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Neuromyelitis optica spectrum disorder (NMOSD) and MOG antibody-associated disease (MOGAD)

“NMOSD and MOGAD are autoimmune inflammatory diseases of the Central Nervous System, primarily affecting the optic nerves and the spinal cord. NMOSD is more common in women and in Asian and African-descendant populations, with an onset typically between 30 and 40 years of age. MOGAD also affects children and adults, without a strong gender prevalence.”

NMOSD and MOGAD are autoimmune diseases. In NMOSD, the immune system produces autoantibodies against aquaporin-4 (AQP4), a protein found on astrocytes; however, seronegative forms also exist, where the anti-AQP4 antibody is not detectable. In MOGAD, the antibodies target the MOG protein, which is present on myelin. The exact etiology is unknown, but a genetic predisposition combined with environmental factors, such as viral infections that trigger the autoimmune response, is believed to be involved.

“Both NMOSD and MOGAD manifest with acute, often severe episodes that can leave permanent neurological deficits, especially if not treated early. MOGAD can have a monophasic course and tends to leave fewer sequelae, whereas NMOSD is typically relapsing with highly intense attacks that can result in disabling sequelae.

Unlike multiple sclerosis, these diseases do not show a progressive accumulation of neurological disability between attacks.

Both conditions preferentially affect the optic nerve and the spinal cord, presenting with distinct clinical manifestations.

Main symptoms of NMOSD:

  • Optic neuritis (often bilateral, with severe visual loss)

  • Longitudinally extensive transverse myelitis (paralysis, sensory disturbances, incontinence)

  • Area postrema syndrome (intractable nausea, vomiting, and hiccups)

Main symptoms of MOGAD:

  • Optic neuritis (unilateral or bilateral, associated with optic disc edema, with a better response to corticosteroids)

  • Myelitis, often with involvement of the conus medullaris and sphincter symptoms

  • Encephalitis or ADEM (Acute Disseminated Encephalomyelitis, more frequent in children)

  • Seizures or encephalopathy (especially in younger patients)”

“The diagnosis of NMOSD and MOGAD is based on the integration of clinical, laboratory, and radiological data. An early and accurate diagnosis allows for targeted treatment and can prevent severe neurological disabilities.

  • Serum antibody testing using cell-based assays: anti-AQP4 antibodies for NMOSD and anti-MOG for MOGAD.

  • Magnetic Resonance Imaging (MRI) of the brain, including dedicated studies of the optic nerves and the entire spinal cord, both with and without contrast medium.

  • Cerebrospinal fluid (CSF) analysis: evaluation for oligoclonal bands (often absent in NMOSD and MOGAD, unlike in multiple sclerosis), mild pleocytosis, or elevated protein levels (hyperproteinorrachia).

  • Visual Evoked Potentials (VEP) and neuro-ophthalmological examination with Optical Coherence Tomography (OCT): evaluation of the visual pathways and measurement of the retinal nerve fiber layer thicknes

Available therapies

1. Acute Phase Therapies These are administered during attacks to reduce inflammation and promote recovery.

  • Common treatments: High-dose steroids (intravenous), intravenous immunoglobulins (IVIG), and plasmapheresis (plasma exchange).

  • Key difference: MOGAD tends to respond better to steroids compared to NMOSD.

2. Preventive Therapies (Long-Term) The goal is to modulate the immune system to prevent new relapses.

  • NMOSD (Neuromyelitis Optica)

Research in progress

  • Identificazione di fattori di rischio
  • Identificazione di nuovi biomarcatori biologici e radiologici per la diagnosi precoce
  • Terapie innovative per contrastare l’evoluzione della malattia

Contacts and informations

Email/ph: malattieneuromuscolari@policlinico.mi.it / 02 5503 6504