[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. CONCLUSION: 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.

[The expression and clinical implications of glucocorticoid receptor isoforms in peripheral blood mononuclear cells in myasthenia gravis].

OBJECTIVE: To evaluate the expression of glucocorticoid receptor (GR) alpha and GRbeta in peripheral blood mononuclear cells (PBMC) from patients of myasthenia gravis (MG). To investigate the relationship between the expression level of GR and glucocorticoid (GC) therapeutic effects to MG patients. METHODS: The clinical score was recorded and used to assessing the therapeutic effects. Then the expression level of GRalpha and GRbeta in PBMC by immunocytochemistry was measured. We analysed the relationship of the therapeutic effects with expression level of GR. RESULTS: The scores of GRalpha positive PBMC score were same in healthy-control group, GC sensitive group, and GC dependent group. But GRalpha positive PBMC score in GC resistance group was decreased compared with the others (P < 0.01). The scores of GRbeta positive PBMC score were same in healthy-control group, GC sensitive group, and GC resistant group. But GRbeta positive PBMC score in GC dependent group was increased compared with the others (P < 0.01). CONCLUSIONS: The expression of GRalpha in GC resistant group are decreased, and the expression of GRbeta in GC dependence group is increased. The expression level of GRalpha or GRbeta is associated with the effect of GC in treatment of MG.

[The characteristics of enhanced gadolinium DTPA brain magnetic resonance images in 298 multiple sclerosis patients].

OBJECTIVE: To investigate the characteristics of enhanced brain magnetic resonance imaging (MRI) with gadolinium-DTPA (GD) in patients with multiple sclerosis (MS). METHODS: Brain enhanced MRI were studied in 186 patients with clinically definite multiple sclerosis. 298 MRI enhanced lesions were followed every 4 weeks and the study lasted 3 months to 2 years. RESULTS: Of the 298 enhanced MRI lesions 207 (69.5%) lasted less than 4 weeks, 251 (84.2%) 8 weeks, and 272 (91.3%) 12 weeks. There were 3 lesions lasting more than 2 years. Round, oval, ring form, arcuate, spotty, or irregular MRI GD enhanced lesions were found in our MS data. CONCLUSIONS: Most of the MRI GD enhanced lesions disappear in 4 weeks. Very few can last more than 2 years. So it is necessary to perform the GD enhanced MRI in 4 weeks for studying MS intracranial GD enhanced lesions. The DA enhanced MRI lesions in MS may be round, oval, ring form, arcuate, spotty or irregular.