Rationale:Longitudinally extensive transverse myelitis(LETM)is a rare inflammatory demyelinating disease predominantly associated with autoimmune and inflammatory disorders.Scrub typhus can cause a variety of neurolog...Rationale:Longitudinally extensive transverse myelitis(LETM)is a rare inflammatory demyelinating disease predominantly associated with autoimmune and inflammatory disorders.Scrub typhus can cause a variety of neurological complications,which may result in poor recovery and long-term disability.This report reveals a rare linkage between LETM and scrub typhus.Patient concerns:A 24-year-old female presented with a history of fever for 10 days associated with progressive quadriparesis.Physical examination revealed bilateral lower limb weakness(grade 0/5)and bilateral upper limb weakness(grade 2/5)with reduced sensation below the T3 dermatome.Diagnosis:Scrub typhus presenting with longitudinally extensive transverse myelitis.Interventions:Steroid pulse therapy with antibiotics supported by early intensive rehabilitation.Outcomes:Complete recovery was achieved.Lessons:This case report highlights the importance of considering scrub typhus as a potential cause of LETM in endemic regions,particularly in patients presenting with febrile illness and neurological symptoms.展开更多
Uric acid is reduced in demyelinating diseases, including multiple sclerosis and neuromyelitis optica (NMO). Longitudinally extensive transverse myelitis (LETM) is often an early manifestation of NMO. Whether uric...Uric acid is reduced in demyelinating diseases, including multiple sclerosis and neuromyelitis optica (NMO). Longitudinally extensive transverse myelitis (LETM) is often an early manifestation of NMO. Whether uric acid levels are reduced at presentation of LETM remains unknown. The present study investigated serum uric acid levels by evaluating 35 patients with LETM, 64 with NMO, 62 with multiple sclerosis, 63 with other neurological diseases and 65 healthy controls. In addition, we tested the correlation between serum uric acid and the clinical characteristics of LETM. All patients were in the acute phase, defined as less than 1 month from onset or relapse. The results revealed that serum uric acid levels in LETM were significantly lower than in multiple sclerosis, other neurological diseases and healthy controls, but no difference was found between LETM and NMO. A negative correlation between uric acid levels and Expanded Disability Status Scale scores was found in LETM patients (r = -0.545, P 〈 0.05). The results suggest that uric acid levels are reduced in patients with LETM, raising the possibility that lower uric acid levels are an indicator of disease disability. Moreover, reduced uric acid levels may be a risk factor in NMO.展开更多
Neuromyelitis optica spectrum disorders, or neuromyelitis optica(NMO), is an autoimmune disease of the central nervous system that must be distinguished from multiple sclerosis. Therapeutic approaches to relapse preve...Neuromyelitis optica spectrum disorders, or neuromyelitis optica(NMO), is an autoimmune disease of the central nervous system that must be distinguished from multiple sclerosis. Therapeutic approaches to relapse prevention in NMO include immunosuppressants and monoclonal antibodies. Rituximab, a monoclonal antibody that targets CD20 antigen expressed on the surface of pre-B, mature B-lymphocytes and a small subset of T-lymphocytes, has been widely used for the treatment of NMO. In this review, we aim to summarize global experience with rituximab in NMO. We identified 13 observational studies that involved a total of 209 NMO patients treated with rituximab. Majority of rituximab-treated patients evidenced stabilization or improvements in their disability scores compared to pre-treatment period and 66% of patients remained relapse-free during treatment period. Monitoring rituximab treatment response with CD19+ or CD27+ cell counts appears to improve treatment outcomes. We offer clinical pointers on rituximab use for NMO based on the literature and authors' experience, and pose questions that would need to be addressed in future studies.展开更多
文摘Rationale:Longitudinally extensive transverse myelitis(LETM)is a rare inflammatory demyelinating disease predominantly associated with autoimmune and inflammatory disorders.Scrub typhus can cause a variety of neurological complications,which may result in poor recovery and long-term disability.This report reveals a rare linkage between LETM and scrub typhus.Patient concerns:A 24-year-old female presented with a history of fever for 10 days associated with progressive quadriparesis.Physical examination revealed bilateral lower limb weakness(grade 0/5)and bilateral upper limb weakness(grade 2/5)with reduced sensation below the T3 dermatome.Diagnosis:Scrub typhus presenting with longitudinally extensive transverse myelitis.Interventions:Steroid pulse therapy with antibiotics supported by early intensive rehabilitation.Outcomes:Complete recovery was achieved.Lessons:This case report highlights the importance of considering scrub typhus as a potential cause of LETM in endemic regions,particularly in patients presenting with febrile illness and neurological symptoms.
基金the National Natural Science Foundation of China,No. 30971037
文摘Uric acid is reduced in demyelinating diseases, including multiple sclerosis and neuromyelitis optica (NMO). Longitudinally extensive transverse myelitis (LETM) is often an early manifestation of NMO. Whether uric acid levels are reduced at presentation of LETM remains unknown. The present study investigated serum uric acid levels by evaluating 35 patients with LETM, 64 with NMO, 62 with multiple sclerosis, 63 with other neurological diseases and 65 healthy controls. In addition, we tested the correlation between serum uric acid and the clinical characteristics of LETM. All patients were in the acute phase, defined as less than 1 month from onset or relapse. The results revealed that serum uric acid levels in LETM were significantly lower than in multiple sclerosis, other neurological diseases and healthy controls, but no difference was found between LETM and NMO. A negative correlation between uric acid levels and Expanded Disability Status Scale scores was found in LETM patients (r = -0.545, P 〈 0.05). The results suggest that uric acid levels are reduced in patients with LETM, raising the possibility that lower uric acid levels are an indicator of disease disability. Moreover, reduced uric acid levels may be a risk factor in NMO.
基金Supported by The National Multiple Sclerosis Society(NMSS),Guthy-Jackson Charitable Foundation,EMD-Serono/Pfizer,Biogen Idec,Serono and Novartis,and served on advisory board for Biogen Idec(Kister I)
文摘Neuromyelitis optica spectrum disorders, or neuromyelitis optica(NMO), is an autoimmune disease of the central nervous system that must be distinguished from multiple sclerosis. Therapeutic approaches to relapse prevention in NMO include immunosuppressants and monoclonal antibodies. Rituximab, a monoclonal antibody that targets CD20 antigen expressed on the surface of pre-B, mature B-lymphocytes and a small subset of T-lymphocytes, has been widely used for the treatment of NMO. In this review, we aim to summarize global experience with rituximab in NMO. We identified 13 observational studies that involved a total of 209 NMO patients treated with rituximab. Majority of rituximab-treated patients evidenced stabilization or improvements in their disability scores compared to pre-treatment period and 66% of patients remained relapse-free during treatment period. Monitoring rituximab treatment response with CD19+ or CD27+ cell counts appears to improve treatment outcomes. We offer clinical pointers on rituximab use for NMO based on the literature and authors' experience, and pose questions that would need to be addressed in future studies.