key: cord-0803759-0oosydgi authors: Li, Xiaoyan; Bedlack, Richard title: COVID‐19–accelerated disease progression in two patients with amyotrophic lateral sclerosis date: 2021-06-23 journal: Muscle Nerve DOI: 10.1002/mus.27351 sha: c2a3774b8b884e3c230ca3a703285d0531024bcd doc_id: 803759 cord_uid: 0oosydgi nan We report two patients with slowly progressive amyotrophic lateral sclerosis (ALS) who experienced rapid functional decline after contracting severe acute respiratory virus-coronavirus-2 (SARS-CoV-2). A 78-year-old man developed proximal leg weakness in 2014 and was diagnosed with probable ALS based on El Escorial criteria 1 His revised ALS Functional Rating Scale (ALSFRS-R) assessment, administered by physicians, showed scoring that progressed at an unusually slow rate since that time ( Figure 1A ). In December 2020, the patient developed a fever, and SARS-CoV-2 viral polymerase chain reaction of the nasopharyngeal swab was positive. One week later, he presented to an emergency department with fever, shortness of breath, and delirium. Computed tomography scan of the chest revealed signs of pneumonia. He also developed deep venous thrombosis in the left femoral vein but did not have a pulmonary embolism. The patient was hospitalized and treated with We confirmed that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions. A patient with distal lower extremity neuropathic pain and anti-contactin-associated protein-2 antibodies The spectrum of antibody-related painful neuropathies is increasing. 1 We report a patient with an anti-contactin-associated protein-2 (CASPR2) antibody-associated painful syndrome, providing new insights into the pain of anti-CASPR2 associated disorders. The diagnosis of anti-CASPR2 antibody-associated painful disorder was challenging in this patient, who did not have a suggestive syndrome. 3 Pain and pinprick hypoesthesia in a peripheral distribution suggested small nerve fiber dysfunction. The absence of recordable LEPs supported nociceptive pathway involvement. The patient did not fulfill the current criteria of SFN based on the IENFD and/or quantitative sensory testing (QST), 2 but those criteria are controversial. 4 Considering the clinical signs and symptoms, the normal EDX, and the absent LEPs, we believe the clinical painful picture was consistent with small nerve fiber involvement. In this context, the presence of anti-CASPR2 immunostaining on Aδ-fibers juxtaparanodes and C-fibers was meaningful. CASPR2 is a neuronal cell protein expressed in dorsal root ganglia neurons where it regulates sensory neuronal excitability. 5 CASPR2 forms a protein complex with the voltage-gated potassium Duplexes of 21-nucleotide RNAs mediate RNA interference in cultured mammalian cells Preclinical evaluation of RNAi as a treatment for transthyretin-mediated amyloidosis an investigational RNAi therapeutic for patients with hereditary transthyretinmediated (hATTR) amyloidosis with polyneuropathy: results from the phase 3 APOLLO study The biological and chemical basis for tissue-selective amyloid disease Genetic microheterogeneity of human transthyretin detected by IEF Amyloid fibril protein in familial amyloidotic polyneuropathy, Portuguese type. Definition of molecular abnormality in transthyretin (prealbumin) Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain-Barre syndrome Inflammatory Neuropathy Cause and Treatment (INCAT) group. Clinimetric evaluation of a new overall disability scale in immune mediated polyneuropathies Rasch-built Overall Disability Scale (R-ODS) for immune-mediated peripheral neuropathies World Federation of Neurology Research Group on Motor Neuron Diseases El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis Increased worsening of amyotrophic lateral sclerosis patients during Covid-19-related lockdown in France Steroid-responsive encephalitis in coronavirus disease 2019