Lambert-Eaton myasthenic syndrome(LEMS) is a paraneoplastic syndrome and only 3%of small cell lung carcinoma(SCLC) patients have LEMS.Moreover,the recurrence of SCLC after a disease-free survival(DFS) of more than 10 ...Lambert-Eaton myasthenic syndrome(LEMS) is a paraneoplastic syndrome and only 3%of small cell lung carcinoma(SCLC) patients have LEMS.Moreover,the recurrence of SCLC after a disease-free survival(DFS) of more than 10 years is rare.We report a patient who had a recurrence of both SCLC and LEMS after a 13-year DFS period.A 69-year-old man was diagnosed with LEMS and SCLC(cT0N2M0,stage ⅢA) 13 years ago.Chemoradiotherapy was performed and a complete response was achieved.With anticancer treatment,the LEMS symptoms was alleviated.At the age of 82 years,gait disturbance appeared followed by left supraclavicular lymphadenopathy and further examination revealed the recurrence of SCLC.Careful screening for the recurrence of SCLC might be needed when the patient has recurrent or secondary paraneoplastic neurological syndrome even after a long DFS period.展开更多
The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding...The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding to the association of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). He also presented concomitant myasthenia gravis and thymoma. The physical examination notes only a disturbed voice. The liver tests showed: alkaline phosphatase 600 U/l, alanine aminotransferase 1280 U/l, and aspartate aminotransferase 985 U/l. Viral serologies of hepatitis were all negative, as well as antinuclear, antimitochondrial and antismooth muscle antibodies. The diagnosis of an overlap syndrome was posed according to the biological and hepatic histhological results. An associated myasthenia was confirmed by the results of the EMG and the positivity of the anti-acetylcholine receptor antibody. A chest CT revealed a thymoma. The treatment consisted of ursodeoxycholic acid, prednisone, azathioprine and pyridostigmine. And the patient improved his voice and the liver function. Thymectomy was practiced without incidents.展开更多
Muscle Specific Receptor Thyrosine Kinase (MuSK) Myasthenia Gravis (MG) is a rare auto-immune disorder of the neuromuscular junction. The clinical presentation of MG is dominated by fluctuating weakness of the extra-o...Muscle Specific Receptor Thyrosine Kinase (MuSK) Myasthenia Gravis (MG) is a rare auto-immune disorder of the neuromuscular junction. The clinical presentation of MG is dominated by fluctuating weakness of the extra-ocular, orofacial and limb muscles. The clinical presentation of MuSK MG can vary, which may delay diagnostic procedures. We present a patient who initially presented with severe weight loss and slowly progressive developing neck extensor weakness and diplopia. A single fiber EMG led to the diagnosis MG and antibodies directed towards MuSK were detected. He was treated with prednisone and the steroid sparing agent azathioprine after which he made full recovery.展开更多
Purpose: To report an unusual case of Miller Fisher Syndrome (MFS) in which the patient presented with concurrent right eye uveitis. Case Report: We report a case of a 51-year-old gentleman who presented with typical ...Purpose: To report an unusual case of Miller Fisher Syndrome (MFS) in which the patient presented with concurrent right eye uveitis. Case Report: We report a case of a 51-year-old gentleman who presented with typical clinical features of Miller Fisher syndrome including ophthalmoplegia, ataxia, areflexia, ptosis and diplopia following an upper respiratory tract infection. Concurrently, he also had right eye uveitis with raised intraocular pressure. The patient was treated with intravenous immunoglobulin (IVIG), topical steroids and anti-glaucoma eye drops in which he demonstrated good recovery. For diagnostic confirmation, serum antiganglioside antibodies (anti-GQ1b IgG) were later reported to be positive. Conclusion: We described the possible association between MFS and uveitis due to its interrelated pathogenesis. This possible association can lead to early detection and treatment of uveitis.展开更多
OBJECTIVE: To study the use of Jianjining in the treatment of myasthenia gravis (MG) patients with a deficiency of both spleen and kidney via the theory on the combination of disease with syndrome. METHODS: Sixty MG p...OBJECTIVE: To study the use of Jianjining in the treatment of myasthenia gravis (MG) patients with a deficiency of both spleen and kidney via the theory on the combination of disease with syndrome. METHODS: Sixty MG patients with a deficiency of both spleen and kidney were randomly divided into an treatment group (n=30) treated with Jianjining granules and Western Medicine (prednisone or pyridostigmine bromide) and a control group (n= 30) treated with Jianjining granules. The dosage of the three drugs was reduced over the course of treatment. After 3 and 6 months of treatment, the curative effect was evaluated with the muscle weakness severity scale (MWSS). RESULTS: The MWSS score after treatment declined significantly in both groups. The score in the treatment group was much lower than that in thecontrol group (P<0.05). The total effective rate was 63.33% (19/30) in the treatment group and 36.67% (11/30) in the control group after 3 months of treatment, and 80.00% (24/30) and 50.00% (15/30), respectively, after 6 months of treatment. The obvious and total effective rates in the treatment group were much higher than those in the control group (P<0.05). After 6 months of treatment, there were no obvious differences (P>0.05) in the obvious and effective rates between the 2 groups. However, the total effective rate in the treatment group was much higher than that in the control group (P<0.01). CONCLUSION: Using the theory on the combination of disease with syndrome, we found that the curative effect of Jianjining and Western Medicine on MG patients with deficiency of both spleen and kidney is worth further exploration.展开更多
文摘Lambert-Eaton myasthenic syndrome(LEMS) is a paraneoplastic syndrome and only 3%of small cell lung carcinoma(SCLC) patients have LEMS.Moreover,the recurrence of SCLC after a disease-free survival(DFS) of more than 10 years is rare.We report a patient who had a recurrence of both SCLC and LEMS after a 13-year DFS period.A 69-year-old man was diagnosed with LEMS and SCLC(cT0N2M0,stage ⅢA) 13 years ago.Chemoradiotherapy was performed and a complete response was achieved.With anticancer treatment,the LEMS symptoms was alleviated.At the age of 82 years,gait disturbance appeared followed by left supraclavicular lymphadenopathy and further examination revealed the recurrence of SCLC.Careful screening for the recurrence of SCLC might be needed when the patient has recurrent or secondary paraneoplastic neurological syndrome even after a long DFS period.
文摘The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding to the association of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). He also presented concomitant myasthenia gravis and thymoma. The physical examination notes only a disturbed voice. The liver tests showed: alkaline phosphatase 600 U/l, alanine aminotransferase 1280 U/l, and aspartate aminotransferase 985 U/l. Viral serologies of hepatitis were all negative, as well as antinuclear, antimitochondrial and antismooth muscle antibodies. The diagnosis of an overlap syndrome was posed according to the biological and hepatic histhological results. An associated myasthenia was confirmed by the results of the EMG and the positivity of the anti-acetylcholine receptor antibody. A chest CT revealed a thymoma. The treatment consisted of ursodeoxycholic acid, prednisone, azathioprine and pyridostigmine. And the patient improved his voice and the liver function. Thymectomy was practiced without incidents.
文摘Muscle Specific Receptor Thyrosine Kinase (MuSK) Myasthenia Gravis (MG) is a rare auto-immune disorder of the neuromuscular junction. The clinical presentation of MG is dominated by fluctuating weakness of the extra-ocular, orofacial and limb muscles. The clinical presentation of MuSK MG can vary, which may delay diagnostic procedures. We present a patient who initially presented with severe weight loss and slowly progressive developing neck extensor weakness and diplopia. A single fiber EMG led to the diagnosis MG and antibodies directed towards MuSK were detected. He was treated with prednisone and the steroid sparing agent azathioprine after which he made full recovery.
文摘Purpose: To report an unusual case of Miller Fisher Syndrome (MFS) in which the patient presented with concurrent right eye uveitis. Case Report: We report a case of a 51-year-old gentleman who presented with typical clinical features of Miller Fisher syndrome including ophthalmoplegia, ataxia, areflexia, ptosis and diplopia following an upper respiratory tract infection. Concurrently, he also had right eye uveitis with raised intraocular pressure. The patient was treated with intravenous immunoglobulin (IVIG), topical steroids and anti-glaucoma eye drops in which he demonstrated good recovery. For diagnostic confirmation, serum antiganglioside antibodies (anti-GQ1b IgG) were later reported to be positive. Conclusion: We described the possible association between MFS and uveitis due to its interrelated pathogenesis. This possible association can lead to early detection and treatment of uveitis.
基金Supported by a Key Project in Basic Research of Shanghai Municipal Committee of Science(No.10JC1414500)a Planned Project in the TCM Fund of Scientific Research under the Shanghai Municipal Health Bureau (2010J002B)
文摘OBJECTIVE: To study the use of Jianjining in the treatment of myasthenia gravis (MG) patients with a deficiency of both spleen and kidney via the theory on the combination of disease with syndrome. METHODS: Sixty MG patients with a deficiency of both spleen and kidney were randomly divided into an treatment group (n=30) treated with Jianjining granules and Western Medicine (prednisone or pyridostigmine bromide) and a control group (n= 30) treated with Jianjining granules. The dosage of the three drugs was reduced over the course of treatment. After 3 and 6 months of treatment, the curative effect was evaluated with the muscle weakness severity scale (MWSS). RESULTS: The MWSS score after treatment declined significantly in both groups. The score in the treatment group was much lower than that in thecontrol group (P<0.05). The total effective rate was 63.33% (19/30) in the treatment group and 36.67% (11/30) in the control group after 3 months of treatment, and 80.00% (24/30) and 50.00% (15/30), respectively, after 6 months of treatment. The obvious and total effective rates in the treatment group were much higher than those in the control group (P<0.05). After 6 months of treatment, there were no obvious differences (P>0.05) in the obvious and effective rates between the 2 groups. However, the total effective rate in the treatment group was much higher than that in the control group (P<0.01). CONCLUSION: Using the theory on the combination of disease with syndrome, we found that the curative effect of Jianjining and Western Medicine on MG patients with deficiency of both spleen and kidney is worth further exploration.
