MOGAD is another inflammatory autoimmune condition of the CNS, defined by IgG antibodies against conformationally intact myelin oligodendrocyte glycoprotein (MOG) localized on the surface of the myelin sheaths ( 13, 33, 34).
Neuropathic pain is the most common type of chronic pain with a prevalence of up to over 80% ( 2, 26), and painful tonic spasms occur with a prevalence of 25–40% ( 29– 32). Over 50% of NMOSD (82% APQ4-Ab positive) patients recalled an increase in pain intensity as the first indicator of a relapse ( 26) and 25% of patients with NMOSD (82% AQP4-Ab positive) reported pain as their worst symptom, despite also experiencing severe weakness and bladder or bowel dysfunction ( 26). Chronic pain occurs in NMOSD with an estimated prevalence between 72 and 86% ( 2, 18, 27, 28). Patients typically suffer from recurrent attacks of severe optic neuritis and/or myelitis ( 13, 14) and, less frequently, brainstem or brain involvement ( 15, 16), leading to a diverse range of symptoms, of which severe pain is one of the most frequent and disabling ( 2, 17– 26). In the majority of cases, NMOSDs are associated with serum immunoglobulin G (IgG) autoantibodies (Abs) targeting the astrocyte aquaporin-4 (AQP4) water channel ( 11, 12). Neuromyelitis optica spectrum disorders (NMOSDs) are rare and, in most cases, relapsing inflammatory diseases of the central nervous system (CNS) ( 10). Terrible, agonizing, and unbearable pain can arise as an acute or chronic symptom in both pathologies ( 2– 4) ( Table 1). 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). One month after admission, the patient suddenly developed acute complete paraparesis and visual loss. The pain is sometimes so strong that it causes the patient to cry.”
Pain attacks may be long or short, affecting one side of the face and the head, sometimes the right side, mostly the left, but the highest intensity is always at the occipital region: the neck and eyeballs. 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. 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 “neuro-myélite optique” for this syndrome. 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. 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. 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. The impact of pain in MOGAD has only recently received increased attention, with an estimated prevalence of over 70%. 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. 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. Neuromyelitis optica spectrum disorders (NMOSDs) and myelin oligodendrocyte glycoprotein-antibody-associated disease (MOGAD) are autoimmune inflammatory disorders of the central nervous system (CNS). 4Department of Neurology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Berlin Institute of Health, Humboldt-Universität zu Berlin, Berlin, Germany.3Einstein Center for Neurosciences, Berlin, Germany.2NeuroCure Clinical Research Center, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Berlin Institute of Health, Humboldt-Universität zu Berlin, Berlin, Germany.1Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine and Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Berlin Institute of Health, Humboldt-Universität zu Berlin, Berlin, Germany.Susanna Asseyer 1,2 Graham Cooper 1,2,3 Friedemann Paul 1,2,3,4 * †
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