LONGITUDINAL EXTENSIVE TRANSVERSE MYELITIS AS FIRST MANIFESTATION OF SARCOIDOISIS

Abstract

Purpose: Sarcoidosis is a systemic granulomatous disease that affects the central nervous system in about 5 to 10% of the cases. Among the described cases, those in which the initial manifestation is longitudinally extensive transverse myelitis are even rarer. Case description: A 46-year-old caucasian woman, presenting bilateral dysesthesia in abdominal region, in dermatomes from T6 to T12, progressing with hypoesthesia at c2 level and motor deficit in the lower limbs, associated with urinary and fecal retention, global hyperreflexia and Lhermitte's signal. Cervical, thoracic and lumbosacral MRI showed longitudinally extensive transverse myelitis with intense contrast impregnation of cervical region to the medullary cone, sparing T1 and T3. On MRI the “Trident Sign” was notable. She was investigated for neoplastic, rheumatologic and infectious-parasitic conditions that were excluded. Anti-AQP4 antibody was negative and the ACE levels were within the normal range. Chest CT revealed mediastinal and perihilar lymph nodes, capturing contrast in FDG-PET/CT, which in immunohistochemical analysis were described as epithelioids non-caseous granulomas suggesting the diagnosis of sarcoidosis. The patient received intravenous corticosteroid therapy with methylprednisolone (1g / day for 5 days) getting good clinical response. Conclusions: Spinal neurosarcoidosis is still a challenge in clinical practice. In this cases, complementary exams are very important. Its treatment remains controversial, based on expert opinions.