[Clinical features of ultra longitudinally extensive transverse myelitis].
OBJECTIVE
To analyze the clinical features of ultra longitudinally extensive transverse myelitis (uLETM).


METHODS
Four first-onset uLETM patients hospitalized during September 2009 and March 2011 were recruited and retrospectively analyzed for clinical and MRI (magnetic resonance imaging) features, as well as therapeutic profiles and prognoses.


RESULTS
The male-to-female ratio was 1:3 and the age-of-onset 29 - 33 years old. Extremity paralysis and intrinsic sphincter disorders were initially observed. The clinical manifestations include visual, motor, sense and intrinsic sphincter disorders caused by optic nerve and spinal cord lesions. Uric acid decreased in 3 cases. Seropositivity for autoimmune antibody spectrum and NMO-IgG (neuromyelitis optica-immunoglobulin G) was found in some patients. Spinal MRI showed overall hypointense T1 and hyperintense T2 lesions in spinal cord with partial swelling and negative in brain MRI. Sjogren's syndrome associated with uLETM was diagnosed in 2 patients. Three cases improved after treatment with high-dose corticosteroids, intravenous immunoglobulin and other immunosuppressive agents. One patient died.


CONCLUSIONS
uLETM is commonly found in young women. Spinal cord is frequently affected. And it may occur concurrently with optical abnormalities and other autoimmune diseases. Intracranial parenchyma is rarely affected. The therapy of corticosteroids is recommended.
Department of Neurology, China-Japan Friendship Hospital, Beijing 100029, China.
Wei-He
WH
Zhang
Yu-Juan
YJ
Jiao
Jin-Song
JS
Jiao
Zun-Jing
ZJ
Liu
Ren-Bin
RB
Wang
D001323:Autoantibodies / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use; D009188:Myelitis, Transverse; D009471:Neuromyelitis Optica / Q000175:diagnosis
D001323:Autoantibodies; D007074:Immunoglobulin G