Devic's index case: A critical reappraisal – AQP4-IgG-mediated neuromyelitis optica spectrum disorder, or rather MOG encephalomyelitis?

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

“…At the patient level, it can be defined as an "unusual tightening of muscles that feels like leg stiffness, jumping of legs, a repetitive bouncing of the foot, muscle cramping in the legs or arms, legs going out tight and straight or drawing up" (140). More than 50% of NMOSD patients are reported to suffer from moderate to severe spasticity (25), but very little is known about spasticity in MOGAD (1,38).…”

“…One month after admission, 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 (2-4) ( Table 1).…”

Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 1: Results from 163 lumbar punctures in 100 adult patients

“…Over the past few years, several studies using newgeneration cell-based assays (CBA) have demonstrated a robust association of immunoglobulin G (IgG) autoantibodies targeting full-length, conformationally intact human myelin oligodendrocyte glycoprotein (MOG) with (mostly recurrent) optic neuritis (ON), myelitis, and brainstem encephalitis, as well as with neuromyelitis optica (NMO)-like and acute-disseminated encephalomyelitis (ADEM)-like presentations, rather than with classic multiple sclerosis (MS) [1][2][3][4][5][6][7][8][9][10][11][12][13]. Based on evidence from (a) immunological studies suggesting a direct pathogenic impact of MOG-IgG, (b) neuropathological studies demonstrating discrete histopathological features, (c) serological studies reporting a lack of aquaporin-4 (AQP4)-IgG in almost all MOG-IgGpositive patients, and (d) cohort studies suggesting differences in clinical and paraclinical presentation, treatment response and prognosis, MOG-IgG is now considered to denote a disease entity in its own right, distinct from classic MS and from AQP4-IgG-positive NMO spectrum disorders (NMOSD) [14][15][16][17][18][19], which is now often referred to as MOG-IgG-associated encephalomyelitis (MOG-EM) or MOG-IgG-associated autoimmune disease [11,20,21].…”

Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 2: Results from 108 lumbar punctures in 80 pediatric patients

“…The suspected pathophysiological role of MOG-IgG was first described in children, who more often present with MOG-IgG-associated disorders than adults [12][13][14][15]. Based on evidence from (a) immunological studies suggesting a direct pathogenic impact of MOG-IgG, (b) neuropathological studies demonstrating discrete histopathological features, (c) serological studies reporting a lack of aquaporin-4 (AQP4)-IgG in almost all MOG-IgG-positive patients, and (d) cohort studies suggesting differences in clinical and paraclinical presentation, treatment response, and prognosis, MOG-IgG is now considered to denote a disease entity in its own right, distinct from classic MS and from AQP4-IgGpositive NMO spectrum disorders (NMOSD) [16][17][18][19][20][21], which is now often referred to as MOG-IgG-associated encephalomyelitis (MOG-EM) or MOG-IgG-associated autoimmune disease [11,22,23]. Several studies have shown that the proportion of patients with autoimmunity against MOG among all patients with inflammatory demyelinating CNS disorders is age-dependent with the highest seropositivity rates and highest MOG-IgG titers found in very young children [1,24,25].…”