A 45-year-old female suffering from severe thoracic
pain was admitted to the emergency department of our hospital. Thorough clinical examination revealed
paresis of the left lower limb and sensory deficit at the level of the Th4 vertebra. MRI of the thoracic spine demonstrated a lesion at the level of Th1-Th7. Despite initial improvement following i.v.
corticosteroid administration, the patient's clinical status deteriorated, with recurrence of
myelitis and extension of the lesion to Th12. She developed
paraparesis,
hyperreflexia and spasticity of both legs, symmetrical sensory deficit below Th4, and sphincter dysfunction. Differential diagnosis included infectious, metabolic, neoplastic/paraneoplastic, and ischemic causes as well as
multiple sclerosis. NMO
IgG was found positive and led to the diagnosis of longitudinal extensive
transverse myelitis (LETM) in the
NMO spectrum disorders. Administration of immunosuppressive therapy resulted in gradual improvement of the patient's clinical status and stabilization for five years. In the setting of LETM, patients with antiaquaporin 4 IgGs can present features of coexisting systemic involvement. A thorough differential diagnosis is required to guide appropriate
therapy.