Background: Amyotrophic lateral sclerosis (ALS) is a disabling and rapidly progressive neurodegenerative disorder. Increasing age is an important risk factor for developing ALS, thus the societal impact of this devastating disease will become more profound as the population ages. A significant hurdle to finding effective treatment has been an inability to accurately quantify cerebral degeneration associated with ALS in humans. Advanced magnetic resonance imaging (MRI) techniques hold promise in providing a set of biomarkers to assist in aiding diagnosis and in efficiently evaluating new drugs to treat ALS. Methods: The Canadian ALS Neuroimaging Consortium (CALSNIC) was founded to develop and evaluate advanced MRI-based biomarkers that delineate biological heterogeneity, track disease progression, and predict survival in a large and heterogeneous sample of ALS patients. Findings: CALSNIC has launched two studies to date (CALSINC-1, CALSNIC-2), acquiring multimodal neuroimaging, neurological, neuropsychological data, and neuropathological data from ALS patients and healthy controls in a prospective and longitudinal fashion from multiple centres in Canada and, more recently, the United States. Clinical and MRI protocols are harmonized across research centres and different MR vendors. Interpretation: CALSNIC provides a multicentre platform for studying ALS biology and developing MRI-based biomarkers. Funding: Canadian Institutes of Health Research, ALS Society of Canada, Brain Canada Foundation, Shelly Mrkonjic Research Fund
Neuromuscular disease in pregnancy is a broad topic and includes focal neuropathies that occur with increased incidence during pregnancy and the puerperium, as well as preexisting inherited neuropathies or myopathies and chronic autoimmune diseases such as myasthenia gravis and chronic inflammatory demyelinating polyneuropathy. Although the precise etiology of the focal neuropathies is not completely understood, they do seem to be a direct effect of pregnancy or delivery. In most cases, prognosis for complete recovery is good. The incidence of inherited and autoimmune neuromuscular diseases in women of childbearing age is relatively rare, and our knowledge of their impact in pregnancy is limited. Most are able to deliver healthy infants; however, these patients present specific challenges to the neurologist and obstetrician. The most recent literature on this topic is reviewed.
Immunotherapy has revolutionized cancer treatment over the past decade. As it is increasingly introduced into routine clinical practice, immune-related complications have become more frequent. Accurate diagnosis and treatment are essential, with the goal of reduced patient morbidity. This review aims to discuss the various clinical manifestations, diagnosis, treatments, and prognosis of neurologic complications associated with the use of immune checkpoint inhibitors, adoptive T-cell therapies, and T-cell redirecting therapies. We also outline a suggested clinical approach related to the clinical use of these agents.
Neuropathies are associated with several systemic diseases. This article provides a general overview of the clinical presentation and management of the various neuropathies associated with common medical illnesses. The term “neuropathy” is used relatively broadly to encompass pathology from the level of the cell body to the terminal axon. Peripheral nerve disorders associated with diabetes, thyroid disease, connective tissue disease, cancer, and infection are discussed. The relationship between the various neuropathies and systemic illness is important to recognize because neuropathy may be the initial presentation of one of these underlying medical conditions. Alternatively, the presence of a known associated medical condition in a patient presenting with neuropathy may affect management decisions and limit unnecessary investigations.
Background: Myasthenia gravis (MG) is frequently associated with thymic abnormalities, including thymomas and hyperplasia. This study aims to analyze the clinical and pathological characteristics of thymectomized patients over a 20-year period, focusing on the relationship between thymoma subtype and MG incidence, as well as post-thymectomy remission outcomes. Methods: We retrospectively analyzed 420 patients who underwent thymectomy (open or VATS), with a mean age of 54.4 years and 59% female. Thymic pathology included thymomas (56.2%), thymic cysts (14.3%), and other lesions. 39.5% of patients had MG, of which 48.8% were thymomatous MG. Multivariate regression was used to identify predictors of MG and remission outcomes. Results: MG was significantly associated with younger age (p < 0.005), germinal hyperplasia (p < 0.001), and thymoma, especially WHO B2 subtype (p = 0.016). Six-month complete remission rates did not differ between thymomatous and non-thymomatous MG. In the subgroup undergoing VATS, median length of stay decreased to 3 days compared to 5 days in the overall cohort. The intraoperative complication rate for VATS was 1.5%, compared to 11.6% for open surgery. Conclusions: This is one of the largest single-center studies to evaluate the link between thymoma histology and MG. WHO type B2 thymoma and germinal hyperplasia were more commonly associated with MG. Comparable remission outcomes support the role of thymectomy in both thymomatous and non-thymomatous MG, emphasizing the need for individualized surgical strategies.