*07:01 was strongly represented [odds ratio = 27.

Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study.

Background: Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial.

Focal CA3 hippocampal subfield atrophy following LGI1 VGKC-complex antibody limbic encephalitis.

Magnetic resonance imaging has linked chronic voltage-gated potassium channel (VGKC) complex antibody-mediated limbic encephalitis with generalized hippocampal atrophy. However, autoantibodies bind to specific rodent hippocampal subfields. Here, human hippocampal subfield (subiculum, cornu ammonis 1-3, and dentate gyrus) targets of immunomodulation-treated LGI1 VGKC-complex antibody-mediated limbic encephalitis were investigated using in vivo ultra-high resolution (0.

New insights into the management of chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and its variants can be challenging to diagnose and treat. A combination of clinical, electrophysiological and laboratory features is often required to reach a diagnosis. New data are emerging about potential biomarkers and factors that may indicate treatment needs in individual patients.

Guillain-Barré syndrome in Arab countries: a systematic review.

Systematic review of the frequency and clinical pattern of Guillain-Barré syndrome (GBS) in Arab countries was initiated by a keyword search of PubMed, Medline and Embase and examination of references in all relevant papers. Seven articles were included from Iraq (n=1), Kuwait (n=1), Libya (n=2), and Saudi Arabia (n=3). The only incidence report from the Arab world, a 1987 study from Libya, gives an incidence of 1.

Long-term efficacy, tolerability and retention rate of azathioprine in 103 aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder patients: a multicentre retrospective observational study from the UK.

Background: Azathioprine (AZA) is a common immunosuppressive drug used for relapse prevention in neuromyelitis optica (NMO).

Objectives: The objective of this paper is to assess efficacy, tolerability and retention of AZA in a large NMO cohort.

Methods: We conducted a retrospective review of medical records of 103 aquaporin-4 antibody-positive NMO and NMO spectrum disorder (NMOSD) patients treated with AZA.

Immunomodulation in inflammatory neuropathies: rationale and safety.

Inflammatory neuropathies are treatable immune-mediated disorders of the peripheral nerves, the prevalence of which is higher than previously believed. Although there are now established treatments of proven benefit for acute and chronic inflammatory neuropathies consisting of intravenous immunoglobulins, corticosteroids and plasma exchanges, there are patients who fail to respond to these therapies. Immunosuppressants have been used in these refractory patients and also to lower the requirements of therapies of established efficacy due to their cost (e.

General anaesthesia for deep brain stimulator electrode insertion in Parkinson's disease.

Background: This paper compares the use of general and local anaesthetic in patients having deep brain stimulator (DBS) surgery. It is a retrospective case note study of 46 patients treated consecutively with subthalamic nucleus stimulation for Parkinson's disease as practise changed in a Neurosurgical unit.

Methods: The first 20 patients (LA group) had permanent electrodes placed under local anaesthesia.

Vascular parkinsonism: a clinical review.

Over the last 75 years there has been continuous debate about the existence of vascular parkinsonism (VP). The condition has been named and renamed several times, with terms such as arteriosclerotic parkinsonism, arteriosclerotic pseudo-parkinsonism and lower-body parkinsonism. Despite the progress in our understanding of other parkinsonian syndromes, such as progressive supranuclear palsy and multiple-system atrophy, and significant developments in neuroimaging techniques, the concept of VP is still unclear and the clinical diagnosis is often difficult.

Parkinson's disease in Arabs: a systematic review.

Studies of specific populations have provided invaluable knowledge about Parkinson's disease (PD), especially in the field of genetics. The present report systematically reviews the medical literature on PD in Arabs. Medline and Embase were searched, and 24 article were identified: genetic (n = 17), epidemiological (n = 3), and clinical series (n = 5).

Neurological disorders in Libya: an overview.

The aim of this study was to review all publications related to the incidence and prevalence of neurological disorders in Libya, and to estimate the burden of these disorders in the country. The PubMed and the Libyan Medical Index were searched using different combinations of keywords. The references in all relevant papers were reviewed for any additional publications.

Parkinsonism and tremor disorders. A clinical approach.

Differentiation of idiopathic Parkinson's disease from other causes of Parkinsonism, such as Multiple System Atrophy, Progressive Supranuclar Palsy and Vascular Parkinsonism can be difficult. Clinicopathological studies suggest that the clinical diagnosis of idiopathic Parkinson's disease is 76% reliable. Also, clinical differentiation of tremor prominent Parkinsonism from Essential Tremor or Drug induced Parkinsonism may be problematic, especially in the early stages of the disease.

Adult-onset ataxia telangiectasia due to ATM 5762ins137 mutation homozygosity.

Ataxia telangiectasia (A-T) is an autosomal recessive neurodegenerative disorder that arises because of mutations in the ATM gene. The 5762ins137 A-->G point mutation activates a cryptic splice donor site resulting in a 137 bp intronic insert being aberrantly spliced into the ATM transcript. However, normal ATM transcript also is produced from this affected allele, albeit at significantly reduced levels.