BACKGROUND：Devic＇s neuromyelitis optica （DNMO） and multiple sclerosis in Asian populations have been considered to be the same disease. However, there is an increasing number of studies suggesting that DNMO and mu...BACKGROUND：Devic＇s neuromyelitis optica （DNMO） and multiple sclerosis in Asian populations have been considered to be the same disease. However, there is an increasing number of studies suggesting that DNMO and multiple sclerosis are different diseases.OBJECTIVE：Little information is available regarding comparisons of DNMO patients between China and other countries, as well as clinical manifestations of Chinese patients with DNMO and multiple sclerosis. The present study performed a multi-center, pathological, retrospective analysis.DESIGN, TIME AND SETTING：A retrospective analysis of clinical data from seven patients with DNMO diagnosed between 1957 and 1998.PARTICIPANTS：Data from Chinese DNMO patients was provided by the Shanghai Second Medical University, Sun Yat-sen University of Medical Sciences and the First Affiliated Hospital of Harbin Medical University in China.METHODS：Clinical and pathological data from Chinese patients with DNMO were retrospectively analyzed. The clinical characteristics of DNMO were compared between Chinese and Caucasian patients. In addition, clinical and pathological differences between DNMO and multiple sclerosis Chinese patients were compared.MAIN OUTCOME MEASURES：Clinical and pathological features of Chinese patients with DNMO.RESULTS：All seven Chinese patients with DNMO exhibited abrupt onset of vision disturbance, with a disease course of 3 clays to 9 years. DNMO recurred in two of the patients. Demyelinating lesions were observed in all patients, with necrotic lesions and gitter cells in five patients, collagenous hyperplasia in one patient, and perivascular inflammatory cell infiltration in six patients. Comparison between Chinese and Caucasian DNMO patients revealed no significant differences in age at onset, clinical onset, duration, or interval between optic neuritis and myelitis. Compared with Chinese multiple sclerosis patients, Chinese DNMO patients presented with fewer recurrences, higher occurrence of necrosis, perivascular inflammatory cell infiltration and gitter cells, and a lower occurrence of collagenous hyperplasia.CONCLUSION：There was no difference in DNMO clinical features between Chinese and Caucasian patients. However, the clinical and pathological features of DNMO were different compared with multiple sclerosis in Chinese patients. Results suggested that the characteristics of DNMO in Chinese patients were significantly different than multiple sclerosis.展开更多
Devic’s neuromyelitis optica (NMO) is a rare inflammatory disease of the central nervous system that results in optic neuropathy and myelitis. Optic neuritis represents the mode of entry into the disease in more than...Devic’s neuromyelitis optica (NMO) is a rare inflammatory disease of the central nervous system that results in optic neuropathy and myelitis. Optic neuritis represents the mode of entry into the disease in more than two thirds of cases. It is a rare entity in children. There is no effective treatment at present, but some molecules can be used, such as corticosteroids, immunosuppressants and plasma exchange. The prognosis in children is generally favorable. Devic’s neuromyelitis is a condition of unknown etiopathogeny which is functionally critical and requires early and appropriate treatment. We report the case of a 12-year-old girl who presented to emergency with a headache and decreased visual acuity, whose investigations led to the diagnosis of Devic’s neuromyelitis optica.展开更多
Recent studies provided evidence that mesenchymal stem cells（MSCs） have regenerative potential in cutaneous repair and profound immunomodulatory properties making them a candidate for therapy of neuroimmunologic dis...Recent studies provided evidence that mesenchymal stem cells（MSCs） have regenerative potential in cutaneous repair and profound immunomodulatory properties making them a candidate for therapy of neuroimmunologic diseases. Neuromyelitis optica（NMO） is an autoimmune, demyelinating central nervous system disorder characterized by a longitudinally extensive spinal cord lesion. A 46-year-old male diagnosed with NMO had relapses with paraplegia despite treatment and developed two stage IV pressure ulcers（PUs） on his legs. The patient consented for local application of autologous MSCs on PUs. MSCs isolated from the patient＇s bone marrow aspirate were multiplied in vitro during three passages and embedded in a tridimensional collagen-rich matrix which was applied on the PUs. Eight days after MSCs application the patient showed a progressive healing of PUs and improvement of disability. Two months later the patient was able to walk 20 m with bilateral assistance and one year later he started to walk without assistance. For 76 months the patient had no relapse and no adverse event was reported. The original method of local application of autologous BM-MSCs contributed to healing of PUs. For 6 years the patient was free of relapses and showed an improvement of disability. The association of cutaneous repair, sustained remission of NMO and improvement of disability might be explained by a promotion/optimization of recovery mechanisms in the central nervous system even if alternative hypothesis should be considered. Further studies are needed to assess the safety and efficacy of mesenchymal stem cells in NMO treatment.展开更多
Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with （n = 6） or without primary Sjogr...Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with （n = 6） or without primary Sjogreffs syndrome （n = 10）. All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren＇s-syndrome-related antigen A an- tibodies, anti-Sjogren＇s-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren＇s syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% （4/6） of patients with primary Sjogren＇s syndrome and in 60% （6/10） of patients without primary Sj6gren＇s syndrome. More brain abnormalities were observed in patients without primary Sj6gren＇s syndrome than in those with primary Sj6gren＇s syndrome. Segments lesions （〉 3 centrum） were noted in 50% （5/10） of patients without primary Sj6gren＇s syndrome and in 67% （4/6） of patients with primary Sjogren＇s syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.展开更多
Neuromyelitis optica(NMO)refers to an antibody mediated,inflammatory disorder of the central nervous system(CNS)characterized by recurrent or monophasic attacks of optic neuritis and myelitis.Most patients with NMO po...Neuromyelitis optica(NMO)refers to an antibody mediated,inflammatory disorder of the central nervous system(CNS)characterized by recurrent or monophasic attacks of optic neuritis and myelitis.Most patients with NMO possess a specific serum immunoglobin,NMO-IgG,which can serve as a biomarker for NMO.The autoantibodies target aquaporin-4(AQP4),the main water channel protein found in the CNS including the brain,spinal cord,and optic nerve.The remaining 10-25%of patients are seronegative for NMO-IgG despite meeting the diagnostic criteria for NMO.Recent studies have shown that a subset of these patients is seropositive for antibodies against myelin oligodendrocyte glycoprotein(MOG).This paper will provide an overview of the current English scientific literature published regarding the history,epidemiology,AQP4 biomarker,MOG biomarker,diagnosis,clinical features,related diseases in NMO spectrum disorder(NMOSD),and treatments of NMO.展开更多
Background: In Iranian patients with opticospinal multiple sclerosis (OSMS), a paucity of brain lesions and short spinal cord lesions extending less than three spinal segments are characteristic findings on magnetic r...Background: In Iranian patients with opticospinal multiple sclerosis (OSMS), a paucity of brain lesions and short spinal cord lesions extending less than three spinal segments are characteristic findings on magnetic resonance imaging (MRI). It also shows a relatively benign course with negative CSF oligo-coonal bands. Objective: We aimed to clarify the possible relationship between clinical phenotype and MRI features of OSMS and human leucocyte antigen (HLA) system in Iran. Methods: Genotyping of HLA class II allele frequencies in 20 patients with OSMS were done, using polymerase chain reaction sequence-specific primer amplification method. Blood samples were extracted and typed for HLA-DRB, DQA, and DQB loci and compared with 100 controls. Results: Significant positive association was observed in DRB1*03, DQA1*0201, DQA1*03, DQB1*0201, and DQB1*0611, while DQB1*0602 was absent in our patients. Conclusion: These finding suggest that HLA-DRB association pattern in OSMS is different from conventional MS in Iran which is mostly associated with DRB1*1501 and from similar Japanese OSMS who are negative for brain lesions fulfilling the Barkhof criteria and negative for the presence of longitudinally extensive spinal cord lesions who carries the DRB*0405 allele. OSMS is immunogenetically heterogeneous. Also absence of DQB1*0602 allele may negatively be associated with the absence of Barkhof brain lesion.展开更多
Optic neuritis is the most common cause of unilateral visual loss associated with orbital pain on ocular movements and impaired colour vision. It can present with anterior optic neuritis, papillitis, a swollen optic d...Optic neuritis is the most common cause of unilateral visual loss associated with orbital pain on ocular movements and impaired colour vision. It can present with anterior optic neuritis, papillitis, a swollen optic disc, retrobulbar neuritis with a normal optic disc, neuritis with oedema of the optic disc and the macular star or anterior ischemic neuropathy. It may be the only manifestation of a disease such as demyelination or associated with systemic diseases such as systemic lupus, giant cell arteritis, Sjogren’s syndrome, herpes simplex, herpes zoster, Lyme disease, CMV, EBV, HIV, toxoplasmosis, West Nile virus, Chikungunya, dengue fever, Rift Valley fever, mumps, rubella, measles, cat scratch disease, tuberculosis, syphilis, rickettsia illnesses, Q fever, Whipple disease, brucellosis, leptospirosis, leprosy, toxocariasis, malaria, Cryptococcus, candidiasis, histoplasmosis, Aspergillosis, mucormycosis, Bacillus Chalmette-Guerin (BCG) vaccination, ethanol and methanol toxicity, sarcoidosis, neuromyelitis optica and Liber’s hereditary optic neuropathy. A systemic approach to the diagnosis is of paramount importance in confirming, diagnosing and treating optic neuritis and underlying systemic diseases to provide definitive cures. Failure to diagnose optic neuritis and treat the cause may result in optic atrophy and permanent blindness. In this paper, we reviewed the diagnosis and differential diagnoses of optic neuritis, including arteritis, ischemic, neuroretina, and vasculitic causes.展开更多
Purpose: This case report highlights a rare instance of a 26-week pregnant woman presenting with limb numbness, diagnosed with acute transverse myelitis (ATM) following COVID-19 vaccination. Notably, she tested positi...Purpose: This case report highlights a rare instance of a 26-week pregnant woman presenting with limb numbness, diagnosed with acute transverse myelitis (ATM) following COVID-19 vaccination. Notably, she tested positive for neuromyelitis optica (NMO) but did not exhibit the typical symptoms of blurred vision. Methods: Data were collected from I Seha in governmental hospitals in Bahrain, providing a comprehensive overview of the patient’s clinical journey. Results: The patient was treated with carbamazepine, steroids, and clexane, leading to the successful delivery of a healthy baby at 34 weeks. However, two years postpartum, she reported new episodes of blurred vision and numbness. Subsequent treatment with Rituximab resulted in significant improvement, with a diagnosis of Devic’s disease established. Conclusion: Acute transverse myelitis is a rare condition, particularly during pregnancy, and in this case, symptoms of Devic’s disease were masked by the absence of blurred vision. This condition predominantly affects females in their 20s during childbearing years and can lead to complications such as premature delivery. Effective management includes steroids and antiepileptic drugs, with clexane playing a crucial role in preventing embolisms. A delivery mode may vary based on obstetric factors, and MRI remains the gold standard for diagnosis. Close follow-up is essential for optimal patient outcomes.展开更多
文摘BACKGROUND：Devic＇s neuromyelitis optica （DNMO） and multiple sclerosis in Asian populations have been considered to be the same disease. However, there is an increasing number of studies suggesting that DNMO and multiple sclerosis are different diseases.OBJECTIVE：Little information is available regarding comparisons of DNMO patients between China and other countries, as well as clinical manifestations of Chinese patients with DNMO and multiple sclerosis. The present study performed a multi-center, pathological, retrospective analysis.DESIGN, TIME AND SETTING：A retrospective analysis of clinical data from seven patients with DNMO diagnosed between 1957 and 1998.PARTICIPANTS：Data from Chinese DNMO patients was provided by the Shanghai Second Medical University, Sun Yat-sen University of Medical Sciences and the First Affiliated Hospital of Harbin Medical University in China.METHODS：Clinical and pathological data from Chinese patients with DNMO were retrospectively analyzed. The clinical characteristics of DNMO were compared between Chinese and Caucasian patients. In addition, clinical and pathological differences between DNMO and multiple sclerosis Chinese patients were compared.MAIN OUTCOME MEASURES：Clinical and pathological features of Chinese patients with DNMO.RESULTS：All seven Chinese patients with DNMO exhibited abrupt onset of vision disturbance, with a disease course of 3 clays to 9 years. DNMO recurred in two of the patients. Demyelinating lesions were observed in all patients, with necrotic lesions and gitter cells in five patients, collagenous hyperplasia in one patient, and perivascular inflammatory cell infiltration in six patients. Comparison between Chinese and Caucasian DNMO patients revealed no significant differences in age at onset, clinical onset, duration, or interval between optic neuritis and myelitis. Compared with Chinese multiple sclerosis patients, Chinese DNMO patients presented with fewer recurrences, higher occurrence of necrosis, perivascular inflammatory cell infiltration and gitter cells, and a lower occurrence of collagenous hyperplasia.CONCLUSION：There was no difference in DNMO clinical features between Chinese and Caucasian patients. However, the clinical and pathological features of DNMO were different compared with multiple sclerosis in Chinese patients. Results suggested that the characteristics of DNMO in Chinese patients were significantly different than multiple sclerosis.
文摘Devic’s neuromyelitis optica (NMO) is a rare inflammatory disease of the central nervous system that results in optic neuropathy and myelitis. Optic neuritis represents the mode of entry into the disease in more than two thirds of cases. It is a rare entity in children. There is no effective treatment at present, but some molecules can be used, such as corticosteroids, immunosuppressants and plasma exchange. The prognosis in children is generally favorable. Devic’s neuromyelitis is a condition of unknown etiopathogeny which is functionally critical and requires early and appropriate treatment. We report the case of a 12-year-old girl who presented to emergency with a headache and decreased visual acuity, whose investigations led to the diagnosis of Devic’s neuromyelitis optica.
基金supported by the Romanian Ministry of Education and Research(Research project:Alternative therapies for major tissue defects 42136/01.10.2008)
文摘Recent studies provided evidence that mesenchymal stem cells（MSCs） have regenerative potential in cutaneous repair and profound immunomodulatory properties making them a candidate for therapy of neuroimmunologic diseases. Neuromyelitis optica（NMO） is an autoimmune, demyelinating central nervous system disorder characterized by a longitudinally extensive spinal cord lesion. A 46-year-old male diagnosed with NMO had relapses with paraplegia despite treatment and developed two stage IV pressure ulcers（PUs） on his legs. The patient consented for local application of autologous MSCs on PUs. MSCs isolated from the patient＇s bone marrow aspirate were multiplied in vitro during three passages and embedded in a tridimensional collagen-rich matrix which was applied on the PUs. Eight days after MSCs application the patient showed a progressive healing of PUs and improvement of disability. Two months later the patient was able to walk 20 m with bilateral assistance and one year later he started to walk without assistance. For 76 months the patient had no relapse and no adverse event was reported. The original method of local application of autologous BM-MSCs contributed to healing of PUs. For 6 years the patient was free of relapses and showed an improvement of disability. The association of cutaneous repair, sustained remission of NMO and improvement of disability might be explained by a promotion/optimization of recovery mechanisms in the central nervous system even if alternative hypothesis should be considered. Further studies are needed to assess the safety and efficacy of mesenchymal stem cells in NMO treatment.
文摘Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with （n = 6） or without primary Sjogreffs syndrome （n = 10）. All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren＇s-syndrome-related antigen A an- tibodies, anti-Sjogren＇s-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren＇s syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% （4/6） of patients with primary Sjogren＇s syndrome and in 60% （6/10） of patients without primary Sj6gren＇s syndrome. More brain abnormalities were observed in patients without primary Sj6gren＇s syndrome than in those with primary Sj6gren＇s syndrome. Segments lesions （〉 3 centrum） were noted in 50% （5/10） of patients without primary Sj6gren＇s syndrome and in 67% （4/6） of patients with primary Sjogren＇s syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.
文摘Neuromyelitis optica(NMO)refers to an antibody mediated,inflammatory disorder of the central nervous system(CNS)characterized by recurrent or monophasic attacks of optic neuritis and myelitis.Most patients with NMO possess a specific serum immunoglobin,NMO-IgG,which can serve as a biomarker for NMO.The autoantibodies target aquaporin-4(AQP4),the main water channel protein found in the CNS including the brain,spinal cord,and optic nerve.The remaining 10-25%of patients are seronegative for NMO-IgG despite meeting the diagnostic criteria for NMO.Recent studies have shown that a subset of these patients is seropositive for antibodies against myelin oligodendrocyte glycoprotein(MOG).This paper will provide an overview of the current English scientific literature published regarding the history,epidemiology,AQP4 biomarker,MOG biomarker,diagnosis,clinical features,related diseases in NMO spectrum disorder(NMOSD),and treatments of NMO.
文摘Background: In Iranian patients with opticospinal multiple sclerosis (OSMS), a paucity of brain lesions and short spinal cord lesions extending less than three spinal segments are characteristic findings on magnetic resonance imaging (MRI). It also shows a relatively benign course with negative CSF oligo-coonal bands. Objective: We aimed to clarify the possible relationship between clinical phenotype and MRI features of OSMS and human leucocyte antigen (HLA) system in Iran. Methods: Genotyping of HLA class II allele frequencies in 20 patients with OSMS were done, using polymerase chain reaction sequence-specific primer amplification method. Blood samples were extracted and typed for HLA-DRB, DQA, and DQB loci and compared with 100 controls. Results: Significant positive association was observed in DRB1*03, DQA1*0201, DQA1*03, DQB1*0201, and DQB1*0611, while DQB1*0602 was absent in our patients. Conclusion: These finding suggest that HLA-DRB association pattern in OSMS is different from conventional MS in Iran which is mostly associated with DRB1*1501 and from similar Japanese OSMS who are negative for brain lesions fulfilling the Barkhof criteria and negative for the presence of longitudinally extensive spinal cord lesions who carries the DRB*0405 allele. OSMS is immunogenetically heterogeneous. Also absence of DQB1*0602 allele may negatively be associated with the absence of Barkhof brain lesion.
文摘Optic neuritis is the most common cause of unilateral visual loss associated with orbital pain on ocular movements and impaired colour vision. It can present with anterior optic neuritis, papillitis, a swollen optic disc, retrobulbar neuritis with a normal optic disc, neuritis with oedema of the optic disc and the macular star or anterior ischemic neuropathy. It may be the only manifestation of a disease such as demyelination or associated with systemic diseases such as systemic lupus, giant cell arteritis, Sjogren’s syndrome, herpes simplex, herpes zoster, Lyme disease, CMV, EBV, HIV, toxoplasmosis, West Nile virus, Chikungunya, dengue fever, Rift Valley fever, mumps, rubella, measles, cat scratch disease, tuberculosis, syphilis, rickettsia illnesses, Q fever, Whipple disease, brucellosis, leptospirosis, leprosy, toxocariasis, malaria, Cryptococcus, candidiasis, histoplasmosis, Aspergillosis, mucormycosis, Bacillus Chalmette-Guerin (BCG) vaccination, ethanol and methanol toxicity, sarcoidosis, neuromyelitis optica and Liber’s hereditary optic neuropathy. A systemic approach to the diagnosis is of paramount importance in confirming, diagnosing and treating optic neuritis and underlying systemic diseases to provide definitive cures. Failure to diagnose optic neuritis and treat the cause may result in optic atrophy and permanent blindness. In this paper, we reviewed the diagnosis and differential diagnoses of optic neuritis, including arteritis, ischemic, neuroretina, and vasculitic causes.
文摘Purpose: This case report highlights a rare instance of a 26-week pregnant woman presenting with limb numbness, diagnosed with acute transverse myelitis (ATM) following COVID-19 vaccination. Notably, she tested positive for neuromyelitis optica (NMO) but did not exhibit the typical symptoms of blurred vision. Methods: Data were collected from I Seha in governmental hospitals in Bahrain, providing a comprehensive overview of the patient’s clinical journey. Results: The patient was treated with carbamazepine, steroids, and clexane, leading to the successful delivery of a healthy baby at 34 weeks. However, two years postpartum, she reported new episodes of blurred vision and numbness. Subsequent treatment with Rituximab resulted in significant improvement, with a diagnosis of Devic’s disease established. Conclusion: Acute transverse myelitis is a rare condition, particularly during pregnancy, and in this case, symptoms of Devic’s disease were masked by the absence of blurred vision. This condition predominantly affects females in their 20s during childbearing years and can lead to complications such as premature delivery. Effective management includes steroids and antiepileptic drugs, with clexane playing a crucial role in preventing embolisms. A delivery mode may vary based on obstetric factors, and MRI remains the gold standard for diagnosis. Close follow-up is essential for optimal patient outcomes.
