ABSTRACT
We evaluated 13 patients affected by myasthenia gravis (MG) who had coronavirus disease 2019 (COVID-19) before vaccination and 14 myasthenic patients who contracted severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection after vaccination to evaluate factors related to different COVID-19 outcomes. We compared the two groups' previous stability of MG and the severity of SARS-CoV-2 infection. Vaccinated and non-vaccinated patients were comparable in terms of severity of the previous MG course (mean maximum myasthenia gravis Foundation of America-MGFA-Class III) and during SARS-CoV-2 infection (mean MGFA Class II). In non-vaccinated patients, the hospitalization and severe course percentages were 61.5%, while the mortality reached 30.8%. The hospitalization, severe course, and mortality percentages in vaccinated patients were 7.1%. In deceased, non-vaccinated patients, greater myasthenia severity in the past clinical history, but not at the time of infection, was observed. Similarly, older age at MG onset and at the time of infection correlated with a more severe COVID-19 course in non-vaccinated patients (p = 0.03 and p = 0.04), but not in the group of vaccinated patients. In summary, our data support a protective role of vaccination in myasthenic patients, even if anti-CD20 therapy might be associated with a poor immune response to vaccines.
ABSTRACT
Background and Objectives: The Charcot-Marie-Tooth Examination Score (CMTES) has been used since 2005 in clinics to measure impairment in patients with CMT and has provided natural history data for patients with CMT1A, CMT1B, CMTX1, CMT2A, and many other subtypes. However, the CMTES requires an in-person visit, and many individuals are unable to travel to CMT centers because of the distance from the clinic or physical disability or more recently because of COVID-19 restrictions. We therefore developed the virtual CMTES (vCMTES) as outlined below. The aim of this study is to create a remote clinical outcome assessment to measure impairment in patients with CMT. Methods: We modified the CMTESv2 replacing the pinprick and vibration items with light touch and position sense, which can be performed remotely by the patient or the patient with an assistant while being observed by the clinic evaluator. Motor evaluations were performed similar to CMTESv2 by the assistant or the patient, while being observed remotely. We developed a standardized protocol to be used with Zoom or a similar format, a training and certification program, and enabled the vCMTES data to be housed in the Inherited Neuropathy Consortium databases. Patients were evaluated in person and remotely for interexaminer and intraexaminer studies. Results: Sixty-four patients with genetically confirmed CMT were evaluated by vCMTES and CMTESv2; 53 patients were evaluated virtually 3 weeks after their initial examination. Ten patients were evaluated with the vCMTES by different examiners 5 days apart. CMTESv2 correlates strongly with the vCMTES in person and virtually (p < 0.0001). There was a strong correlation between the vCMTES made in person and virtually (p < 0.0001). Similar results were obtained comparing symptoms score items, sensory items, and motor items. Interclass correlation coefficients (ICCs) were ≥0.92. Discussion: Statistical analyses demonstrated that the vCMTES was reproducible and reliable as a clinical outcome assessment for CMT. Further studies are needed to test responsiveness to change and progression in different subtypes. The vCMTES also offers the potential to reach diverse populations that do not have access to CMT centers.
ABSTRACT
We report, to the best of our knowledge, the first case of a probable COVID-19 infection in a 28-year-old man with Charcot-Marie-Tooth disease. The diagnosis was established through a remote interaction with the patient after early discharge from outpatient therapy due to upcoming traveling restrictions. The COVID-19 disease appeared mild, without major respiratory problems, and no obvious neuromuscular deterioration was reported or observed. Telerehabilitation provided an opportunity to continue with hand rehabilitation after tendon transfer surgery, perform an ad-hoc online evaluation, and advise the patient how to prevent the spread of infection and cope with restrictions limiting outpatient visits. This experience seems valuable for further development of telerehabilitation in anticipation of future pandemics or adversarial events since it allows reaching out to patients unable to travel and overcomes the need for regular outpatient visits.