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Paraneoplastic sensorimotor polyneuropathy and inflammatory myopathy associated with Merkel cell carcinoma
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Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
immunoglobulin therapy without enough effects. After transfer to our university hospital, we paid attention to both cases of thrombocytosis. Both had hepatosplenomegaly, lambda type M-proteinemia, and significantly elevated serum VEGF. doi:10.1016/j.jns.2013.07.1638
Abstract — WCN 2013 No: 2291 Topic: 7 — Neuromuscular disorders Etiological consideration of 26 patients with dropped head syndrome K. Uchino, G. Ogawa, T. Chiba, K. Hokkoku, Y. Hatanaka, M. Sonoo. Department of Neurology, Teikyo University School of Medicine, Itabashi-ku, Japan Background: The dropped head syndrome (DHS) is caused by a wide variety of neurological disorders ranging from CNS degenerative diseases to peripheral neuromuscular problems. There have been few studies that identified causes of DHS for a large number of patients. Objective: To elucidate the frequencies of individual causes in a case series of DHS patients. Materials and methods: Patients presenting with DHS were extracted from the database of our institution for 13 years since 1999. Their medical and EMG records were retrospectively reviewed. Results: Twenty-six patients were extracted (4 men and 22 women, age 71.1 ± 8.6 years). The final diagnoses were 13 myasthenia gravis (MG), 4 myositis, 1 myopathy of undetermined etiology, 3 amyotrophic lateral sclerosis, 3 Parkinson's disease,1 dementia with Lewy body, and 1 with unknown etiology. One MG and 1 myositis patients were associated with Parkinson's disease, but they were diagnosed as myopathies by EMG. All but one MG patients were negative for AChR antibody, and MuSK antibody was also negative for all of 10 patients examined. They were diagnosed as MG by the abnormal single-fiber EMG (SFEMG) of the splenius muscle, together with lack of profuse fibrillation potentials and positive sharp waves in the concentric needle EMG (CNEMG) of the same muscle. Immunotherapies were attempted in 6 MG patients (5 IVIg and 1 steroid) and were effective at least transiently. Conclusion: MG was the most frequent cause of DHS, followed by myositis. CNEMG and SFEMG of the splenius muscle were the key to their diagnosis. doi:10.1016/j.jns.2013.07.1639
Abstract — WCN 2013 No: 2252 Topic: 7 — Neuromuscular disorders Paraneoplastic sensorimotor polyneuropathy and inflammatory myopathy associated with Merkel cell carcinoma E. Papageorgiou, K. Psychogios, E. Karamouzos, I. Xydakis, I. Markakis, G. Gekas. Neurology, 'St. Panteleimon' General State Hospital, Nikaia, Greece Background: Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignancy. In the literature MCC has been very rarely associated with paraneoplastic neurological complications. Objective: To report a case of paraneoplastic polyneuropathy and myopathy in a patient with MCC of unknown primary origin (MCCUP). Patient and methods: A 65-year-old man was admitted to our department with progressive limb weakness, numbness and myalgias.
Physical examination revealed a left axillary nodular mass. On neurological examination he exhibited asymmetric, predominantly proximal tetraparesis with absent tendon reflexes. Brain MRI was unremarkable and spinal MRI demonstrated multiple levels of mild spondylosis. Cerebrospinal fluid examination revealed a normal cell count with increased protein content (77.2 mg/dl) and positive oligoclonal bands. Nerve conduction studies were consistent with a mixed sensorimotor polyneuropathy. Treatment with intravenous immunoglobulin was initiated followed by oral steroids, with subsequent gradual improvement of muscle strength. Biopsy of the axillary mass revealed an undifferentiated MCC. Screening for an underlying primary site and testing for anti-onconeural and anti-ganglioside antibodies were negative. Deltoid muscle biopsy revealed inflammatory myopathy and sural nerve biopsy demonstrated mixed axonal and demyelinating changes. The patient underwent complete regional lymph node resection followed by adjuvant chemotherapy, with significant clinical improvement, remaining relapse-free during a 2 year follow-up period. Results and conclusion: Both the clinical presentation and the positive response of our patient to cancer treatment support the paraneoplastic origin of his neurological condition. MCC should be considered as a diagnostic possibility in patients presenting with peripheral neurologic syndromes with atypical features. doi:10.1016/j.jns.2013.07.1640
Abstract — WCN 2013 No: 2330 Topic: 7 — Neuromuscular disorders 26 years' experience in myasthenia gravis in Iran A. Soltanzadeh, S. Jamal Omidi, S. Nafissi, F. Fatehi. Neurology, Tehran University of Medical Sciences, Tehran, Iran Background: Myasthenia gravis is an autoimmune disorder affecting the neuromuscular junction. Signs and symptoms present a wide spectrum ranging from only ocular symptoms to a life-threatening respiratory failure. Objective: This study was carried out to show the demographic characteristics of myasthenia gravis in Iran. Unusual presenting features of our patients were also taken into account. Methods: The files of all myasthenia patients arriving to clinic during 26 years were studied on a retrospective basis; their demographic and clinical data were registered and duly analysed. Then, cases of congenital myasthenia were excluded from the study. Results: Out of the 316 myasthenia gravis patients included in the study, 58.55% were female and 41.45% male. 272 patients suffered from the generalized form of myasthenia while the remaining 51 patients had only ocular presentations. 6 patients had atypical presenting features as the first manifestation (jaw-hanging, cervical weakness, dyspnea). Among those with the generalized type, 11% had suffered crisis and 13.6% experienced at-least 1 episode of disease exacerbation. Interestingly the youngest and oldest ages of onset were 8 and 70 years, respectively. Out of the 128 patients who underwent thymectomy, 21.09% and 48.43% had pathology reports of thymoma and hyperplasia, respectively. Unfortunately 6 ladies and 12 men were taken by deaths, two of which died with pneumosepsis. Conclusion: Epidemiologic findings of Myasthenia Gravis in Iran are more or less similar to those of other countries, but it is very important to bear in mind the atypical presenting features of this disease that can otherwise be misleading. doi:10.1016/j.jns.2013.07.1641
Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
immunoglobulin therapy without enough effects. After transfer to our university hospital, we paid attention to both cases of thrombocytosis. Both had hepatosplenomegaly, lambda type M-proteinemia, and significantly elevated serum VEGF. doi:10.1016/j.jns.2013.07.1638
Abstract — WCN 2013 No: 2291 Topic: 7 — Neuromuscular disorders Etiological consideration of 26 patients with dropped head syndrome K. Uchino, G. Ogawa, T. Chiba, K. Hokkoku, Y. Hatanaka, M. Sonoo. Department of Neurology, Teikyo University School of Medicine, Itabashi-ku, Japan Background: The dropped head syndrome (DHS) is caused by a wide variety of neurological disorders ranging from CNS degenerative diseases to peripheral neuromuscular problems. There have been few studies that identified causes of DHS for a large number of patients. Objective: To elucidate the frequencies of individual causes in a case series of DHS patients. Materials and methods: Patients presenting with DHS were extracted from the database of our institution for 13 years since 1999. Their medical and EMG records were retrospectively reviewed. Results: Twenty-six patients were extracted (4 men and 22 women, age 71.1 ± 8.6 years). The final diagnoses were 13 myasthenia gravis (MG), 4 myositis, 1 myopathy of undetermined etiology, 3 amyotrophic lateral sclerosis, 3 Parkinson's disease,1 dementia with Lewy body, and 1 with unknown etiology. One MG and 1 myositis patients were associated with Parkinson's disease, but they were diagnosed as myopathies by EMG. All but one MG patients were negative for AChR antibody, and MuSK antibody was also negative for all of 10 patients examined. They were diagnosed as MG by the abnormal single-fiber EMG (SFEMG) of the splenius muscle, together with lack of profuse fibrillation potentials and positive sharp waves in the concentric needle EMG (CNEMG) of the same muscle. Immunotherapies were attempted in 6 MG patients (5 IVIg and 1 steroid) and were effective at least transiently. Conclusion: MG was the most frequent cause of DHS, followed by myositis. CNEMG and SFEMG of the splenius muscle were the key to their diagnosis. doi:10.1016/j.jns.2013.07.1639
Abstract — WCN 2013 No: 2252 Topic: 7 — Neuromuscular disorders Paraneoplastic sensorimotor polyneuropathy and inflammatory myopathy associated with Merkel cell carcinoma E. Papageorgiou, K. Psychogios, E. Karamouzos, I. Xydakis, I. Markakis, G. Gekas. Neurology, 'St. Panteleimon' General State Hospital, Nikaia, Greece Background: Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignancy. In the literature MCC has been very rarely associated with paraneoplastic neurological complications. Objective: To report a case of paraneoplastic polyneuropathy and myopathy in a patient with MCC of unknown primary origin (MCCUP). Patient and methods: A 65-year-old man was admitted to our department with progressive limb weakness, numbness and myalgias.
Physical examination revealed a left axillary nodular mass. On neurological examination he exhibited asymmetric, predominantly proximal tetraparesis with absent tendon reflexes. Brain MRI was unremarkable and spinal MRI demonstrated multiple levels of mild spondylosis. Cerebrospinal fluid examination revealed a normal cell count with increased protein content (77.2 mg/dl) and positive oligoclonal bands. Nerve conduction studies were consistent with a mixed sensorimotor polyneuropathy. Treatment with intravenous immunoglobulin was initiated followed by oral steroids, with subsequent gradual improvement of muscle strength. Biopsy of the axillary mass revealed an undifferentiated MCC. Screening for an underlying primary site and testing for anti-onconeural and anti-ganglioside antibodies were negative. Deltoid muscle biopsy revealed inflammatory myopathy and sural nerve biopsy demonstrated mixed axonal and demyelinating changes. The patient underwent complete regional lymph node resection followed by adjuvant chemotherapy, with significant clinical improvement, remaining relapse-free during a 2 year follow-up period. Results and conclusion: Both the clinical presentation and the positive response of our patient to cancer treatment support the paraneoplastic origin of his neurological condition. MCC should be considered as a diagnostic possibility in patients presenting with peripheral neurologic syndromes with atypical features. doi:10.1016/j.jns.2013.07.1640
Abstract — WCN 2013 No: 2330 Topic: 7 — Neuromuscular disorders 26 years' experience in myasthenia gravis in Iran A. Soltanzadeh, S. Jamal Omidi, S. Nafissi, F. Fatehi. Neurology, Tehran University of Medical Sciences, Tehran, Iran Background: Myasthenia gravis is an autoimmune disorder affecting the neuromuscular junction. Signs and symptoms present a wide spectrum ranging from only ocular symptoms to a life-threatening respiratory failure. Objective: This study was carried out to show the demographic characteristics of myasthenia gravis in Iran. Unusual presenting features of our patients were also taken into account. Methods: The files of all myasthenia patients arriving to clinic during 26 years were studied on a retrospective basis; their demographic and clinical data were registered and duly analysed. Then, cases of congenital myasthenia were excluded from the study. Results: Out of the 316 myasthenia gravis patients included in the study, 58.55% were female and 41.45% male. 272 patients suffered from the generalized form of myasthenia while the remaining 51 patients had only ocular presentations. 6 patients had atypical presenting features as the first manifestation (jaw-hanging, cervical weakness, dyspnea). Among those with the generalized type, 11% had suffered crisis and 13.6% experienced at-least 1 episode of disease exacerbation. Interestingly the youngest and oldest ages of onset were 8 and 70 years, respectively. Out of the 128 patients who underwent thymectomy, 21.09% and 48.43% had pathology reports of thymoma and hyperplasia, respectively. Unfortunately 6 ladies and 12 men were taken by deaths, two of which died with pneumosepsis. Conclusion: Epidemiologic findings of Myasthenia Gravis in Iran are more or less similar to those of other countries, but it is very important to bear in mind the atypical presenting features of this disease that can otherwise be misleading. doi:10.1016/j.jns.2013.07.1641