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Título

Hypersomnolence and encephalopathy as a manifestation of Neuromyelitis Optica: A Case Report

RESUMO

Case Presentation: Female, 77 years old, hypertensive. In January 2021, she started presenting behavioral changes, difficulty looking up and binocular horizontal diplopia. The medical team performed brain MRI which presented a slight expansive effect on the mesial portions of the thalamus, hypothalamus and midbrain. After being hospitalized for investigation, the patient evolved to an altered level of consciousness, with severe drowsiness. She was submitted to a lumbar puncture and a non-reactive anti-AQP4 test. Part of the liquor was sent to Vienna. On 02/02, a stereotaxic biopsy was performed due to suspicion of a tumoral lesion. Treatment with IV pulse therapy was started. On 02/08 she had reentrant seizures and one week after started IV immunoglobulin. On 02/25 she was discharged from hospital with partial improvement in her consciousness level. The next day she returned due to worsening of the drowsiness. In a new examination, a reduction of previously altered areas in the thalamus was seen. A new therapeutic attempt was performed, including Cyclophosphamide 1g IV, evolving with a significant improvement in the level of consciousness, but still presenting periods of drowsiness and cognitive impairment. On 03/26, a result of anti-AQP4 from Vienna was sent back with a reactive result.Discussion: Neuromyelitis Optica (NMO) is characterized by the presence of antibodies called Aquain 4 present in the hematoencephalic barrier. It has predominantly female occurrences and the first symptoms occur between the 3rd and 4th decades of life. The most common clinical manifestations include visual impairment and bilateral motor symptoms due to optic neuritis and transverse myelitis. There are other NMO spectrum syndromes, including an encephalopathy characterized by other levels of consciousness syndromes and seizures. The diagnoses are based on the presence of AQP4 antibody associated with at least one outbreak of clinical syndromes. One can still have the diagnosis without the presence of the antibody along with two clinical outbreaks and the presence of extensive lesions in the optic nerve , spinal cord or brain stem.Final comments: In patients with positive AQP4 antibody, it is possible to make the diagnosis of NMO even with atypical clinical presentations. Our patient presented from the beginning the typical symptoms of NMO Encephalopathy in addition to radiological changes, but the diagnosis was concluded after the second AQP4-AB test with a positive result.

Palavras Chave

Neuromyelitis Optica; Encephalopathy; Hypersomnolence;