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PO6.11 Nocturnal Capnography Monitoring in Patients with Amyotrophic Lateral Sclerosis
2009 Asian and Oceanian Congress of Clinical Neurophysiology of atrophy of the brain according to the degree of corticospinal tract degeneration. Results: Patients showed a gray matter volume decrease in several frontal and temporal areas bilaterally (P < 0.01 uncorrected) compared with controls in VBM study. The premotor cortical atrophy was observed in the disease progression using with the Korean-ALS-Functional rating scales-revised (K-ALSFRS-r) score. As the disease progressed, motor and extramotor systems were all involved regardless of CMCT, indirect indices of corticospinal tract degeneration. Decreased respiratory function affected the parahippocampal & occipital cortical atrophic changes. Motor systems predominantly affected in the limb-onset group compared to bulbar onset showing the fronto-temporal atrophic changes in the subgroup analysis. Conclusions: These data supported the theory that ALS is multisystem disorder affecting motor system and extra-motor system including cerebellum. Also these widespread systematic degenerations can be intercurrently affected by the physiological deterioration such as chronic hypoxia. PO6.10 A Case of Motor Neuron Disease Associated with Multiple Myeloma Se Ho Oh1 *, Sang-Soon Park1 , Yoon-Ho Hong3 , Seong-Ho Park1 , Kwang-Woo Lee2 , Kyung Seok Park1 1 Dept. of Neurology, Seoul National University Bundang Hospital, College of Medicine, Seoul National University Hospital, Korea, 2 Dept. of Neurology, Seoul National University Hospital, College of Medicine, Seoul National University, Korea, 3 Dept. of Neurology, Seoul Boramae Municipal Hospital, College of Medicine, Seoul National University, Korea E-mail address: [email protected] Background: Motor neuron disease (MND) is known to occur as a paraneoplastic syndrome. We report a case of motor neuron disease associated with multiple myeloma. Case report: A 47-year-old man presented with progressive limb muscle weakness and atrophy over the prior 3 years. He suffered from poliomyelitis at the age of 3 in the left leg. On initial neurological examination, he showed right lower limb weakness with atrophy and generalized hyporeflexia. During the follow-up, bilateral upper limb weakness was developed. Electromyography revealed abnormal findings suggestive of motor neuron disease. Serum and urine electrophoresis revealed monoclonal gammopathy. A diagnosis of multiple myeloma was confirmed by bone marrow biopsy. He was treated with chemotherapy and bone marrow transplantation. During the follow-up of 2 years, his limb weakness was slightly improved. Conclusions: Our case provides elements for a causal relationship between MND and paraproteinemia, and suggests the need of careful search for the underlying malignancy in the patients with MND. PO6.11 Nocturnal Capnography Monitoring in Patients with Amyotrophic Lateral Sclerosis Sung-Min Kim1 *, Yoon-Ho Hong2 , Kyung Seok Park1 , Hyun-Woo Nam1 , Jung-Joon Sung1 , Kwang-Woo Lee1 1 Dept. of Neurology, Seoul National University College of Medicine, Korea, 2 Dept. of Neurology, Seoul Boramae Hospital, Korea E-mail address: [email protected] Background: Patients with amyotrophic lateral sclerosis (ALS) suffer from nocturnal hypoventilation. The aim of this study is to evaluate the efficacy of nocturnal capnography for detecting nocturnal hypoventilation in patients with ALS. Methods: Thirty three definite ALS patients were included, most of whom had symptoms of dyspnea, orthopnea, or dyspnea on exertion. Patients underwent nocturnal capnography monitoring, nocturnal pulse oximetry monitoring, daytime arterial blood gas analysis (ABGA), measurement of forced vital capacity (FVC) and ALS functional score revised (ALSFRSr). Patients with severe nocturnal hypercapnea or with severe subjective dyspnea were treated with non-invasive ventilation (NIV). Spearman’s correlation test was used for statistical analysis. Results: Duration of nocturnal hypoventilation (a duration of time when end tidal CO2 >47 mmHg in capnography) correlated well with duration of nocturnal hypoxia (p = 0.001), daytime blood CO2 level (p = 0.000), and degree of orthopnea in ALSFRSr (p = 0.048). Patients with longer
S63 duration of nocturnal hypocapnea also showed good NIV compliance after monitoring. (p = 0.028) Conclusions: Nocturnal capnography is a simple, efficient tool in measuring nocturnal hypoventilation in patients with ALS. Moreover, this can also be useful in detecting ALS patients who are in need of NIV and who will show good compliance to NIV treatment. PO6.12 Mesenchymal Stem Cell as a Neuroprotective Therapy in Patients with Amyotrophic Lateral Sclerosis Hee-Kyung Park *, Hyun Young Kim, Seong-Ho Koh, Young Joo Lee, Kyu-Yong Lee, Hee Jin Kim, Juhan Kim, Hee-Tae Kim, Seung H. Kim Dept. of Neurology, Hanyang Univeristy Medical Center, Korea E-mail address: [email protected] Background: Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease characterized by selective loss of motor neurons, leading to death. Especially, thanks to the increasing understanding of crucial roles of supporting cells for motor neuron survival, mesenchymal stem cells (MSCs) have been focused as a neuroprotective therapy. We aimed to determine whether MSCs therapy is safe and effective in slowing disease progression in ALS. Methods: Eighteen patients with definite ALS were recruited. The diagnosis of ALS was based on El Escorial criteria. MSCs were extracted from autologous bone marrow, and ex vivo expanded for about 4 weeks. Expanded MSCs were injected through intrathecal route twice at an interval of 4 weeks. ALS functional rating scale-revised (ALSFRS-R) was used as a primary scale for evaluating the effect of treatment. The patients were monitored by ALSFRS-R scale, pulmonary function tests, neurophysiological assessments, and laboratory findings. With lead-in 3 months period, patients were followed up for 6 months after the first treatment. As a primary efficacy measurement, the change of slope of ALSFRS-R scores before and after treatment was used. Results: Compared with the ALSFRS-R score change during lead-in period, MSCs therapy could reduce the change of slope of ALSFRS-R scores during 1st half follow up period statistically. These disease modifying effects were observed in 12 patients among total 18 ALS patients. Moreover, immediate beneficial effects such as improvement of muscle power, breathing stabilization, decreased drooling and spasticity, were observed in 14 patients. Conclusions: Disease modifying with slowing down the disease progression has not been reported yet. Moreover, there are no serious adverse events in this study. Therefore, MSCs therapy with intrathecal injection could provide an effective and safe strategy for ALS. PO6.13 The Physiological Basis of the Split Hand Syndrome in ALS: An Axonal Excitability Study Kazuaki Kanai1 *, Jong Seok Bae2 , Setsu Sawai1 , Sonoko Misawa1 , Sagiri Isose1 , Satoshi Kuwabara1 1 Dept. of Neurology, Graduate School of Medicine, Chiba University, Japan, 2 Dept. of Neurology, Busan Paik Hospital, Inje University College of Medicine, Korea E-mail address: [email protected] Background: Previous studies have shown the “split hand syndrome” in patients with amyotrophic lateral sclerosis (ALS), in which the abductor pollicis brevis (APB) and first dorsal interosseous (FDI) are more severely involved than abductor digiti minimi (ADM). Recent studies have also revealed abnormal motor nerve excitability in ALS. To clarify the underlying pathophysiology of the split hand syndrome, we investigated whether excitability properties differ among APB-, FDI-, and ADM-motor axons. Methods: We examined nerve excitability properties of APB, FDI and ADM motor axons in the 12 healthy volunteers using multiple excitability measurements with threshold tracking. In 6 of the subjects, we also examined the excitability properties of the nerves using latent addition methods. All subjects gave informed consent to the experimental procedures approved by the ethics committee of the Chiba university School of Medicine. Results: The strength-duration time constant, which depends on persistent sodium conductance, was significantly longer in APB and FDI axons than in ADM axons. The latent addition studies showed greater threshold changes at the conditioning-test stimulus of 0.2 ms in APB and FDI than in ADM. These findings suggest that persistent sodium
S63 duration of nocturnal hypocapnea also showed good NIV compliance after monitoring. (p = 0.028) Conclusions: Nocturnal capnography is a simple, efficient tool in measuring nocturnal hypoventilation in patients with ALS. Moreover, this can also be useful in detecting ALS patients who are in need of NIV and who will show good compliance to NIV treatment. PO6.12 Mesenchymal Stem Cell as a Neuroprotective Therapy in Patients with Amyotrophic Lateral Sclerosis Hee-Kyung Park *, Hyun Young Kim, Seong-Ho Koh, Young Joo Lee, Kyu-Yong Lee, Hee Jin Kim, Juhan Kim, Hee-Tae Kim, Seung H. Kim Dept. of Neurology, Hanyang Univeristy Medical Center, Korea E-mail address: [email protected] Background: Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease characterized by selective loss of motor neurons, leading to death. Especially, thanks to the increasing understanding of crucial roles of supporting cells for motor neuron survival, mesenchymal stem cells (MSCs) have been focused as a neuroprotective therapy. We aimed to determine whether MSCs therapy is safe and effective in slowing disease progression in ALS. Methods: Eighteen patients with definite ALS were recruited. The diagnosis of ALS was based on El Escorial criteria. MSCs were extracted from autologous bone marrow, and ex vivo expanded for about 4 weeks. Expanded MSCs were injected through intrathecal route twice at an interval of 4 weeks. ALS functional rating scale-revised (ALSFRS-R) was used as a primary scale for evaluating the effect of treatment. The patients were monitored by ALSFRS-R scale, pulmonary function tests, neurophysiological assessments, and laboratory findings. With lead-in 3 months period, patients were followed up for 6 months after the first treatment. As a primary efficacy measurement, the change of slope of ALSFRS-R scores before and after treatment was used. Results: Compared with the ALSFRS-R score change during lead-in period, MSCs therapy could reduce the change of slope of ALSFRS-R scores during 1st half follow up period statistically. These disease modifying effects were observed in 12 patients among total 18 ALS patients. Moreover, immediate beneficial effects such as improvement of muscle power, breathing stabilization, decreased drooling and spasticity, were observed in 14 patients. Conclusions: Disease modifying with slowing down the disease progression has not been reported yet. Moreover, there are no serious adverse events in this study. Therefore, MSCs therapy with intrathecal injection could provide an effective and safe strategy for ALS. PO6.13 The Physiological Basis of the Split Hand Syndrome in ALS: An Axonal Excitability Study Kazuaki Kanai1 *, Jong Seok Bae2 , Setsu Sawai1 , Sonoko Misawa1 , Sagiri Isose1 , Satoshi Kuwabara1 1 Dept. of Neurology, Graduate School of Medicine, Chiba University, Japan, 2 Dept. of Neurology, Busan Paik Hospital, Inje University College of Medicine, Korea E-mail address: [email protected] Background: Previous studies have shown the “split hand syndrome” in patients with amyotrophic lateral sclerosis (ALS), in which the abductor pollicis brevis (APB) and first dorsal interosseous (FDI) are more severely involved than abductor digiti minimi (ADM). Recent studies have also revealed abnormal motor nerve excitability in ALS. To clarify the underlying pathophysiology of the split hand syndrome, we investigated whether excitability properties differ among APB-, FDI-, and ADM-motor axons. Methods: We examined nerve excitability properties of APB, FDI and ADM motor axons in the 12 healthy volunteers using multiple excitability measurements with threshold tracking. In 6 of the subjects, we also examined the excitability properties of the nerves using latent addition methods. All subjects gave informed consent to the experimental procedures approved by the ethics committee of the Chiba university School of Medicine. Results: The strength-duration time constant, which depends on persistent sodium conductance, was significantly longer in APB and FDI axons than in ADM axons. The latent addition studies showed greater threshold changes at the conditioning-test stimulus of 0.2 ms in APB and FDI than in ADM. These findings suggest that persistent sodium