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Development of service for patients with Motor Neuron Disease (MND) in Singapore
Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
patterns: Classic 10 (21%); bigger medially 25 (53%), bigger inferiorly 5 (11%), bigger both 3 (6%), restricted 11 (23%). Conclusions: Sensory loss in MP frequently differs from classic descriptions. The area is often larger, sometimes smaller. Explanations may include anatomic variation and the degree of damage of the LCNT. doi:10.1016/j.jns.2013.07.1610
Abstract - WCN 2013 No: 1899 Topic: 7 - Neuromuscular disorders Electromyography in a small city in Ecuador
e451
(5.7%), 77 girls, 25 boys (3.1:1). AChR seropositive MG was diagnosed in 86%, MuSK positive in 3%, seronegative in 11% of MG patients. Ocular MG: 328 (18.4%), generalised MG: 1457 (81.6%) patients. Thymoma was histologically proven in 158 (8.9%) patients. Conclusion: The prevalence rate of MG has increased over the past 35 years, because the mortality rates declined and MG patients have longer life span due to significant advances in the treatment. Since the 1980s a gradually increased incidence has been evident, mainly due to late-onset MG, giving a significant shift to older age at onset of MG. These findings are relevant for non-thymoma MG. The epidemiological data of thymoma-associated MG were constant. doi:10.1016/j.jns.2013.07.1612
F. Estevez. Universidad de Cuenca, Cuenca, Ecuador Background: EMG studies are helpful in diagnosis for many neurological diseases. There are not enough reports to take care about the necessities for a General Hospital in any region around the World. Objective: To know the absolute number of patients who can be assessed by a novel EMG laboratory; correlate the true positive relation between the suspected diagnosis and the final report; try to avoid for the future, the excess of demand. Results: During 2009 we studied a total of 209 patients, mean age 49 years (SD 17), 56.4% women and 43.5% men. Neurosurgery (34.4%), Rheumatology (22%), Traumatology (15%) and Neurology (12%) were the most frequent senders for the studies. The most common diagnosis included: neuropathic pathology (50.7%) in this item, the entrapment neuropathies were the most common diagnosis (carpal tunnel syndrome 34%); root lesions (27.7%) with compromise of the lower back in 58.6% (L4, L5 and S1 roots); and finally 17% of all were normal. 43% of cases had positive relation between the suspected diagnosis and the final report; 40% did not have a presumed diagnosis. Conclusions: It is important to know the absolute demand for an EMG laboratory, and it is also necessary to establish an educational program for the medical staff in the sense of a better understanding of this kind of tests, the presumed diagnosis as a crucial tool for the electromyographist and of course for the patient because it is not a simple test and to avoid unnecessary discomfort. doi:10.1016/j.jns.2013.07.1611
Abstract - WCN 2013 No: 1911 Topic: 7 - Neuromuscular disorders Epidemiology of myasthenia gravis in Slovak Republic I. Martinka, P. Spalek. Department of Neurology, University Hospital Bratislava, Hospital Ruzinov, Bratislava, Slovak Republic Background: 1785 patients were registered up to December 31, 2012 in the Centre for Myasthenia Gravis (MG) in Slovakia. Objective: Analysis of MG epidemiological data in Slovakia (1978– 2012). Patients and methods: The diagnosis of MG was based on clinical and electrophysiological findings, positive AChR and MuSK autoantibodies. Results: The prevalence rate on Dec 31, 2012: 241.8 per 1 million. The average annual incidence rate (2003–2012): 14.8 per 1 million. Sex: 1033 females, 752 males (1.37:1). The median age at MG onset: 54 years, females 48 years, males 62 years. Early-onset MG prior the age of 50 years: 800 patients, 605 females, 195 males (3.1:1). Lateonset MG after the age of 50 years: 985 patients, 428 females, 557 males (1:1.3). Infantile MG prior the age of 15 years: 102 children
Abstract - WCN 2013 No: 1915 Topic: 7 - Neuromuscular disorders Guillain–Barré syndrome: subtypes and predictors of outcome from India J. Kalita, G. Goyal, M. Das, U.K. Misra. Dept. of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India Background: Acute motor axonal variant of Guillain Barre syndrome has been reported to be commoner from South East Asia. The types of GBS however depend on the extent and timing of electrodiagnostic study. There is paucity of large study evaluating the subtypes of GBS and their outcome from South East Asia. Objective: We report cliniconeurophysiological subtypes of Guillain Barré syndrome (GBS) and their outcome from Northern India. Patients and methods: 328 GBS patients were categorized into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), pure sensory (PS) and Miller–Fisher syndrome (MFS) based on nerve conduction study (NCS). The various patterns of GBS correlation with triggering factors, severity of illness, clinical characteristics, and 3 months outcome. Results: The patients' mean age was 31.2 years. Clinically 204 (62.2%) patients had pure motor, 106 (32.3%) motor sensory, 16 (4.9%) MFS and 2 (0.6%) had pure sensory GBS. Based on NCS, 242 (73.8%) had AIDP, 44 (13.4%) AMAN, 15 (4.6%) AMSAN and 27 (8.2%) had equivocal GBS. AIDP patients were older, lesser disabled at admission and better outcome compared to AMAN. 11 (3.4%) patients died and 48 (14.6%) had poor outcome. The poor outcome was related to severity, dysautonomia and inexcitable motor nerves. Conclusion: AIDP is the commonest subtype of GBS in Northern India. Patients with severe disability, dysautonomia and inexcitable nerves had poor outcome. doi:10.1016/j.jns.2013.07.1613
Abstract - WCN 2013 No: 1409 Topic: 7 - Neuromuscular disorders Development of service for patients with Motor Neuron Disease (MND) in Singapore K. Anga, C.Y. Cheonga, T. Umapathia, I.M.H. Woob. aNational Neuroscience Institute (Tan Tock Seng Hospital), Singapore, Singapore; bTan Tock Seng Hospital, Singapore, Singapore Background: Observations made in clinical practice suggest that people living with MND need a comprehensive set of services that can meet their bio-psychosocial-spiritual needs. However, there is no known
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Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
literature that can inform the development of services appropriate for patients with MND living in Singapore, a multicultural and familycentered society. Objective/method: Drawing from findings of our study that explored the needs of patients with MND and observations made in clinical practice, this presentation will attempt to describe services that can meet the needs of patients with MND in Singapore. Results: Research findings and clinical observations point to the need to develop proactive services that are sensitive to the needs of patients and their caregivers. It was found that “staging” the disease will better meet the needs of the patients as they progress along the disease trajectory. At the earlier stage of the disease, timely supportive counseling for patients and their caregivers after disclosure of diagnosis is needed to manage impact of the disclosure. Counseling is also required to open up communication between patients and their caregivers. Paced education for patients and their caregivers through verbal communication and audiovisual aids, complemented by support groups, will benefit both patients and their caregivers. At a later stage of the disease, there is a need to help 1) the patients work towards a respectful death and 2) their caregivers achieve a peaceful farewell. Advance care planning can be a platform for patients and their caregivers to achieve the closure needed by both parties. doi:10.1016/j.jns.2013.07.1614
Abstract - WCN 2013 No: 1976 Topic: 7 - Neuromuscular disorders Brachial plexopathy due to localized axillary recurrence of breast cancer J. Cho. Neurology, National Health Insurance Service Ilsan Hospital, Goyangsi, Gyeonggido, Republic of Korea The brachial plexopathy in breast cancer patients has been reported to be due to loco-regional metastasis or radiation plexopathy. Localized axillary recurrence (LAR) is rare. The time to LAR after primary breast cancer is various from a few months to many years. We report a patient of brachial plexopathy due to LAR that developed twenty four years after radical mastectomy and radiation therapy for breast cancer. A 58-year-old woman was admitted with left hand weakness, paresthesia and pain. The examination revealed edema, weakness and sensory change of left arm and hand. But there was no palpable mass in subclavian fossa or axilla. The NCS and EMG revealed left brachial plexus lesion (multiple levels of roots and trunks). Chest CT and breast sonography showed huge mass in the left axilla with invasion of left subclavian vessels. PET CT showed huge malignant mass along left axillary fossa without distant metastasis. We performed ultrasonography-guided gun-biopsy of mass and the pathologic diagnosis was invasive carcinoma with positive estrogen and progesterone receptors. LAR of breast cancer is rare but can occur anytime after initial treatment. LAR is associated with poor prognosis because it is an independent risk factor for distant metastasis and death. We need to evaluate lymph node by sonography in the patient of breast cancer because the physical examination of the axilla can reveal falsenegative finding in spite of huge mass. doi:10.1016/j.jns.2013.07.1615
Abstract - WCN 2013 No: 1938 Topic: 7 - Neuromuscular disorders Idiopathic inflammatory myopathies—Potential role of the immunoexpression of interleukin-35 in muscle biopsy J. Zamecnika,b, J. Haceka,b, R.A. Dahmena,b, H. Mannc, L. Senoltc, J. Vencovskyc. aDept. of Pathology and Molecular Medicine, Charles University Prague, Prague, Czech Republic; bUniversity Hospital Motol, Prague, Czech Republic; cInstitute of Rheumatology, Dept. of Experimental and Clinical Rheumatology, Charles University Prague, 1st Faculty of Medicine, Prague, Czech Republic Background: Interleukin-35 (IL-35) is a newly described cytokine that belongs to the IL-12 family. Although the experiments in mouse models showed immunosuppressive functions for IL-35, recent papers reported different expression pattern and rather pro-inflammatory properties of IL-35 in humans. Currently available biologic treatment for systemic rheumatic autoimmune diseases including the idiopathic inflammatory myopathies (i.e. polymyositis and dermatomyositis) targets specific cellular and molecular mechanisms; therefore, studies of various molecular pathways of immunopathogenesis in idiopathic inflammatory myopathies are of interest for future therapies. Objective: To analyze immunoexpression of interleukin-35 in muscle biopsies of inflammatory and non-inflammatory myopathies to assess its pathogenetic and diagnostic value. Materials and methods: Immunoexpression and its localization of IL35 was studied by primary rabbit anti-human EBI3 polyclonal antibody and mouse anti-human IL-12a(p35) monoclonal antibody in a series of 19 muscle biopsy samples of idiopathic inflammatory myopathies (9 dermatomyositis, 10 polymyositis). Results were compared to those obtained in 10 cases of non-inflammatory myopathies and in 10 control muscles biopsies. Results: We showed localization of the p35 and EBI3 subunits of IL35 in B cells, T cells and macrophages of the inflammatory infiltrate in idiopathic inflammatory myopathies. No immunoreactivity was observed in healthy controls and in non-inflammatory myopathies. Conclusion: Our data suggest pro-inflammatory properties of IL-35 in humans and a potential role in the pathogenesis of idiopathic inflammatory myopathies. The immunohistochemical expression of IL-35 might be also of diagnostic help in muscle biopsy. Supported by: IGA NT/12440-4. doi:10.1016/j.jns.2013.07.1616
Abstract - WCN 2013 No: 1968 Topic: 7 - Neuromuscular disorders Spinal pain syndromes: Psycho-emotional facets Y. Goncharovaa, N. Bozhenkob. aNeurology Department, State Institution, Institute of Urgent and Recovery Surgery n.a. V.K. Gusak, National Academy of Medical Sciences of Ukraine, Donetsk, Ukraine; bNeurology Department, Lviv National Medical University named after Danylo Galytsky, Lviv, Ukraine Background: Low back pain syndromes, especially the chronic ones, are the important link in neurology. Pain as personal experience develops not only due to physical pathology, but as consequence of person's attitude towards disease, previous experience, described in terms of sensory and emotional disorders. Material and methods: We studied 23 patients with lumbar-sacral radiculopathies. Features of psycho-emotional state were studied using Hamilton scale and Aysenk questionnaire. Pain syndrome was assesses using VAS and DN4 scale. Results: Disorders like depressive syndrome were observed among 58% patients. Pain syndrome according to VAS was 6.9 ± 0.3 in persons with
patterns: Classic 10 (21%); bigger medially 25 (53%), bigger inferiorly 5 (11%), bigger both 3 (6%), restricted 11 (23%). Conclusions: Sensory loss in MP frequently differs from classic descriptions. The area is often larger, sometimes smaller. Explanations may include anatomic variation and the degree of damage of the LCNT. doi:10.1016/j.jns.2013.07.1610
Abstract - WCN 2013 No: 1899 Topic: 7 - Neuromuscular disorders Electromyography in a small city in Ecuador
e451
(5.7%), 77 girls, 25 boys (3.1:1). AChR seropositive MG was diagnosed in 86%, MuSK positive in 3%, seronegative in 11% of MG patients. Ocular MG: 328 (18.4%), generalised MG: 1457 (81.6%) patients. Thymoma was histologically proven in 158 (8.9%) patients. Conclusion: The prevalence rate of MG has increased over the past 35 years, because the mortality rates declined and MG patients have longer life span due to significant advances in the treatment. Since the 1980s a gradually increased incidence has been evident, mainly due to late-onset MG, giving a significant shift to older age at onset of MG. These findings are relevant for non-thymoma MG. The epidemiological data of thymoma-associated MG were constant. doi:10.1016/j.jns.2013.07.1612
F. Estevez. Universidad de Cuenca, Cuenca, Ecuador Background: EMG studies are helpful in diagnosis for many neurological diseases. There are not enough reports to take care about the necessities for a General Hospital in any region around the World. Objective: To know the absolute number of patients who can be assessed by a novel EMG laboratory; correlate the true positive relation between the suspected diagnosis and the final report; try to avoid for the future, the excess of demand. Results: During 2009 we studied a total of 209 patients, mean age 49 years (SD 17), 56.4% women and 43.5% men. Neurosurgery (34.4%), Rheumatology (22%), Traumatology (15%) and Neurology (12%) were the most frequent senders for the studies. The most common diagnosis included: neuropathic pathology (50.7%) in this item, the entrapment neuropathies were the most common diagnosis (carpal tunnel syndrome 34%); root lesions (27.7%) with compromise of the lower back in 58.6% (L4, L5 and S1 roots); and finally 17% of all were normal. 43% of cases had positive relation between the suspected diagnosis and the final report; 40% did not have a presumed diagnosis. Conclusions: It is important to know the absolute demand for an EMG laboratory, and it is also necessary to establish an educational program for the medical staff in the sense of a better understanding of this kind of tests, the presumed diagnosis as a crucial tool for the electromyographist and of course for the patient because it is not a simple test and to avoid unnecessary discomfort. doi:10.1016/j.jns.2013.07.1611
Abstract - WCN 2013 No: 1911 Topic: 7 - Neuromuscular disorders Epidemiology of myasthenia gravis in Slovak Republic I. Martinka, P. Spalek. Department of Neurology, University Hospital Bratislava, Hospital Ruzinov, Bratislava, Slovak Republic Background: 1785 patients were registered up to December 31, 2012 in the Centre for Myasthenia Gravis (MG) in Slovakia. Objective: Analysis of MG epidemiological data in Slovakia (1978– 2012). Patients and methods: The diagnosis of MG was based on clinical and electrophysiological findings, positive AChR and MuSK autoantibodies. Results: The prevalence rate on Dec 31, 2012: 241.8 per 1 million. The average annual incidence rate (2003–2012): 14.8 per 1 million. Sex: 1033 females, 752 males (1.37:1). The median age at MG onset: 54 years, females 48 years, males 62 years. Early-onset MG prior the age of 50 years: 800 patients, 605 females, 195 males (3.1:1). Lateonset MG after the age of 50 years: 985 patients, 428 females, 557 males (1:1.3). Infantile MG prior the age of 15 years: 102 children
Abstract - WCN 2013 No: 1915 Topic: 7 - Neuromuscular disorders Guillain–Barré syndrome: subtypes and predictors of outcome from India J. Kalita, G. Goyal, M. Das, U.K. Misra. Dept. of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India Background: Acute motor axonal variant of Guillain Barre syndrome has been reported to be commoner from South East Asia. The types of GBS however depend on the extent and timing of electrodiagnostic study. There is paucity of large study evaluating the subtypes of GBS and their outcome from South East Asia. Objective: We report cliniconeurophysiological subtypes of Guillain Barré syndrome (GBS) and their outcome from Northern India. Patients and methods: 328 GBS patients were categorized into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), pure sensory (PS) and Miller–Fisher syndrome (MFS) based on nerve conduction study (NCS). The various patterns of GBS correlation with triggering factors, severity of illness, clinical characteristics, and 3 months outcome. Results: The patients' mean age was 31.2 years. Clinically 204 (62.2%) patients had pure motor, 106 (32.3%) motor sensory, 16 (4.9%) MFS and 2 (0.6%) had pure sensory GBS. Based on NCS, 242 (73.8%) had AIDP, 44 (13.4%) AMAN, 15 (4.6%) AMSAN and 27 (8.2%) had equivocal GBS. AIDP patients were older, lesser disabled at admission and better outcome compared to AMAN. 11 (3.4%) patients died and 48 (14.6%) had poor outcome. The poor outcome was related to severity, dysautonomia and inexcitable motor nerves. Conclusion: AIDP is the commonest subtype of GBS in Northern India. Patients with severe disability, dysautonomia and inexcitable nerves had poor outcome. doi:10.1016/j.jns.2013.07.1613
Abstract - WCN 2013 No: 1409 Topic: 7 - Neuromuscular disorders Development of service for patients with Motor Neuron Disease (MND) in Singapore K. Anga, C.Y. Cheonga, T. Umapathia, I.M.H. Woob. aNational Neuroscience Institute (Tan Tock Seng Hospital), Singapore, Singapore; bTan Tock Seng Hospital, Singapore, Singapore Background: Observations made in clinical practice suggest that people living with MND need a comprehensive set of services that can meet their bio-psychosocial-spiritual needs. However, there is no known
e452
Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
literature that can inform the development of services appropriate for patients with MND living in Singapore, a multicultural and familycentered society. Objective/method: Drawing from findings of our study that explored the needs of patients with MND and observations made in clinical practice, this presentation will attempt to describe services that can meet the needs of patients with MND in Singapore. Results: Research findings and clinical observations point to the need to develop proactive services that are sensitive to the needs of patients and their caregivers. It was found that “staging” the disease will better meet the needs of the patients as they progress along the disease trajectory. At the earlier stage of the disease, timely supportive counseling for patients and their caregivers after disclosure of diagnosis is needed to manage impact of the disclosure. Counseling is also required to open up communication between patients and their caregivers. Paced education for patients and their caregivers through verbal communication and audiovisual aids, complemented by support groups, will benefit both patients and their caregivers. At a later stage of the disease, there is a need to help 1) the patients work towards a respectful death and 2) their caregivers achieve a peaceful farewell. Advance care planning can be a platform for patients and their caregivers to achieve the closure needed by both parties. doi:10.1016/j.jns.2013.07.1614
Abstract - WCN 2013 No: 1976 Topic: 7 - Neuromuscular disorders Brachial plexopathy due to localized axillary recurrence of breast cancer J. Cho. Neurology, National Health Insurance Service Ilsan Hospital, Goyangsi, Gyeonggido, Republic of Korea The brachial plexopathy in breast cancer patients has been reported to be due to loco-regional metastasis or radiation plexopathy. Localized axillary recurrence (LAR) is rare. The time to LAR after primary breast cancer is various from a few months to many years. We report a patient of brachial plexopathy due to LAR that developed twenty four years after radical mastectomy and radiation therapy for breast cancer. A 58-year-old woman was admitted with left hand weakness, paresthesia and pain. The examination revealed edema, weakness and sensory change of left arm and hand. But there was no palpable mass in subclavian fossa or axilla. The NCS and EMG revealed left brachial plexus lesion (multiple levels of roots and trunks). Chest CT and breast sonography showed huge mass in the left axilla with invasion of left subclavian vessels. PET CT showed huge malignant mass along left axillary fossa without distant metastasis. We performed ultrasonography-guided gun-biopsy of mass and the pathologic diagnosis was invasive carcinoma with positive estrogen and progesterone receptors. LAR of breast cancer is rare but can occur anytime after initial treatment. LAR is associated with poor prognosis because it is an independent risk factor for distant metastasis and death. We need to evaluate lymph node by sonography in the patient of breast cancer because the physical examination of the axilla can reveal falsenegative finding in spite of huge mass. doi:10.1016/j.jns.2013.07.1615
Abstract - WCN 2013 No: 1938 Topic: 7 - Neuromuscular disorders Idiopathic inflammatory myopathies—Potential role of the immunoexpression of interleukin-35 in muscle biopsy J. Zamecnika,b, J. Haceka,b, R.A. Dahmena,b, H. Mannc, L. Senoltc, J. Vencovskyc. aDept. of Pathology and Molecular Medicine, Charles University Prague, Prague, Czech Republic; bUniversity Hospital Motol, Prague, Czech Republic; cInstitute of Rheumatology, Dept. of Experimental and Clinical Rheumatology, Charles University Prague, 1st Faculty of Medicine, Prague, Czech Republic Background: Interleukin-35 (IL-35) is a newly described cytokine that belongs to the IL-12 family. Although the experiments in mouse models showed immunosuppressive functions for IL-35, recent papers reported different expression pattern and rather pro-inflammatory properties of IL-35 in humans. Currently available biologic treatment for systemic rheumatic autoimmune diseases including the idiopathic inflammatory myopathies (i.e. polymyositis and dermatomyositis) targets specific cellular and molecular mechanisms; therefore, studies of various molecular pathways of immunopathogenesis in idiopathic inflammatory myopathies are of interest for future therapies. Objective: To analyze immunoexpression of interleukin-35 in muscle biopsies of inflammatory and non-inflammatory myopathies to assess its pathogenetic and diagnostic value. Materials and methods: Immunoexpression and its localization of IL35 was studied by primary rabbit anti-human EBI3 polyclonal antibody and mouse anti-human IL-12a(p35) monoclonal antibody in a series of 19 muscle biopsy samples of idiopathic inflammatory myopathies (9 dermatomyositis, 10 polymyositis). Results were compared to those obtained in 10 cases of non-inflammatory myopathies and in 10 control muscles biopsies. Results: We showed localization of the p35 and EBI3 subunits of IL35 in B cells, T cells and macrophages of the inflammatory infiltrate in idiopathic inflammatory myopathies. No immunoreactivity was observed in healthy controls and in non-inflammatory myopathies. Conclusion: Our data suggest pro-inflammatory properties of IL-35 in humans and a potential role in the pathogenesis of idiopathic inflammatory myopathies. The immunohistochemical expression of IL-35 might be also of diagnostic help in muscle biopsy. Supported by: IGA NT/12440-4. doi:10.1016/j.jns.2013.07.1616
Abstract - WCN 2013 No: 1968 Topic: 7 - Neuromuscular disorders Spinal pain syndromes: Psycho-emotional facets Y. Goncharovaa, N. Bozhenkob. aNeurology Department, State Institution, Institute of Urgent and Recovery Surgery n.a. V.K. Gusak, National Academy of Medical Sciences of Ukraine, Donetsk, Ukraine; bNeurology Department, Lviv National Medical University named after Danylo Galytsky, Lviv, Ukraine Background: Low back pain syndromes, especially the chronic ones, are the important link in neurology. Pain as personal experience develops not only due to physical pathology, but as consequence of person's attitude towards disease, previous experience, described in terms of sensory and emotional disorders. Material and methods: We studied 23 patients with lumbar-sacral radiculopathies. Features of psycho-emotional state were studied using Hamilton scale and Aysenk questionnaire. Pain syndrome was assesses using VAS and DN4 scale. Results: Disorders like depressive syndrome were observed among 58% patients. Pain syndrome according to VAS was 6.9 ± 0.3 in persons with