Neuromyelitis Optica and Anti-MOG Disease Poster Presentation

P0699 - Clinical and radiological features of a hospital cohort of Neuromyelitis optica. (ID 324)

Speakers
  • G. Alvarez Bravo
Authors
  • G. Alvarez Bravo
  • L. Ramió I Torrentà
  • R. Robles Cedeño
  • E. Quintana
  • J. Gich
  • M. González
Presentation Number
P0699
Presentation Topic
Neuromyelitis Optica and Anti-MOG Disease

Abstract

Background

Neuromyelitis optica spectrum disorders (NMOSD) are mostly relapsing autoimmune inflammatory disorders of the central nervous system. Hallmark features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis or transverse myelitis. Attacks most often occur over days, with variable degrees of recovery over weeks to months

Other suggestive symptoms include episodes of intractable nausea, vomiting, hiccups, excessive daytime somnolence or narcolepsy, reversible posterior leukoencephalopathy syndrome, neuroendocrine disorders, and seizures

The discovery of AQP-4 antibodies supposed a breakthrough for understanding NMOSD. Recently, MOG antibodies have been also related to this entity. However, about 10-50% of NMOSD patients are still seronegative. These patients are a heterogeneous subgroup that may be associated with other autoantibodies

Objectives

To identify main clinical and radiological characteristics

To recognize differences between seropositive and seronegative NMOSD patients

To evaluate safety and effectiveness of Rituximab

Methods

We presented 12 patients diagnosed of NMOSD according revised consensus criteria published in 2015 at Unit of Neuroimmunology and Multiple Sclerosis Unit of Girona, Spain

The data were collected during the course of clinical care. We focused on medical history, neurologic symptoms, MRI features, CSF findings

Rituximab response was assessed using the annualized relapse rate (ARR)

Results

Disease-onset form: optic neuritis 7 (58,3%) 1 of them had bilateral optic neuritis; myelitis 4 (33,3%); brainstem syndrome 1 (8,3%)

Abnormal laboratory findings: 2 patients had positivity for lupus anticoagulant, 1 for TPO-Ab and 1 for ANAs

8 patients were seronegative NMOSD

6 patients (50%) had cognitive impairment

CSF findings: 8 patients had CSF abnormalities include pleocytosis and elevated protein levels. Oligoclonal bands were positive in 4 patients

4 patients were positive for antibodies: AQP-4= 2 / MOG= 2

MRI findings: 4 patients had brain MRI abnormalities that matched with NMOSD pattern, rest of patients had normal or unspecific MRI. Longitudinally extensive spinal cord lesions were observed in 7 patients. Cervical and thoracic segments were most affected

4 patients had positivity for other antibodies. 3 of them are seronegative NMOSD

Of 4 seropositive patients, 3 had cognitive impairment

Of 4 patients with presence of CSF oligoclonal bands, 3 were seronegative NMOSD

ARR before Rituximab: 1,25 and ARR after Rituximab: 0,27

No patient had serious adverse events after Rituximab treatment. Rituximab was discontinued in 1 patient due to an allergic reaction

Conclusions

Seronegative NMOSD has a prevalence up to 60% and it could be related with presence of other serum antibodies and with positivity for CSF oligoclonal bands

Cognitive impairment is frequent in NMOSD and can be more prevalent in seropositive patients

Rituximab is safe and effective to reduce ARR

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