Dados do Trabalho

Título

Differential diagnosis in demyelinating diseases: A case report.

RESUMO

Case Report: A 46 years old female patient came to our Neurology Service in 2003 with decreased visual acuity in her left eye, which evolved to amaurosis after 7 days. Brain MRI revealed two hyperintense signs on T2 and FLAIR in the semioval center, without post-contrast enhancement, and hyper signal in the left optic nerve on the STIR sequence. She was treated with pulse therapy and partially improved. She returned after a few months complaining of low visual acuity in her right eye. A cervical MRI revealed contrast-enhanced lesions in C2-C3, CSF test was positive for oligoclonal bands (OCB) and a serum aquaporin-4 antibody was negative. She was treated with three cycles of pulsetherapy and began Interferon ß1-a 44mcg, being episode free for several years, even though routine brain MRI revealed new lesions in 2009 and 2011. In 2020, at the age of 61, she complained of paresthesia in her left inferior limb, which ascended to the dorsal region. Neurologic examination revealed crural paraparesis and posterior cord syndrome. A new cervical and dorsal MRI showed an extensive hypointense lesion on T1, and hyperintense on T2, with peripheral contrast enhancement involving the central and posterior portions of the spinal cord, with an expansive aspect from C5 to D1, surrounded by edema from C2 to D3. A new CSF study demonstrated positive OCB, positive aquaporin-4 antibody in CSF and serum, and non-reactive anti-MOG. A new pulsetherapy and azathioprine were initiated after the denial of rituximab by her healthcare plan, with a good response to treatment. Discussion: Neuromyelitis optica (NMO) is a demyelinating disease with severe and more disabling outbreaks than multiple sclerosis (MS). In this reported case, the patient had neuroimaging studies suggestive of MS, with positive OCB in the CSF and negative anti-aquaporin-4 antibody in the serum. After 16 years without any recurrent episodes, or significant gain of lesion load, she presented with a new severe bout with posterior cord ataxia and crural paraparesia, with evidence of extensive myelitis on cervical-dorsal MRI. Conclusion: As a highly sensitive test, aquaporin-4 antibody has gained prominence to differentiate MS from NMO, increasing the diagnostic accuracy of NMO to 90% of cases, when associated with clinical criteria.Therefore, in diagnostic doubt aquporine-4 antibody must be tested more than once.The differential diagnosis between these two entities is essential and requires specific treatments.

Palavras Chave

Neuromyelitis optica, multiple sclerosis, hyperintense signs.