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Sporadic Amyotrophic Lateral Sclerosis (SALS) and environment
Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
Abstract — WCN 2013 No: 2311 Topic: 7 — Neuromuscular disorders Sporadic Amyotrophic Lateral Sclerosis (SALS) and environment
e461
Conclusions: Pesticides and environmental factors and gene interactions seem to act together to increase PD risk. doi:10.1016/j.jns.2013.07.1643
H.A. Idrisoglu. Neurology, Istanbul University Faculty of Medicine, Istanbul, Turkey Background: The cause of Sporadic Amyotrophic Lateral Sclerosis (ALS) is not known. Studies associate toxic, dietary, infectious, neoplastic, and physical factors as underlying, predisposing or pathogenic influences. Methods: 131 SALS patients are followed prospectively all of the patients investigated SOD1 mutation and blood and urine lead level. Also environmental exposure was assessed, in sporadic ALS patients. Results: All of the patients are diagnosed definite ALS according to El-Escorial criteria. 81 of 131 patients who are diagnosed with definite ALS according to ElEscorial criteria are men. The others are women. Men–women ratio is 1.5/1. The age of onset of 131 definite ALS cases is between 15 and 73. The average age is 48.7. The beginning of age in men is 40.1. It is 49.7 in women. The average examination age in men is 49.1. It is 51.1 in women. 131 patients are separated to the specific types based on examination findings during examination. 16 of them are progressive bulbar paralysis (PBP), 72 of them are in PBP + spinal form, and 43 of them are in spinal form. When we search blood and urine lead levels in all 131 patients and when we take family individuals. Of this patients as central group and compare them Blood and urine lead levels of this group are statistically and significantly found high. Blood lead level is p b 0.001, urine lead level is p b 0.01. We don't encounter abnormal (toxic) levels (0.80 mg/dl) in one of the cases. All patients are followed prospectively. Conclusions: We found blood and lead levels of SALS patients statistically and significantly high. doi:10.1016/j.jns.2013.07.1642
Abstract— WCN 2013 No: 2373 Topic: 7 — Neuromuscular disorders Parkinson, pesticides and environment H.A. Idrisoğlu. Neurology, Istanbul University Faculty of Medicine, Istanbul, Turkey Background: Parkinson's disease (PD) is the most common neurodegenerative movement disorder that is a consequence of premature death of dopamine-containing neurons in the substantia nigra. A number of observations have led to the hypothesis that environmental factors, including pesticides, play a significant role in the development of PD. Method: 350 Parkinson patients applied to our clinic between the years 2008 and 2012.We achieved prospective follow up of these patients. In the result of this follow-up we determined that 35 patients were exposed to pesticide intoxication. Result: Age interval of 35 patients was between 23 and 90. Mean age was 61.7. 33 of them were male and the other patients were female. We determined that 10% of 350 patients were exposed to toxic substances. The first sign of 8 patients was bradykinesias and the first sign of 27 patients was tremor. We determined pesticide intoxication in 19 patients, CO2 intoxication in 2 patients, heavy metal exposure in 10 patients, CO2 intoxication in 2 patients and electrical charging in 2 patients. Finally, we thought 10% toxic etiology risk factor. The other characteristics that we were interested in were male patients. The patients were male and PH signs were in association with tremor.
Abstract - WCN 2013 No: 2369 Topic: 7 - Neuromuscular disorders Initial dip and bilobed N21 peak in the tibial nerve SEPs suggest predominant demyelination in the nerve root T. Chibaa, M. Sonooa, C. Oishib, K. Uchinoa, H. Okumaa, H. Kuronoa, K. Hokkokua, Y. Hatanakaa. aDepartment of Neurology, Teikyo University School of Medicine, Itabashi-ku, Japan; bDepartment of Neurology, Kyorin University School of Medicine, Mitaka, Japan Background: Sensory GBS or CIDP may be difficult to diagnose, especially when routine nerve conduction studies show unremarkable findings. Tibial nerve SEPs are useful because they can evaluate the proximal portions of the peripheral nerve. We noticed a previouslyundescribed characteristic pattern in tibial nerve SEPs of such patients. Objective: To document the significance of the “initial dip and bilobed N21 peak” pattern in the tibial nerve SEPs. Material and methods: Subjects consisted of 4 patients presenting with this pattern. Their clinical and electrophysiological records were retrospectively reviewed. Results: All patients presented with pure sensory or sensory-dominant symptoms. Routine nerve conduction studies did not show any sign of demyelination for all patients, except for the slightly reduced amplitude of the sensory nerve action potentials. The final diagnoses were sensory GBS in 2 patients, sensory CIDP in 1 patient, and sensory-dominant MADSAM in 1 patient. Tibial nerve SEPs revealed slightly delayed N8 and P15 components. The L1S-ICc (ipsilateral iliac crest) lead registered a positive dip 2 to 3 ms later than the P15 latency for all patients, and the N21 peak was either lost (1 patient) or showed a characteristic bilobed pattern (3 patients) with the later peak at a markedly delayed latency. Conclusion: The positive peak must correspond to the “killed-end potential” suggesting the near interruption of the sensory conduction. The mechanism of bilobed N21 potential is unknown, but the recognition of this characteristic pattern will help to identify demyelination of the sensory nerve predominant at the root level. doi:10.1016/j.jns.2013.07.1644
Abstract — WCN 2013 No: 2433 Topic: 7 — Neuromuscular disorders Ultrasound diagnostics and amyotrophic lateral sclerosis Y. Rushkevich, G. Zabrodets, S. Likhachev. Republican Research and Clinical Center of Neurology and Neurosurgery, Minsk, Belarus Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with progressive destruction of the central (upper) and peripheral (lower) motor neurons. One of the specific symptoms of ALS is the appearance of fasciculations, which may precede the development of muscle weakness and atrophy. Objective: To explore the possibilities of ultrasound to detect muscle fasciculations in the differential diagnosis of suspected ALS. Materials and methods: 150 patients were included (ALS, spinal amyotrophy, cervical myelopathy — 126 (84%), 11 (7%), 13 (9%) patients, respectively). Male/female — 93/37, age (median (25—75%)) — 56 (46, 62). The diagnosis of ALS was based on an analysis of clinical data electroneuromyographic El Escorial criteria (Brooks, 1998). All patients underwent electromyography (VikingSelect; Nicolet; USA). Ultrasound
Abstract — WCN 2013 No: 2311 Topic: 7 — Neuromuscular disorders Sporadic Amyotrophic Lateral Sclerosis (SALS) and environment
e461
Conclusions: Pesticides and environmental factors and gene interactions seem to act together to increase PD risk. doi:10.1016/j.jns.2013.07.1643
H.A. Idrisoglu. Neurology, Istanbul University Faculty of Medicine, Istanbul, Turkey Background: The cause of Sporadic Amyotrophic Lateral Sclerosis (ALS) is not known. Studies associate toxic, dietary, infectious, neoplastic, and physical factors as underlying, predisposing or pathogenic influences. Methods: 131 SALS patients are followed prospectively all of the patients investigated SOD1 mutation and blood and urine lead level. Also environmental exposure was assessed, in sporadic ALS patients. Results: All of the patients are diagnosed definite ALS according to El-Escorial criteria. 81 of 131 patients who are diagnosed with definite ALS according to ElEscorial criteria are men. The others are women. Men–women ratio is 1.5/1. The age of onset of 131 definite ALS cases is between 15 and 73. The average age is 48.7. The beginning of age in men is 40.1. It is 49.7 in women. The average examination age in men is 49.1. It is 51.1 in women. 131 patients are separated to the specific types based on examination findings during examination. 16 of them are progressive bulbar paralysis (PBP), 72 of them are in PBP + spinal form, and 43 of them are in spinal form. When we search blood and urine lead levels in all 131 patients and when we take family individuals. Of this patients as central group and compare them Blood and urine lead levels of this group are statistically and significantly found high. Blood lead level is p b 0.001, urine lead level is p b 0.01. We don't encounter abnormal (toxic) levels (0.80 mg/dl) in one of the cases. All patients are followed prospectively. Conclusions: We found blood and lead levels of SALS patients statistically and significantly high. doi:10.1016/j.jns.2013.07.1642
Abstract— WCN 2013 No: 2373 Topic: 7 — Neuromuscular disorders Parkinson, pesticides and environment H.A. Idrisoğlu. Neurology, Istanbul University Faculty of Medicine, Istanbul, Turkey Background: Parkinson's disease (PD) is the most common neurodegenerative movement disorder that is a consequence of premature death of dopamine-containing neurons in the substantia nigra. A number of observations have led to the hypothesis that environmental factors, including pesticides, play a significant role in the development of PD. Method: 350 Parkinson patients applied to our clinic between the years 2008 and 2012.We achieved prospective follow up of these patients. In the result of this follow-up we determined that 35 patients were exposed to pesticide intoxication. Result: Age interval of 35 patients was between 23 and 90. Mean age was 61.7. 33 of them were male and the other patients were female. We determined that 10% of 350 patients were exposed to toxic substances. The first sign of 8 patients was bradykinesias and the first sign of 27 patients was tremor. We determined pesticide intoxication in 19 patients, CO2 intoxication in 2 patients, heavy metal exposure in 10 patients, CO2 intoxication in 2 patients and electrical charging in 2 patients. Finally, we thought 10% toxic etiology risk factor. The other characteristics that we were interested in were male patients. The patients were male and PH signs were in association with tremor.
Abstract - WCN 2013 No: 2369 Topic: 7 - Neuromuscular disorders Initial dip and bilobed N21 peak in the tibial nerve SEPs suggest predominant demyelination in the nerve root T. Chibaa, M. Sonooa, C. Oishib, K. Uchinoa, H. Okumaa, H. Kuronoa, K. Hokkokua, Y. Hatanakaa. aDepartment of Neurology, Teikyo University School of Medicine, Itabashi-ku, Japan; bDepartment of Neurology, Kyorin University School of Medicine, Mitaka, Japan Background: Sensory GBS or CIDP may be difficult to diagnose, especially when routine nerve conduction studies show unremarkable findings. Tibial nerve SEPs are useful because they can evaluate the proximal portions of the peripheral nerve. We noticed a previouslyundescribed characteristic pattern in tibial nerve SEPs of such patients. Objective: To document the significance of the “initial dip and bilobed N21 peak” pattern in the tibial nerve SEPs. Material and methods: Subjects consisted of 4 patients presenting with this pattern. Their clinical and electrophysiological records were retrospectively reviewed. Results: All patients presented with pure sensory or sensory-dominant symptoms. Routine nerve conduction studies did not show any sign of demyelination for all patients, except for the slightly reduced amplitude of the sensory nerve action potentials. The final diagnoses were sensory GBS in 2 patients, sensory CIDP in 1 patient, and sensory-dominant MADSAM in 1 patient. Tibial nerve SEPs revealed slightly delayed N8 and P15 components. The L1S-ICc (ipsilateral iliac crest) lead registered a positive dip 2 to 3 ms later than the P15 latency for all patients, and the N21 peak was either lost (1 patient) or showed a characteristic bilobed pattern (3 patients) with the later peak at a markedly delayed latency. Conclusion: The positive peak must correspond to the “killed-end potential” suggesting the near interruption of the sensory conduction. The mechanism of bilobed N21 potential is unknown, but the recognition of this characteristic pattern will help to identify demyelination of the sensory nerve predominant at the root level. doi:10.1016/j.jns.2013.07.1644
Abstract — WCN 2013 No: 2433 Topic: 7 — Neuromuscular disorders Ultrasound diagnostics and amyotrophic lateral sclerosis Y. Rushkevich, G. Zabrodets, S. Likhachev. Republican Research and Clinical Center of Neurology and Neurosurgery, Minsk, Belarus Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with progressive destruction of the central (upper) and peripheral (lower) motor neurons. One of the specific symptoms of ALS is the appearance of fasciculations, which may precede the development of muscle weakness and atrophy. Objective: To explore the possibilities of ultrasound to detect muscle fasciculations in the differential diagnosis of suspected ALS. Materials and methods: 150 patients were included (ALS, spinal amyotrophy, cervical myelopathy — 126 (84%), 11 (7%), 13 (9%) patients, respectively). Male/female — 93/37, age (median (25—75%)) — 56 (46, 62). The diagnosis of ALS was based on an analysis of clinical data electroneuromyographic El Escorial criteria (Brooks, 1998). All patients underwent electromyography (VikingSelect; Nicolet; USA). Ultrasound