Dados do Trabalho

Título

An unsual presentation of thoracic spinal cord atrophy due to Susac Syndrome

RESUMO

Case presentation
A 35-year-old man presented with 3 years history of progressive crural paraparesis and urinary incontinence. He had a previous hospitalization for cognitive symptoms, headache and seizure. Was observed in MRI increased T2 signal in the corpus callosum and lumbar puncture showed an increase protein ( 86 mg/dL) and White cells (9 with 88% neutrophils). Because of these findings, at this moment, the main hypothesis was multiple sclerosis (MS). A treatment for presumed primary demyelinantion disease with methylprednisolone was performed with improvement of the mental confusion. Three years later was performed another MRI, showing increased T2/FLAIR signal in the central corpus callosus, assuming snowball appearance. At this time he reported urinary incontinence, erectile dysfunction, sensory symptoms and weakness. Physical exam showed a crural paraparesis and a spastic gait. Moreover he had an ophtalmological examination showing areas of ischaemia demonstrating branch retinal artery occlusion and the MRI images had involvement of central corpus callosum and deep grey matter lesions in the basal ganglia. The image of spinal cord showed a thoracic atrophy with no sign enhance and cerebrospinal fluid analysis was normal. Under these circumstances another acute treatment was not performed. The presumptive diagnosis of Susac Syndrome was made.

Discussion
Susac`s syndrome is a rare autoimmune disease that affects the microvasculature. It’s an endoteliopathy that can be manifest with neurological, visual or auditive symptoms. (1) The classic clinical presentation was described as the triad of encephalopathy, sensorineural hearing loss and branch retinal artery occlusion. (2) Our patient had bilateral and multiple retinal ischaemia, but no involviment of the optic nerve. Although his spinal cord image showed an thoracic atrophy, spinal cord involvement was extremely rare in Susac’s syndrome. (4)

Final comments
Susac syndrome is a rare disease and the differential diagnosis includes others autoimmune diseases, infectious diseases and paraneoplastic syndromes. The ocular and vestibule-cochlear involvement, along with neurological symptoms were cardinal features of the syndrome, but it could not be seen at the same time. Presentation with spinal cord involvement is rare, but have been described and it’s important to be known or could lead to a misdiagnosed with other demyelination disease.