Longitudinally Extensive Transverse Myelitis: One Disease, Variable Outcomes-A Case Series

Abstract

Longitudinal extensive transverse myelitis (LETM) is a rare form of widespread inflammation of the spinal cord causing T2 hyperintensity in spinal magnetic resonance imaging (MRI) extending across three or more vertebral segments. It is an acute onset of sensory, motor, and autonomic dysfunction of variable etiology with a likely poor outcome. We present a case series of three cases wherein children between the ages of 4 and 13 years had diverse symptoms from gradual painless loss of vision in both eyes with headache, vomiting and seizure, and a normal central nervous system examination except involvement of the optic nerve to another child with abdominal pain, urinary retention and constipation for 3 days with exaggerated DTR, and patchy sensory loss without any definite sensory level, and to the third child with fever and weakness of lower limbs, hypotonia and grade 1-2/5 power in lower limbs and normal upper limb power. Contrast-enhanced MRI spine of all children showed long segment T2 hyperintensity with variable involvement of the brain. The first two children were treated with pulsed dose methylprednisolone, and the last child received intravenous immunoglobulin followed by methylprednisolone. All were followed with oral prednisolone. LETM has a varied presentation with different etiologies. Antineuromyelitis optica immunoglobulin G (IgG) antibody (Aquaporin-4 IgG) and antimyelin oligodendrocyte glycoprotein antibody are strongly recommended though they may not be locally available or not affordable. Early and aggressive immunomodulatory therapy may help faster recovery, as did with two of our three children.

Keywords: Aquaporin-4; myelin oligodendrocyte glycoprotein; paraparesis.

Fig. 1

( A ) T2 hyperintense signal with minimal enhancement in the cord involving both gray and white matter with mild cord expansion extending from D6–D7 to D10–D11. ( B ) T2 hyperintensity in the cord opposite D4–D5. ( C ) T2-hyperintense signals seen in the central aspect of the cord occupying more than two-thirds of the cross-sectional area in cervical and dorsal (cervical > dorsal) regions extending cranially till anterior medulla and caudally till conus medullaris.