Dados do Trabalho

Título

A remarkable radiological improvement in a patient with NMOSD

RESUMO

A 43-year-old woman presented to the neurology department quadriplegic, with hypoesthesia below the C4 level, bilateral impaired vision, no alterations in other cranial nerves and bilateral Babinski sign. She had a history of similar symptoms 7 months before, but was not examined at that time. The dosage of vitamin B12 was 88 pg/mL, the AQP4-IgG was negative, MOG-IgG was not available, and other laboratory results were non-significant. Lumbar puncture was not performed because the patient was in full anti-coagulation. A magnetic resonance imaging (MRI) of the spine evidenced areas of hyperintensity on T2-weighted sequences from the area postrema to the conus medullaris, compatible with longitudinally extensive transverse myelitis. The patient met the diagnostic criteria for neuromyelitis optica spectrum disorders (NMOSD) without AQP4-IgG, and a course of pulse therapy with methylprednisolone was prescribed, as well as one plasma exchange therapy, and vitamin B12 replacement. She used oral prednisone until azathioprine was available.
Five years after this episode, the patient returned to the neurology department for a follow-up consultation. She was in a good general state, no longer in need of a wheelchair, with a spastic paretic gait, no alterations in cranial nerves, muscular strength of 4/5 in the upper limbs and 3/5 in the lower limbs, and hypoesthesia below T10 level. Her EDSS was 5,5. A new MRI of the spinal cord showed a great radiological improvement, with only small areas of hyperintensity at the cervical spine.
The reported case shows a patient diagnosed with NMOSD with an important clinical and radiological improvement after 5 years of evolution. The initial manifestation on MRI, with a centrally located lesion on axial imaging, a large cord expansion and post-contrast enhancement, was compatible with the general presentation of NMOSD. While vitamin B12 deficiency could be a confounding factor, radiological findings do not support the hypothesis, although it could enhance symptoms.
Different studies discuss the link between AQP4-IgG, MOG-IgG and the disease prognosis, with better outcomes for patients who are AQP4-IgG-seronegative and MOG-IgG-seropositive. In the present case, MOG-IgG was not tested, which weakened the ability to completely interpret the clinical observations.
Nevertheless, a remarkable reduction of the lesions extent on MRI and a less likely chance of relapse are seen in AQP4-seronegative cases, as in this case.

Palavras Chave

NMOSD, AQP4-IgG, Longitudinally extensive transverse myelitis