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Lineu Cesar Werneck - One of the best experts on this subject based on the ideXlab platform.
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neuromyotonia an unusual presentation in Inflammatory Myopathy p2 045
Neurology, 2015Co-Authors: Rosana Herminia Scola, Paulo José Lorenzoni, Renata Dalpra Ducci, Lineu Cesar WerneckAbstract:OBJECTIVE: To report the association between neuromyotonia and Inflammatory Myopathy. BACKGROUND: Neuromyotonia is a rare condition with spontaneous and continuous muscle fiber activity of peripheral nerve origin. Clinically is characterized by muscle twitching at rest (visible myokymia), cramps and impaired muscle relaxation (pseudomyotonia) associated with excessive sweating. Neuromyotonia can be immune mediated, as well as, occurs isolated or in association with other disorders, such as neoplasia, auto-immune disorders, anterior horn cell degeneration and Schwartz-Jampel syndrome. Neuromyotonia associated to Inflammatory Myopathy has rarely been documented. DESIGN/METHODS: Case Report RESULTS:A 60-year-old woman presented with dysphagia and weaknessin lower limbs, which did not improve with prednisone 60mg/day after one year. Neurologic examination revealed proximal weakness in lower limbs (MRC grade 4-). Laboratorial tests showed: CK 4,163U/L (Normal: 30-200), erythrocyte sedimentation rate 72mm/h (Normal: <30). Neurophysiologic study showed motor axonal/demyelinating polyneuropathy, myopathic potentials and neuromyotonia discharges. Muscle biopsy revealed Inflammatory Myopathy compatible with polymyositis. Extensive research for malignancy was negative. The diagnosis of Inflammatory Myopathy with neuromyotonia was made and immunosuppressive therapy (cyclophosphamide and prednisone) was done. CONCLUSIONS. Neuromyotonia and Inflammatory Myopathy is a very rare association and in our case the patient had atypical clinical manifestations. The diagnosis of neuromyotonia was made with neurophysiologic study. The patient received immunosuppressive treatment, whereas she did not have other clinical manifestations of neuromyotonia. Neuromyotonia is an unusual presentation in Inflammatory Myopathy. Disclosure: Dr. Scola has nothing to disclose. Dr. Ducci has nothing to disclose. Dr. Lorenzoni has nothing to disclose. Dr. Werneck has nothing to disclose.
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Neuromyotonia: an Unusual Presentation in Inflammatory Myopathy (P2.045)
Neurology, 2015Co-Authors: Rosana Herminia Scola, Paulo José Lorenzoni, Renata Dalpra Ducci, Lineu Cesar WerneckAbstract:OBJECTIVE: To report the association between neuromyotonia and Inflammatory Myopathy. BACKGROUND: Neuromyotonia is a rare condition with spontaneous and continuous muscle fiber activity of peripheral nerve origin. Clinically is characterized by muscle twitching at rest (visible myokymia), cramps and impaired muscle relaxation (pseudomyotonia) associated with excessive sweating. Neuromyotonia can be immune mediated, as well as, occurs isolated or in association with other disorders, such as neoplasia, auto-immune disorders, anterior horn cell degeneration and Schwartz-Jampel syndrome. Neuromyotonia associated to Inflammatory Myopathy has rarely been documented. DESIGN/METHODS: Case Report RESULTS:A 60-year-old woman presented with dysphagia and weaknessin lower limbs, which did not improve with prednisone 60mg/day after one year. Neurologic examination revealed proximal weakness in lower limbs (MRC grade 4-). Laboratorial tests showed: CK 4,163U/L (Normal: 30-200), erythrocyte sedimentation rate 72mm/h (Normal:
J. Van De Vlekkert - One of the best experts on this subject based on the ideXlab platform.
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Combining MRI and muscle biopsy improves diagnostic accuracy in subacute-onset idiopathic Inflammatory Myopathy.
Muscle & nerve, 2015Co-Authors: J. Van De Vlekkert, M. De Visser, Mario Maas, Jessica E. Hoogendijk, Ivo N. Van SchaikAbstract:Introduction: In 10-20% of patients with subacute- onset idiopathic Inflammatory Myopathy (IIM), muscle biopsy is normal or shows nonspecific findings. MRI can be used as a tri- age test before muscle biopsy and as an add-on test if the biopsy is nondiagnostic. Methods: MRI scans of skeletal muscles and muscle biopsies were evaluated prospectively in 48 patients suspected to have IIM. The interpretations of MRI and muscle biopsy were compared with the definite diagnosis (based on European Neuromuscular Centre criteria and response to corticosteroids). Results: The false negative rate (FNR) of all muscle biopsies was 0.23. Biopsies of a muscle showing hyperintensity on MRI (as triage test) had an FNR of 0.19. The result of MRI as an add-on test in patients with a nondiagnostic muscle biopsy decreased the FNR from 0.23 to 0.06. Conclusions: We recommend both MRI and muscle biopsy in patients suspected of having IIM. Muscle Nerve 51: 253-258, 2015 Adult subacute-onset idiopathic Inflammatory myopathies (IIM) are subclassified into polymyosi- tis (PM), dermatomyositis (DM), nonspecific myo- sitis (NSM), and necrotizing autoimmune
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P.21.4 Combining MRI and muscle biopsy improves diagnostic accuracy in subacute-onset idiopathic Inflammatory Myopathy
Neuromuscular Disorders, 2013Co-Authors: M. De Visser, J. Van De Vlekkert, Mario Maas, Jessica E. Hoogendijk, I.n. SchaikAbstract:In approximately 10–20% of patients suffering from subacute-onset idiopathic Inflammatory Myopathy (IIM) muscle biopsy is normal or shows non-specific findings. MRI can be used as triage test prior to a muscle biopsy and as add-on test if a muscle biopsy has proven to be non-diagnostic. A prospective study was performed on forty-eight consecutive patients with subacute-onset IIM of whom MRI scans of the skeletal muscles and muscle biopsies were systematically evaluated. The results of MRI, muscle biopsy findings and definite diagnosis including response to treatment which was considered the gold standard were collected and compared. The false negative rate of all muscle biopsies was 0.23. Biopsies taken from a muscle showing hyperintensity on MRI (as triage test) had a false negative rate of 0.19. Using the result of MRI as add-on test in patients with a non-diagnostic muscle biopsy decreased the false negative rate to 0.06. MRI has a high diagnostic accuracy as add-on test to the muscle biopsy in patients with a negative or non-specific muscle biopsy but otherwise a clinical presentation consistent with a diagnosis of subacute-onset IIM. Our results also suggest that MRI may be useful as triage test in reducing false negative muscle biopsy results. Therefore, in patients presumed to have a subacute-onset IIM it is recommended to perform both MRI and a muscle biopsy.
Rosana Herminia Scola - One of the best experts on this subject based on the ideXlab platform.
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neuromyotonia an unusual presentation in Inflammatory Myopathy p2 045
Neurology, 2015Co-Authors: Rosana Herminia Scola, Paulo José Lorenzoni, Renata Dalpra Ducci, Lineu Cesar WerneckAbstract:OBJECTIVE: To report the association between neuromyotonia and Inflammatory Myopathy. BACKGROUND: Neuromyotonia is a rare condition with spontaneous and continuous muscle fiber activity of peripheral nerve origin. Clinically is characterized by muscle twitching at rest (visible myokymia), cramps and impaired muscle relaxation (pseudomyotonia) associated with excessive sweating. Neuromyotonia can be immune mediated, as well as, occurs isolated or in association with other disorders, such as neoplasia, auto-immune disorders, anterior horn cell degeneration and Schwartz-Jampel syndrome. Neuromyotonia associated to Inflammatory Myopathy has rarely been documented. DESIGN/METHODS: Case Report RESULTS:A 60-year-old woman presented with dysphagia and weaknessin lower limbs, which did not improve with prednisone 60mg/day after one year. Neurologic examination revealed proximal weakness in lower limbs (MRC grade 4-). Laboratorial tests showed: CK 4,163U/L (Normal: 30-200), erythrocyte sedimentation rate 72mm/h (Normal: <30). Neurophysiologic study showed motor axonal/demyelinating polyneuropathy, myopathic potentials and neuromyotonia discharges. Muscle biopsy revealed Inflammatory Myopathy compatible with polymyositis. Extensive research for malignancy was negative. The diagnosis of Inflammatory Myopathy with neuromyotonia was made and immunosuppressive therapy (cyclophosphamide and prednisone) was done. CONCLUSIONS. Neuromyotonia and Inflammatory Myopathy is a very rare association and in our case the patient had atypical clinical manifestations. The diagnosis of neuromyotonia was made with neurophysiologic study. The patient received immunosuppressive treatment, whereas she did not have other clinical manifestations of neuromyotonia. Neuromyotonia is an unusual presentation in Inflammatory Myopathy. Disclosure: Dr. Scola has nothing to disclose. Dr. Ducci has nothing to disclose. Dr. Lorenzoni has nothing to disclose. Dr. Werneck has nothing to disclose.
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Neuromyotonia: an Unusual Presentation in Inflammatory Myopathy (P2.045)
Neurology, 2015Co-Authors: Rosana Herminia Scola, Paulo José Lorenzoni, Renata Dalpra Ducci, Lineu Cesar WerneckAbstract:OBJECTIVE: To report the association between neuromyotonia and Inflammatory Myopathy. BACKGROUND: Neuromyotonia is a rare condition with spontaneous and continuous muscle fiber activity of peripheral nerve origin. Clinically is characterized by muscle twitching at rest (visible myokymia), cramps and impaired muscle relaxation (pseudomyotonia) associated with excessive sweating. Neuromyotonia can be immune mediated, as well as, occurs isolated or in association with other disorders, such as neoplasia, auto-immune disorders, anterior horn cell degeneration and Schwartz-Jampel syndrome. Neuromyotonia associated to Inflammatory Myopathy has rarely been documented. DESIGN/METHODS: Case Report RESULTS:A 60-year-old woman presented with dysphagia and weaknessin lower limbs, which did not improve with prednisone 60mg/day after one year. Neurologic examination revealed proximal weakness in lower limbs (MRC grade 4-). Laboratorial tests showed: CK 4,163U/L (Normal: 30-200), erythrocyte sedimentation rate 72mm/h (Normal:
M. De Visser - One of the best experts on this subject based on the ideXlab platform.
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Combining MRI and muscle biopsy improves diagnostic accuracy in subacute-onset idiopathic Inflammatory Myopathy.
Muscle & nerve, 2015Co-Authors: J. Van De Vlekkert, M. De Visser, Mario Maas, Jessica E. Hoogendijk, Ivo N. Van SchaikAbstract:Introduction: In 10-20% of patients with subacute- onset idiopathic Inflammatory Myopathy (IIM), muscle biopsy is normal or shows nonspecific findings. MRI can be used as a tri- age test before muscle biopsy and as an add-on test if the biopsy is nondiagnostic. Methods: MRI scans of skeletal muscles and muscle biopsies were evaluated prospectively in 48 patients suspected to have IIM. The interpretations of MRI and muscle biopsy were compared with the definite diagnosis (based on European Neuromuscular Centre criteria and response to corticosteroids). Results: The false negative rate (FNR) of all muscle biopsies was 0.23. Biopsies of a muscle showing hyperintensity on MRI (as triage test) had an FNR of 0.19. The result of MRI as an add-on test in patients with a nondiagnostic muscle biopsy decreased the FNR from 0.23 to 0.06. Conclusions: We recommend both MRI and muscle biopsy in patients suspected of having IIM. Muscle Nerve 51: 253-258, 2015 Adult subacute-onset idiopathic Inflammatory myopathies (IIM) are subclassified into polymyosi- tis (PM), dermatomyositis (DM), nonspecific myo- sitis (NSM), and necrotizing autoimmune
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P.21.4 Combining MRI and muscle biopsy improves diagnostic accuracy in subacute-onset idiopathic Inflammatory Myopathy
Neuromuscular Disorders, 2013Co-Authors: M. De Visser, J. Van De Vlekkert, Mario Maas, Jessica E. Hoogendijk, I.n. SchaikAbstract:In approximately 10–20% of patients suffering from subacute-onset idiopathic Inflammatory Myopathy (IIM) muscle biopsy is normal or shows non-specific findings. MRI can be used as triage test prior to a muscle biopsy and as add-on test if a muscle biopsy has proven to be non-diagnostic. A prospective study was performed on forty-eight consecutive patients with subacute-onset IIM of whom MRI scans of the skeletal muscles and muscle biopsies were systematically evaluated. The results of MRI, muscle biopsy findings and definite diagnosis including response to treatment which was considered the gold standard were collected and compared. The false negative rate of all muscle biopsies was 0.23. Biopsies taken from a muscle showing hyperintensity on MRI (as triage test) had a false negative rate of 0.19. Using the result of MRI as add-on test in patients with a non-diagnostic muscle biopsy decreased the false negative rate to 0.06. MRI has a high diagnostic accuracy as add-on test to the muscle biopsy in patients with a negative or non-specific muscle biopsy but otherwise a clinical presentation consistent with a diagnosis of subacute-onset IIM. Our results also suggest that MRI may be useful as triage test in reducing false negative muscle biopsy results. Therefore, in patients presumed to have a subacute-onset IIM it is recommended to perform both MRI and a muscle biopsy.
Geraldine Cambridge - One of the best experts on this subject based on the ideXlab platform.
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Clinical outcome following B cell depletion therapy in eight patients with refractory idiopathic Inflammatory Myopathy.
Clinical and Experimental Rheumatology, 2008Co-Authors: S. M. Sultan, David A. Isenberg, Kristine P. Ng, Jcw Edwards, Geraldine CambridgeAbstract:OBJECTIVE: To assess the efficacy of B lymphocyte depletion therapy (BCDT) in patients with refractive idiopathic Inflammatory Myopathy (IIM). METHODS: Eight patients thought to have IIM were treated with BCDT utilising rituximab. Five were treated as part of an open label trial and three on the basis of perceived clinical need. Rituximab (1 gram) and methylprednisolone (100 mg) were given as intravenous infusions on days 0 and 14. The primary efficacy outcome at 6 months was 15% improvement in muscle strength and 30% reduction in CPK. RESULTS: Two patients with Jo-1 antibody positive dermatomyositis (DM) demonstrated a clinical response. Both achieved >30% improvement in CPK. In one, the CPK remained within the normal range for 10 months, the other had a normalised CPK and stabilisation of lung function tests for 36 months. Muscle strength by myometry, however, did not achieve the primary outcome, although, patient 1, demonstrated an improvement of 20% at 8 months (the patient had elective surgery of the hand during the study period). Jo-1 antibody levels fell modestly in both patients but remained detectable. Re-evaluation of three patients revealed that one had inclusion body myositis, one had sporadic muscular dystrophy and one subsequently developed nodular sclerosing lymphoma. All except one patient showed adequate B cell depletion with re-population occurring 3- >42 months after BCDT. One patient did not deplete and died of an unrelated cause. CONCLUSIONS: This study emphasizes the importance of identifying and selecting the appropriate sub-group of patients with IIM most likely to respond to BCDT.
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Clinical outcome following B cell depletion therapy in eight patients with refractory idiopathic Inflammatory Myopathy.
Clinical and Experimental Rheumatology, 2008Co-Authors: S. M. Sultan, David A. Isenberg, Kristine P. Ng, Jcw Edwards, Geraldine CambridgeAbstract:OBJECTIVE: To assess the efficacy of B lymphocyte depletion therapy (BCDT) in patients with refractive idiopathic Inflammatory Myopathy (IIM). METHODS: Eight patients thought to have IIM were treated with BCDT utilising rituximab. Five were treated as part of an open label trial and three on the basis of perceived clinical need. Rituximab (1 gram) and methylprednisolone (100 mg) were given as intravenous infusions on days 0 and 14. The primary efficacy outcome at 6 months was 15% improvement in muscle strength and 30% reduction in CPK. RESULTS: Two patients with Jo-1 antibody positive dermatomyositis (DM) demonstrated a clinical response. Both achieved >30% improvement in CPK. In one, the CPK remained within the normal range for 10 months, the other had a normalised CPK and stabilisation of lung function tests for 36 months. Muscle strength by myometry, however, did not achieve the primary outcome, although, patient 1, demonstrated an improvement of 20% at 8 months (the patient had elective surgery of the hand during the study period). Jo-1 antibody levels fell modestly in both patients but remained detectable. Re-evaluation of three patients revealed that one had inclusion body myositis, one had sporadic muscular dystrophy and one subsequently developed nodular sclerosing lymphoma. All except one patient showed adequate B cell depletion with re-population occurring 3- >42 months after BCDT. One patient did not deplete and died of an unrelated cause. CONCLUSIONS: This study emphasizes the importance of identifying and selecting the appropriate sub-group of patients with IIM most likely to respond to BCDT.