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The initial electrodiagnostic signs that distinguish acute inflammatory demyelinating polyneuropathy from acute motor axonal neuropathy
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Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
attending the NMD clinic underwent phenotypic characterisation, muscle immunohistochemistry (IHC) and immunoblotting (IB). Results: 280 cases biopsied, 226 had IHC and 176 of these IB. 54 patients excluded. Consanguinity—45.2%. 200 confirmed to have specific ARLGMD by IHC and/or IB. Commonest form:LGMD 2B (82/246– 33.33%). Mean age of onset—21.17 ± 6.32 (8–41 years). Mean duration—7.24 ± 5.86 (1–36 years). Mean CK—7966.7 ± 6029.6 IU/L(131– 24037). All demonstrated dysferlin deficiency on IHC/IB. Second commonest-LGMD 2I (51/246–20.73%). Mean age of onset—12.64 ± 7.17 (1–29 years). Mean duration—8.54 ± 6.50 (1–27 years) Mean CK—4059.56 ± 3210.8 (211–14667 IU/L). α-DG deficiency by IHC-9, IB-51. Third commonest LGMD 2C-F (35/246–14.23%). All confirmed by IHC/IB. Mean age of onset—5.89 ± 3.45(1–20 years). Mean duration— 4.56 ± 2.85 (1–12 years). Mean CK—8688.31 ± 6113.86 IU/L (684– 23577). Fourth group-LGMD 2A (25/246–10.16%). Age of onset— 15.52 ± 11.18 (2–41 years). Mean duration—9.84 ± 9.33 (2–37 years). Mean CK—2742.58 ± 2144.96 IU/L (286–9018). All confirmed by IB. Fifth group-LGMD 2G (8/246–3.25%). Mean age of onset—12.38 ±11.35 (5 to 40 years). Mean duration—8.50 ± 6.87 (2–23 years). CK-718-9253 IU/L (mean ± SD; 2574.4 ± 2847.52). Proximo-distal form with muscle atrophy, calf hypertrophy, foot drop. The course was slow in majority. All confirmed on IB. Last group-LGMD 2J-2 cases. Confirmed on IHC. Conclusion: Our study confirms that LGMD 2B is the most common form of ARLGMD among our cohort. Further LGMD2I and 2G have a wider existence and may be among the common ARLGMDs in Indian population. doi:10.1016/j.jns.2013.07.1537
Abstract - WCN 2013 No: 763 Topic: 7 - Neuromuscular disorders The importance of follow-up thoracic imaging in myasthenia gravis patients H. Muraia, K. Utsugisawab, S. Suzukic, T. Imaid, Y. Naganeb, M. Masudae, S. Konnof, Y. Suzukig, S. Nakaneh, J.-I. Kiraa. aDepartment of Neurology, Kyushu University, Fukuoka, Japan; bHanamaki General Hospital, Hanamaki, Japan; cKeio University School of Medicine, Tokyo, Japan; d Sapporo Medical University School of Medicine, Sapporo, Japan; eTokyo Medical University, Tokyo, Japan; fToho University Medical Center Ohhashi Hospital, Tokyo, Japan; gNational Hospital Organization Sendai Medical Center, Sendai, Japan; hNational Hospital Organization Nagasaki Kawatana Medical Center, Kawatana, Japan Background: Approximately 20–30% of myasthenia gravis (MG) patients are accompanied by thymoma. In elderly patients, extra-thymic malignancies are also a matter of concern. Although most MG patients undergo thoracic imaging at the initial diagnosis, the follow-up protocol for thoracic imaging is not clear. Objective: To elucidate the follow-up status of thoracic imaging in MG. Patients and methods: From among 676 MG patients in 11 neurological centers, we evaluated 649 patients whose disease duration was over 1 year. The latest thoracic imaging (CT or MRI) after an interval of 1 year or more from the initial scan was evaluated in each case. The rate of abnormal findings and the details of findings in cases without thymoma (group N) and with thymoma (group T) were then investigated. Results: Thoracic imaging follow-up was performed in 337 (51.9%) of MG patients studied (48.8% in group N, 74.1% in group T). The average interval was 7.3 years in group N and 7.8 years in group T. Abnormal findings were detected in 9.0% in group N and 25.2% in group T. Abnormal findings included breast cancer, swelling of lymph nodes and respiratory system infection in group N, while recurrence or enlargement of thymoma accounted for 76.9% of the abnormal findings in group T.
Conclusion: It is important to keep following up thoracic imaging in MG patients since the rate of abnormal findings is high even 7 years after onset. doi:10.1016/j.jns.2013.07.1538
Abstract - WCN 2013 No: 719 Topic: 7 - Neuromuscular disorders The initial electrodiagnostic signs that distinguish acute inflammatory demyelinating polyneuropathy from acute motor axonal neuropathy C.S.J. Lima, N. Yukib, T. Umapathic. aYong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore; bDepartment of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore; cDepartment of Neurology, National Neuroscience Institute, Singapore, Singapore Background: Guillain–Barré syndrome (GBS) is divided into 2 neurophysiological subtypes, acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The early electrodiagnostic findings of AIDP overlap with AMAN, leading to the latter's under-diagnosis. Serial nerve conduction studies (NCSs) are more sensitive, but not always feasible. Objective: To derive diagnostic criteria that distinguish AMAN from AIDP at presentation, by delineating their nascent electrodiagnostic features. Patients and methods: We used at least 2 serial NCSs to categorise GBS patients into AIDP and AMAN. The initial NCS was scrutinized for features discriminating the 2 subtypes. Results: Using serial NCSs, 30 GBS patients were divided into 11 AIDP, 14 AMAN, and 5 unclassified cases. The initial NCS, done at median 7 and range 2-14 days, showed prolonged distal motor latency in 7 AIDP and 8 AMAN patients. Six AIDP patients had slowing in non-entrapment sites and 4 had temporal dispersion (TD). Only 1 AMAN patient had these. Slowing at entrapment site was less specific, occurring in 8 AIDP and 3 AMAN patients. Three AIDP patients had conduction block (CB), all with slowing across the block. Seven nerves in 5 AMAN patients had CB, 5 without slowing. Conclusion: The most specific initial signs of AIDP were slowing in nonentrapment sites and TD. CMAP reduction in at least 2 nerves without TD or slowing at non-entrapment sites and non-slowing CB predict AMAN. Using these features, 22 out of 30 patients were assigned to the right GBS subtypes; current criteria were correct in 14 patients. doi:10.1016/j.jns.2013.07.1539
Abstract - WCN 2013 No: 761 Topic: 7 - Neuromuscular disorders LGMD2I: Immunohistochemical and immunoblot technique assisted identification of 51 cases with both duchenne and becker phenotype S. Modia, A. Nalinia, N. Gayathrib, M.S. Bharathc, B. Sunithab, K. Polavarapua. a Department of Neurology, NIMHANS, Bengaluru, India; bDepartment of Neuropathology, NIMHANS, Bengaluru, India; cDepartment of Neurochemistry, NIMHANS, Bengaluru, India Background: LGMD 2I is caused by mutations in fukutin-related protein gene (FKRP). Objective: To describe the phenotype and findings of immunohistochemistry (IHC) and immunoblot (IB) in LGMD2I. Materials and methods: Prospective study of 300 cases of ARLGMD seen at Neuromuscular Disorders clinic between February 2010–
Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
attending the NMD clinic underwent phenotypic characterisation, muscle immunohistochemistry (IHC) and immunoblotting (IB). Results: 280 cases biopsied, 226 had IHC and 176 of these IB. 54 patients excluded. Consanguinity—45.2%. 200 confirmed to have specific ARLGMD by IHC and/or IB. Commonest form:LGMD 2B (82/246– 33.33%). Mean age of onset—21.17 ± 6.32 (8–41 years). Mean duration—7.24 ± 5.86 (1–36 years). Mean CK—7966.7 ± 6029.6 IU/L(131– 24037). All demonstrated dysferlin deficiency on IHC/IB. Second commonest-LGMD 2I (51/246–20.73%). Mean age of onset—12.64 ± 7.17 (1–29 years). Mean duration—8.54 ± 6.50 (1–27 years) Mean CK—4059.56 ± 3210.8 (211–14667 IU/L). α-DG deficiency by IHC-9, IB-51. Third commonest LGMD 2C-F (35/246–14.23%). All confirmed by IHC/IB. Mean age of onset—5.89 ± 3.45(1–20 years). Mean duration— 4.56 ± 2.85 (1–12 years). Mean CK—8688.31 ± 6113.86 IU/L (684– 23577). Fourth group-LGMD 2A (25/246–10.16%). Age of onset— 15.52 ± 11.18 (2–41 years). Mean duration—9.84 ± 9.33 (2–37 years). Mean CK—2742.58 ± 2144.96 IU/L (286–9018). All confirmed by IB. Fifth group-LGMD 2G (8/246–3.25%). Mean age of onset—12.38 ±11.35 (5 to 40 years). Mean duration—8.50 ± 6.87 (2–23 years). CK-718-9253 IU/L (mean ± SD; 2574.4 ± 2847.52). Proximo-distal form with muscle atrophy, calf hypertrophy, foot drop. The course was slow in majority. All confirmed on IB. Last group-LGMD 2J-2 cases. Confirmed on IHC. Conclusion: Our study confirms that LGMD 2B is the most common form of ARLGMD among our cohort. Further LGMD2I and 2G have a wider existence and may be among the common ARLGMDs in Indian population. doi:10.1016/j.jns.2013.07.1537
Abstract - WCN 2013 No: 763 Topic: 7 - Neuromuscular disorders The importance of follow-up thoracic imaging in myasthenia gravis patients H. Muraia, K. Utsugisawab, S. Suzukic, T. Imaid, Y. Naganeb, M. Masudae, S. Konnof, Y. Suzukig, S. Nakaneh, J.-I. Kiraa. aDepartment of Neurology, Kyushu University, Fukuoka, Japan; bHanamaki General Hospital, Hanamaki, Japan; cKeio University School of Medicine, Tokyo, Japan; d Sapporo Medical University School of Medicine, Sapporo, Japan; eTokyo Medical University, Tokyo, Japan; fToho University Medical Center Ohhashi Hospital, Tokyo, Japan; gNational Hospital Organization Sendai Medical Center, Sendai, Japan; hNational Hospital Organization Nagasaki Kawatana Medical Center, Kawatana, Japan Background: Approximately 20–30% of myasthenia gravis (MG) patients are accompanied by thymoma. In elderly patients, extra-thymic malignancies are also a matter of concern. Although most MG patients undergo thoracic imaging at the initial diagnosis, the follow-up protocol for thoracic imaging is not clear. Objective: To elucidate the follow-up status of thoracic imaging in MG. Patients and methods: From among 676 MG patients in 11 neurological centers, we evaluated 649 patients whose disease duration was over 1 year. The latest thoracic imaging (CT or MRI) after an interval of 1 year or more from the initial scan was evaluated in each case. The rate of abnormal findings and the details of findings in cases without thymoma (group N) and with thymoma (group T) were then investigated. Results: Thoracic imaging follow-up was performed in 337 (51.9%) of MG patients studied (48.8% in group N, 74.1% in group T). The average interval was 7.3 years in group N and 7.8 years in group T. Abnormal findings were detected in 9.0% in group N and 25.2% in group T. Abnormal findings included breast cancer, swelling of lymph nodes and respiratory system infection in group N, while recurrence or enlargement of thymoma accounted for 76.9% of the abnormal findings in group T.
Conclusion: It is important to keep following up thoracic imaging in MG patients since the rate of abnormal findings is high even 7 years after onset. doi:10.1016/j.jns.2013.07.1538
Abstract - WCN 2013 No: 719 Topic: 7 - Neuromuscular disorders The initial electrodiagnostic signs that distinguish acute inflammatory demyelinating polyneuropathy from acute motor axonal neuropathy C.S.J. Lima, N. Yukib, T. Umapathic. aYong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore; bDepartment of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore; cDepartment of Neurology, National Neuroscience Institute, Singapore, Singapore Background: Guillain–Barré syndrome (GBS) is divided into 2 neurophysiological subtypes, acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The early electrodiagnostic findings of AIDP overlap with AMAN, leading to the latter's under-diagnosis. Serial nerve conduction studies (NCSs) are more sensitive, but not always feasible. Objective: To derive diagnostic criteria that distinguish AMAN from AIDP at presentation, by delineating their nascent electrodiagnostic features. Patients and methods: We used at least 2 serial NCSs to categorise GBS patients into AIDP and AMAN. The initial NCS was scrutinized for features discriminating the 2 subtypes. Results: Using serial NCSs, 30 GBS patients were divided into 11 AIDP, 14 AMAN, and 5 unclassified cases. The initial NCS, done at median 7 and range 2-14 days, showed prolonged distal motor latency in 7 AIDP and 8 AMAN patients. Six AIDP patients had slowing in non-entrapment sites and 4 had temporal dispersion (TD). Only 1 AMAN patient had these. Slowing at entrapment site was less specific, occurring in 8 AIDP and 3 AMAN patients. Three AIDP patients had conduction block (CB), all with slowing across the block. Seven nerves in 5 AMAN patients had CB, 5 without slowing. Conclusion: The most specific initial signs of AIDP were slowing in nonentrapment sites and TD. CMAP reduction in at least 2 nerves without TD or slowing at non-entrapment sites and non-slowing CB predict AMAN. Using these features, 22 out of 30 patients were assigned to the right GBS subtypes; current criteria were correct in 14 patients. doi:10.1016/j.jns.2013.07.1539
Abstract - WCN 2013 No: 761 Topic: 7 - Neuromuscular disorders LGMD2I: Immunohistochemical and immunoblot technique assisted identification of 51 cases with both duchenne and becker phenotype S. Modia, A. Nalinia, N. Gayathrib, M.S. Bharathc, B. Sunithab, K. Polavarapua. a Department of Neurology, NIMHANS, Bengaluru, India; bDepartment of Neuropathology, NIMHANS, Bengaluru, India; cDepartment of Neurochemistry, NIMHANS, Bengaluru, India Background: LGMD 2I is caused by mutations in fukutin-related protein gene (FKRP). Objective: To describe the phenotype and findings of immunohistochemistry (IHC) and immunoblot (IB) in LGMD2I. Materials and methods: Prospective study of 300 cases of ARLGMD seen at Neuromuscular Disorders clinic between February 2010–