AANEM News Express

AANEM News Express

Science News: Diagnostic Utility of Gold Coast Criteria in Amyotrophic Lateral Sclerosis

Existing diagnostic criteria for amyotrophic lateral sclerosis (ALS) such as El Escorial criteria (rEEC) and Awaji criteria have certain limitations, particularly for early disease or atypical presentations of ALS. A new diagnostic criterion has been proposed, with the goal of making it simpler to implement and include atypical presentations.

Science News: Charcot-Marie-Tooth Disease Misdiagnosed as a Chronic Inflammatory Demyelinating Polyradiculoneuropathy: An International Multicentric Retrospective Study

Charcot–Marie–Tooth (CMT) disease, a common hereditary polyneuropathy, has no treatment besides symptomatic relief. Authors performed a large retrospective study in 16 university hospitals in three European countries (France, Belgium, and Switzerland). Among 1,104 chronic inflammatory demyelinating polyneuropathy (CIDP) cases, there were 35 CMT patients (3.2%) misdiagnosed as CIDP.

Science News: Assessing Diagnostic and Severity Grading Accuracy of Ultrasound Measurements for Carpal Tunnel Syndrome Compared to Electrodiagnostics

Prospective study of 95, 18-80 y.o. patients referred for EDX of idiopathic CTS. Exclusion criteria for prior CTS and other concomitant confounding factors.  CSI performed and Bland criteria used to assess for severity.

Science News: Effect of High-Frequency (10-kHz) Spinal Cord Stimulation in Patients With Painful Diabetic Neuropathy: A Randomized Clinical Trial

Nevro's SENZA-PDN study, RCT of spinal cord stimulation treatment conducted for PDN-painful diabetic polyneuropathy, compares 10 kHz SCS plus Conventional Medical Management (CMM) to CMM alone in 216 patients at 18 centers in the United States.

Science News: Skeletal Muscle and Peripheral Nerve Histopathology in COVID-19

This study discusses spectrum of skeletal muscle and nerve pathology of patients who died following SARS-CoV-2 infection.
Psoas muscle and femoral nerve sampled from 35 consecutive autopsies of patients who died following SARS-CoV-2 infection were collected and analyzed.

Science News: Impact of Coronavirus Disease 2019 in a French Cohort of Myasthenia Gravis

Among 3,558 patients with MG registered in the French database for rare disorders, 34 (0.96%) had COVID-19. The mean age at COVID-19 onset was 55.0 ± 19.9 years (mean MG duration: 8.5 ± 8.5 years). By the end of the study period, 28 patients recovered from COVID-19, 1 remained affected, and 5 died.

Science News: Childhood Amyotrophic Lateral Sclerosis Caused by Excess Sphingolipid Synthesis

ALS is a devastating disease with poorly understood underlying pathophysiology. This article describes a rare cause of the early onset of motor neuron disease in children due to mutation in SPLTC1, a subunit of the SPT (serine palmitoyltransferase).

Science News: Epidemiology and Natural History of Inclusion Body Myositis A 40-Year Population-Based Study

This study determines the prevalence and natural history of sporadic inclusion body myositis (sIBM) and tests the hypothesis that patients with sIBM have higher cancer or mortality rates than the general population. The study identified 20 patients (10 clinicopathologically defined, 9 clinically defined, and 1 probable) according to the ENMC criteria and 1 patient with all features of clinicopathologically defined sIBM except for symptom onset at <45 years of age. The prevalence of sIBM in 2010 was 18.20 per 100,000 people ≥50 years old.

Patient Safety: NM Complications of Immune Checkpoint Inhibitors

Immune checkpoint inhibitors (ICPI’s) are monoclonal antibodies that target the immune checkpoints programmed death 1 (PD1), programmed death ligand 1 (PDL-1) and cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) on T cells and promote immune mediated destruction of cancer cells. They are used to treat a variety of cancers including breast, bladder, cervical, colon, lung cancer, Hodgkin’s lymphoma, melanoma, etc. ICPI treatment is associated with an array of immune related adverse events affecting many organ systems. Neurologic adverse events have also been reported.

Science News: Acute Flaccid Myelitis: Long-Term Outcomes Recorded in the CAPTURE Study Compared With Paediatric Transverse Myelitis

This is a prospective, multicenter, non-randomized, observational cohort study of children with myelitis seen at academic centers in North America. The study lasted 5 years and the length of follow-up was 1 year. Pediatric patients diagnosed with myelitis were eligible for enrollment in the study within 6 months of onset of symptoms. Patients were classified as transverse myelitis (TM) or the acute flaccid myelitis (AFM) variant based on clinical and radiographic findings.