Spontaneous development of myasthenia gravis and myositis following treatment with pembrolizumab: a case report.

Background: Immune checkpoint inhibitors are a relatively new advancement in the world of cancer therapy. As such, their adverse effects have yet to be fully understood, with only recent literature documenting autoimmune phenomena secondary to their utilization. Specific immune checkpoint inhibitors have recently been linked with the development of myasthenia gravis, which is classically known to manifest spontaneously in patients.

Two Cases of Sporadic Amyotrophic Lateral Sclerosis With Contrasting Clinical Phenotypes: Genetic Insights.

Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease that affects individuals of diverse racial and ethnic backgrounds. There is currently no cure for ALS, and the number of efficient disease-modifying drugs for ALS is limited to a few, despite the large number of clinical trials conducted in recent years. The latter could be attributed to the significant heterogeneity of ALS clinical phenotypes even in their familial forms.

The natural history of ALS: Baseline characteristics from a multicenter clinical cohort.

Background: Amyotrophic lateral sclerosis (ALS) is a rare disease with urgent need for improved treatment. Despite the acceleration of research in recent years, there is a need to understand the full natural history of the disease. As only 40% of people living with ALS are eligible for typical clinical trials, clinical trial datasets may not generalize to the full ALS population.

Prodromal depression and subsequent risk of developing Parkinson's disease: a systematic review with meta-analysis.

Parkinson's disease (PD) is a progressive neurological disorder that predominately affects dopaminergic neurons. We believe that this pooling of data will help to better understand the prodromal nature of depression in PD. We conducted this study in accordance with PRISMA guidelines 2020.

Electrodiagnostic Assessment of Uncommon Mononeuropathies.

Carpal tunnel syndrome, ulnar neuropathy at the elbow, and peroneal neuropathy are the most common mononeuropathies; however, other individual nerves may also be injured by various processes. These uncommon mononeuropathies may be less readily diagnosed owing to unfamiliarity with the presentations and vague symptoms. Electrodiagnostic studies are essential in the evaluation of uncommon mononeuropathies and can assist in localization and prognostication.

Patient Assisted Intervention for Neuropathy: Comparison of Treatment in Real Life Situations (PAIN-CONTRoLS): Bayesian Adaptive Comparative Effectiveness Randomized Trial.

Importance: Cryptogenic sensory polyneuropathy (CSPN) is a common generalized slowly progressive neuropathy, second in prevalence only to diabetic neuropathy. Most patients with CSPN have significant pain. Many medications have been tried for pain reduction in CSPN, including antiepileptics, antidepressants, and sodium channel blockers.

Primary lateral sclerosis (PLS) functional rating scale: PLS-specific clinimetric scale.

Introduction: Our research aim was to develop a novel clinimetric scale sensitive enough to detect disease progression in primary lateral sclerosis (PLS).

Methods: A prototype of the PLS Functional Rating Scale (PLSFRS) was generated. Seventy-seven participants with PLS were enrolled and evaluated at 21 sites that comprised the PLSFRS study group.

Prevalence of Axonal Sensory Neuropathy With IgM Binding to Trisulfated Heparin Disaccharide in Patients With Fibromyalgia.

Objective: To assess the intraepidermal nerve fiber density in patients diagnosed with fibromyalgia (FM) and to evaluate the role of IgM binding to trisulfated heparin disaccharide (TS-HDS) in these patients.

Methods: FM is a poorly understood pain disorder with several proposed pathophysiologic mechanisms. It is characterized by widespread pain, fatigue, and sleep abnormalities.

Rasagiline for amyotrophic lateral sclerosis: A randomized, controlled trial.

Introduction: Rasagiline is a monoamine oxidase B (MAO-B) inhibitor with possible neuroprotective effects in patients with amyotrophic lateral sclerosis (ALS).

Methods: We performed a randomized, double-blind, placebo-controlled trial of 80 ALS participants with enrichment of the placebo group with historical controls (n = 177) at 10 centers in the United States. Participants were randomized in a 3:1 ratio to 2 mg/day rasagiline or placebo.

Creutzfeldt-Jakob Disease: Analysis of Four Cases.

Background: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive neurodegenerative disease that almost always results in death in under a year from onset of symptoms. Here, we report four cases of CJD with different clinical presentations diagnosed at our institution over a 2-year period.

Cases: The first patient is an 82-year-old woman who presented with depression, cognitive decline, and word-finding difficulty over 4 weeks.

Idiopathic Inflammatory Myopathies: Clinical Approach and Management.

Idiopathic inflammatory myopathies (IIM) are a group of chronic, autoimmune conditions affecting primarily the proximal muscles. The most common types are dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with sub-acute to chronic onset of proximal weakness manifested by difficulty with rising from a chair, climbing stairs, lifting objects, and combing hair.

Chronic herpes simplex type-1 encephalitis with intractable epilepsy in an immunosuppressed patient.

Background: Chronic herpes simplex virus type-1 encephalitis (HSE-1) is uncommon. Past reports focused on its association with prior documented acute infection. Here, we describe a patient with increasingly intractable epilepsy from chronic HSE-1 reactivation without history of acute central nervous system infection.

A role for functional classification in the early identification of prognostic factors in ALS.

The aim of this study was to determine if the interval between onset of symptoms to initial electrodiagnostic studies indicates disease progression in amyotrophic lateral sclerosis (ALS). Fifty consecutive patients referred to our neurophysiology laboratory with clinical evidence of ALS were divided into two groups by outcome scores on the ALS Functional Rating Scale (ALSFRS) using 26 as a cut-off. Our results, which showed a median of four months (range 2-24 months) duration to initial electrodiagnostics for Group I (ALSFRS scores below 26) versus 10 months (range 1-24 months) for Group II (p = 0.

Cauda equina and conus medullaris syndrome in sarcoidosis.

Background: Neurosarcoidosis is a rare manifestation of sarcoidosis. Involvement of the nervous system in sarcoidosis can range from peripheral or cranial neuropathy to central nervous system disease. Cauda equina sarcoidosis is distinctly rare.

Static magnetic field therapy for symptomatic diabetic neuropathy: a randomized, double-blind, placebo-controlled trial.

Objective: To determine if constant wearing of multipolar, static magnetic (450G) shoe insoles can reduce neuropathic pain and quality of life (QOL) scores in symptomatic diabetic peripheral neuropathy (DPN).

Design: Randomized, placebo-control, parallel study.

Setting: Forty-eight centers in 27 states.