Pain in NMOSD and MOGAD: A Systematic Literature Review of Pathophysiology, Symptoms, and Current Treatment Strategies.

Susanna Asseyer,Graham Cooper,Friedemann Paul

doi:10.3389/fneur.2020.00778

Abstract

Neuromyelitis optica spectrum disorders (NMOSDs) and myelin oligodendrocyte glycoprotein-antibody-associated disease (MOGAD) are autoimmune inflammatory disorders of the central nervous system (CNS). Pain is highly prevalent and debilitating in NMOSD and MOGAD with a severe impact on quality of life, and there is a critical need for further studies to successfully treat and manage pain in these rare disorders. In NMOSD, pain has a prevalence of over 80%, and pain syndromes include neuropathic, nociceptive, and mixed pain, which can emerge in acute relapse or become chronic during the disease course. The impact of pain in MOGAD has only recently received increased attention, with an estimated prevalence of over 70%. These patients typically experience not only severe headache, retrobulbar pain, and/or pain on eye movement in optic neuritis but also neuropathic and nociceptive pain. Given the high relevance of pain in MOGAD and NMOSD, this article provides a systematic review of the current literature pertaining to pain in both disorders, focusing on the etiology of their respective pain syndromes and their pathophysiological background. Acknowledging the challenge and complexity of diagnosing pain, we also provide a mechanism-based classification of NMOSD- and MOGAD-related pain syndromes and summarize current treatment strategies.

Highlights

In 1894, Eugène Devic (1858–1930) and his doctoral student Fernand Gault (1873–1936) reported a historical case on a patient with optic neuritis (ON) and myelitis and proposed the name “neuromyélite optique” for this syndrome
Two reviews on pain in neuromyelitis optica spectrum disorder (NMOSD) are available, one focusing on potential mechanisms underlying the pathogenesis of pain in NMOSD and another focusing on the impact of neuropathic pain medication on patients’ quality of life [3, 57]
Pain is a very frequent symptom in NMOSD and myelin oligodendrocyte glycoprotein-antibody-associated disease (MOGAD) and has a prevalence of over 80% with a severe impact on the quality of life of affected patients

Summary

Introduction

In 1894, Eugène Devic (1858–1930) and his doctoral student Fernand Gault (1873–1936) reported a historical case on a patient with optic neuritis (ON) and myelitis and proposed the name “neuromyélite optique” for this syndrome. The patient, a 45-year-old woman, was admitted for suspected “neurasthenia,” suffering from disturbed sleep, gastrointestinal symptoms, neuromuscular asthenia, palpitations, and, especially, headache: “The pain occurs in attacks, both during the day and night. The patient suddenly developed acute complete paraparesis and visual loss. It is currently a matter of debate whether the patient suffered from a neuromyelitis optica spectrum disorder (NMOSD) or a myelin oligodendrocyte glycoprotein-antibody-associated disease (MOGAD) [1]. Terrible, agonizing, and unbearable pain can arise as an acute or chronic symptom in both pathologies [2,3,4] (Table 1)

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