Femoral and obturator neuropathies.

The femoral and obturator nerves both arise from the L2, L3, and L4 spinal nerve roots and descend into the pelvis before emerging in the lower limbs. The femoral nerve's primary function is knee extension and hip flexion, along with some sensory innervation to the leg. The obturator nerve's primary function is thigh adduction and sensory innervation to a small area of the medial thigh.

Radial neuropathy.

Radial neuropathy is the third most common upper limb mononeuropathy after median and ulnar neuropathies. Muscle weakness, particularly wrist drop, is the main clinical feature of most cases of radial neuropathy, and an understanding of the radial nerve's anatomy generally makes localizing the lesion straightforward. Electrodiagnosis can help confirm a diagnosis of radial neuropathy and may help with more precise localization of the lesion.

Education Research: Qualitative Assessment of Virtual Teaching of the Neurological Examination to Students Reveals Importance of Technique, Process, and Documentation.

Introduction: Virtual teaching sessions during the coronavirus disease 2019 pandemic were challenging for students and teachers but were also an opportunity to find creative ways to teach physical examination skills, including the neurologic examination. We examined expert opinions of the pros and cons that arise using a virtual platform to teach the neurologic examination and strategies to best address these challenges.

Methods: This was a qualitative study incorporating a focus group of faculty and resident neurologists.

Novel homozygous nonsense mutation of MLIP and compensatory alternative splicing.

Despite the growing accessibility of clinical sequencing, functional interpretation of variants remains a major hurdle to molecular diagnostics of Mendelian diseases. We aimed to describe a new adult-onset myopathy with muscle weakness and hyperCKemia caused by a nonsense variant in muscular LMNA-interacting protein (MLIP). Following RNA-sequencing, differential expression analysis uncovered a significant downregulation of this gene, which had a surprisingly mild effect on MLIP protein expression.

Minimal manifestation status and prednisone withdrawal in the MGTX trial.

Objective: To examine whether sustained minimal manifestation status (MMS) with complete withdrawal of prednisone is better achieved in thymectomized patients with myasthenia gravis (MG).

Methods: This study is a post hoc analysis of data from a randomized trial of thymectomy in MG (Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone Therapy [MGTX]). MGTX was a multicenter, randomized, rater-blinded 3-year trial that was followed by a voluntary 2-year extension for patients with acetylcholine receptor (AChR) antibody-positive MG without thymoma.

Poor Yield of Routine Transthyretin Screening in Patients with Idiopathic Neuropathy.

Background And Objectives: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is caused by a mutation in the transthyretin (TTR) gene. Although classically described as rapidly progressive and life-threatening, recent studies on TTR-FAP show significant genetic and phenotypic heterogeneity depending on geographic localization. In light of new therapeutic advances and their implication for patient management, the aim of our study was to determine the prevalence of TTR-FAP within patients with idiopathic neuropathy in a North American population.

Medical treatment for botulism.

Background: Botulism is an acute paralytic illness caused by a neurotoxin produced by Clostridium botulinum. Supportive care, including intensive care, is key, but the role of other medical treatments is unclear. This is an update of a review first published in 2011.

Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial.

Background: The Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone (MGTX) showed that thymectomy combined with prednisone was superior to prednisone alone in improving clinical status as measured by the Quantitative Myasthenia Gravis (QMG) score in patients with generalised non-thymomatous myasthenia gravis at 3 years. We investigated the long-term effects of thymectomy up to 5 years on clinical status, medication requirements, and adverse events.

Methods: We did a rater-blinded 2-year extension study at 36 centres in 15 countries for all patients who completed the randomised controlled MGTX and were willing to participate.

Autoantibodies to Low-Density Lipoprotein Receptor-Related Protein 4 in Double Seronegative Myasthenia Gravis: A Systematic Review.

Background: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction in which a clinical diagnosis may be confirmed with serological testing. The most common autoantibodies used to support a diagnosis of MG are anti-acetylcholine receptor antibodies and anti-muscle-specific tyrosine kinase antibodies. In cases in which both of these autoantibodies are negative (termed double-seronegative [dSNMG]), other autoantibodies such as low-density lipoprotein receptor-related protein 4 (LRP4) may be used to aid in diagnosis.

A New Mutation in FIG4 Causes a Severe Form of CMT4J Involving TRPV4 in the Pathogenic Cascade.

Mutations in FIG4, coding for a phosphoinositol(3,5) bisphosphate 5' phosphatase and involved in vesicular trafficking and fusion, have been shown causing a recessive form of Charcot-Marie-Tooth (CMT). We have identified a novel intronic mutation in the FIG4 in a wheel-chair bound patient presenting with a severe form of CMT4J and provide a longitudinal study. Investigations indicated a demyelinating sensorimotor polyneuropathy with diffuse active denervation and severe axonal loss.

Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews.

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic progressive or relapsing and remitting disease that usually causes weakness and sensory loss. The symptoms are due to autoimmune inflammation of peripheral nerves. CIPD affects about 2 to 3 per 100,000 of the population.

Randomized Trial of Thymectomy in Myasthenia Gravis.

Background: Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone.

Methods: We compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone.

Gain-of-function mutation in TRPV4 identified in patients with osteonecrosis of the femoral head.

Background: Osteonecrosis of the femoral head is a debilitating disease that involves impaired blood supply to the femoral head and leads to femoral head collapse.

Methods: We use whole-exome sequencing and Sanger sequencing to analyse a family with inherited osteonecrosis of the femoral head and fluorescent Ca(2+) imaging to functionally characterise the variant protein.

Results: We report a family with four siblings affected with inherited osteonecrosis of the femoral head and the identification of a c.

Treatment for cryoglobulinemic and non-cryoglobulinemic peripheral neuropathy associated with hepatitis C virus infection.

Background: Peripheral neuropathy is the most common neurologic complication of hepatitis C virus (HCV) infection. The pathophysiology of the neuropathy associated with HCV is not definitively known; however, proposed mechanisms include cryoglobulin deposition in the vasa nervorum and HCV-mediated vasculitis. The optimal treatment for HCV-related peripheral neuropathy has not been established.

Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study.

Background: Susac syndrome (SuS) is a rare disorder thought to be caused by autoimmune-mediated occlusions of microvessels in the brain, retina and inner ear leading to central nervous system (CNS) dysfunction, visual disturbances due to branch retinal artery occlusions (BRAO), and hearing deficits. Recently, a role for anti-endothelial cell antibodies (AECA) in SuS has been proposed.

Objectives: To report the clinical and paraclinical findings in the largest single series of patients so far and to investigate the frequency, titers, and clinical relevance of AECA in SuS.

Medical treatment for botulism.

Background: Botulism is an acute paralytic illness caused by a neurotoxin produced by Clostridium botulinum. Supportive care, including intensive care, is key but the role of other medical treatments is unclear. This is an update of a review first published in 2011.

'What would my classmates say?' An international study of the prediction-based method of course evaluation.

Objectives: Traditional student feedback questionnaires are imperfect course evaluation tools, largely because they generate low response rates and are susceptible to response bias. Preliminary research suggests that prediction-based methods of course evaluation - in which students estimate their peers' opinions rather than provide their own personal opinions - require significantly fewer respondents to achieve comparable results and are less subject to biasing influences. This international study seeks further support for the validity of these findings by investigating: (i) the performance of the prediction-based method, and (ii) its potential for bias.

Using simulation to train junior psychiatry residents to work with agitated patients: a pilot study.

Objective: This article examines the benefit and feasibility of introducing a new, simulation-based learning intervention for junior psychiatry residents.

Method: Junior psychiatry residents were invited to participate in a new simulation-based learning intervention focusing on agitated patients. Questionnaires were used to explore the success of the intervention.

Peer review: an effective approach to cultivating lecturing virtuosity.

Background: Most university faculty members are expected to teach. Many would benefit from instruction designed to improve lecturing.

Aims: To explore the impact of a program in which video-recorded lectures were critiqued by peers.