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Background  Longitudinally Extensive Transverse Myelitis LETM is a specific pattern of myelitis wherein at least three continuous vertebral segments are involved. Characteristically, it is a defining feature of neuromyelitis optica NMO. However, it is described in many other etiologies.    Case presentation  We present a case of 60 year old male who presented with symptoms and signs of regional sympathetic dystrophy RSD followed by symptoms of myelitis. Spinal cord MRI revealed cervical LETM extending to the brainstem. In spite of serological negativity, treatment of suspected neuromyelitis optica spectrum disorder NMOSD was initiated and resulted in symptom relief. Meanwhile, sudden death occurred and autonomic dysreflexia was the main culprit.    Conclusions  This case suggests that RSD could be the mere primary presentation of LETM, discusses the differential diagnoses of LETM in elderly patients, and suggests the possible risk of autonomic dysreflexia in such patients.

LETM presented with causalgia and ensued by sudden death