Dados do Trabalho

Título

Paraneoplastic Neuromyelitis Optica Spectrum Disorder with anti-Aquaporin-4 Associated With Squamous Cell Carcinoma of the Lung

RESUMO

Case report

A 61-year-old man with a past medical history of liver transplantation in 2001 due to alcoholic cirrhosis was admitted for untreatable hiccups and vomiting, which had started 14 days earlier. Initial imaging showed a left lung apex mass. A biopsy was made and the patient was diagnosed with squamous cell lung carcinoma.
One month after the first symptoms, the patient developed paraparesis, upper right limb paresis, complete anesthesia of the lower limbs, urinary and fecal retention. During the following 24 hours, the symptoms worsened and he became paraplegic. Brain and spine MRI showed a longitudinally extensive transverse myelitis, with high T2 signal involving C5 to T11 segments, along with high T2 signal within the area postrema. CSF analysis were as follows: protein 472 mg/dl, 124 cells (81% neutrophils), with no other relevant findings. Anti-aquaporin 4 antibodies were detected on a blood sample. The patient was not tested for onconeural antibodies due to unavailability.
The patient was started on high dose EV corticosteroids (methylprednisolone 1g for five days), without any improvement. A second treatment attempt with a five-day course of plasma exchange therapy was also unsuccessful. During hospital stay, the patient developed sepsis and was intubated. Due to respiratory muscular weakness, ventilator weaning was challenging. After a prolonged ICU stay, the patient died.

Discussion
Paraneoplastic neuromyelitis optica is a rare condition, with an estimated incidence of less than 4 per cent of all NMOSD cases. It is more often associated with lung and breast adenocarcinoma. However, other cancer types can be involved. The mean age of onset is 55 years. Brainstem lesions and longitudinally extensive transverse myelitis are the most common presentations, and some studies suggest cancer screening in these patients.

Conclusion
Although the condition is rare, it is important for the neurologist to recognize paraneoplastic NMOSD as a cause of demyelinating disease in cancer patients, but also as a red flag for possible occult tumors in older patients who develop neuromyelitis optica, especially those over the age of 55.