Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful Diagnosis

Abstract

Neuromuscular junction disorders (NJDs) are a heterogeneous group of diseases including myasthenia gravis (MG). In some cases, patients are present with myasthenic symptoms without evidence of autoimmune antibodies, making diagnosis challenging. Total plasma exchange (TPE) has proven efficacy in NJDs. The objective is to describe the safety and efficacy of TPE in NJD patients with questionable disease activity or uncertain diagnosis in order to assess the diagnostic potential of TPE. We report an observational, retrospective cohort study of clinical routine data. All the data were derived from the electronic medical records of the Department of Neurology at University Hospital Essen. We searched for patients with NJDs between 1 July 2018 and 30 June 2021. Of the 303 patients who presented to the department with NJDs, 20 were treated with TPE; 9 patients did not show a measurable benefit from TPE (45%), 6 of whom were diagnosed with seronegative MG. Of these, 3 (50%) had long-standing ocular symptoms. There were decreases in the mean arterial pressure, hemoglobin, hematocrit and fibrinogen during treatment, which were not considered clinically relevant. In (seronegative) myasthenic patients, TPE may help to verify an uncertain diagnosis or to reveal possible muscle damage, allowing unnecessary therapy to be avoided.

Post-Intensive Care Syndrome after Critical Illness: An Imperative for Effective Prevention

“…While the prevalence of ICU-acquired weakness did not differ between male and female sex, female sex was associated with a significantly reduced insulin sensitivity index and lower myocyte cross-sectional area. In another study on neurological disorders that may contribute to physical impairments after ICU discharge, Totzeck et al explored the safety and diagnostic potential of total plasma exchange among 20 ICU patients with neuromuscular junction disorders with uncertain disease activity or diagnosis [ 27 ]. To identify patients prone to long-term physical impairment, Moayed et al developed a prediction model for physical disability after discharge in Iranian ICU patients [ 28 ].…”