RELAPSING LONGITUDINALLY EXTENSIVE TRANSVERSE MYELITIS IN HUMAN T-LYMPHOTROPIC VIRUS TYPE 1-2 INFECTION

Background

Neuromyelitis optica spectrum disorders (NMOSD) are

severe, inflammatory, immune-mediated disorders of the

central nervous system which predominantly affect the

optic nerves and the spinal cord. Longitudinally extensive

transverse myelitis (LETM) is the most suggestive sign of

NMOSD although it may occur in a number of other

conditions. HTLV-1 infection has a worldwide distribution

but is highly prevalent in Latin America, Middle East and

Africa. Its associated myelopathy or tropical spastic

paraparesis (HAM/TSP) is the most common neurological

complication of the viral infection. HAM/TSP usually has a

slow onset with chronic progression, but an acute and more

severe form of the disease, characterized by rapid

progression, may involve extensive length of the spinal

cord with swelling and vacuolization mimicking the

longitudinally extensive transverse myelitis (LETM)

associated with anti-aquaporin-4 autoimmunity. Cases of

LETM with rapid progressive course have been reported in

post-transfusion and post-transplantation patients. We

report a previously healthy anti-AQP4- IgG seronegative

patient who developed relapsing LETM associated with

HTLV-1 infection.

Case report

A 41-year-old mulattoe female, was in good health until

2004 when noticed slight weakness of the left lower limb.

She denied any sensation or sphincter disturbance in

association with the weakness. No work-up was done. She

did well with no progression of her symptom until 2011

when she developed

Juliana M. S. S. Amaral1, Natália C. Talim1, Lívia E. C. Talim1, Márcia Prates1, Rodrigo Kleinpaul1, Carolina R. Araujo1, Cristiane F. Rocha1, Kazuo Fujihara2, Douglas Sato2, Marco A. Lana-Peixoto1.1CIEM MS Research Center, Federal University of Minas Gerais Medical School, Belo Horizonte, Brazil, and 2Tohoku University, Sendai, Japan.

severe paraparesis and dysesthesia in both legs. Spinal MRI disclosed severe atrophy of the spinal cord between its T1 – T10 vertebral segments. Brain MRI was unrevealing. Search for serum aquaporin 4-IgG by cell-based assay was negative, whereas ELISA detected HTLV I-II serum antibodies.

Conclusion

Very rarely HAM/TSP may manifest as relapsing LETM in previously healthy patients. It has to be included in the differential diagnosis of NMOSD, mainly in countries where infection by HTLV-1 is highly prevalent.

References:1. Delgado SR, Sheremata WA, Brown Adet al. Human T-lymphotropic virus type I or II (HTLV-I/II) associated with recurrent longitudinally extensive transverse myelitis (LETM): two case reports. J Neurovirol 2010; 16: 249-53.2- Souza A, Tanajura D, Toledo-Cornell C, Santos S, et al. Immunopathogenesis and neurological manifestations associated to HTLV-1 infection. Rev Soc Bras Med Trop 2012; 45: 545-552.

Figure. Spinal MRI.T2-weighted sequence showing spinal atrophy from T1 to T10.