Su-Youn Kim

P566: Factors associated with the time to next attack in neuromyelitis optica: accelerated failure time models with random effects

S.-M. Kim1, S.-Y. Park2, J.Y. Kim3, J.-J. Sung1, K.S. Park3, O. Kwon4, S.H. Kim5, J. Park5, K.-W. Lee1 1Seoul Nationl University Hospital, Neurology, Seoul, Republic of Korea; 2National cancer center, Neurology, Seoul, Republic of Korea; 3Seoul National University, Bundang Hospital, Neurology, Gyeonggi, Republic of Korea; 4Eulji General Hospital, Neurology, Seoul, Republic of Korea; 5Seoul National University Hospital, Medical Research Collaborating Center, Seoul, Republic of Korea

P7-3 Motor unit number estimation in evaluating disease progression in patients with amyotrophic lateral sclerosis

Therefore, we investigated differences in electrophysiological findings and clinical symptoms of ALS by comparing patients with rapid and slow clinical courses. In 40 ALS patients, 14 patients showed a clinical course lasting less than one year and 13 patients showed a clinical course lasting more than three years. The mean interval from onset to diagnosis of ALS patients with a rapid clinical course was shorter than that of patients with a slow clinical course (4.3 months vs. 20 months P< 0.05). Patients with a rapid clinical course showed FPs in almost all muscles in the examined extremities. The muscle number showing FPs was more than that of patients with a slow clinical course (96.3% vs. 53.1% P< 0.05). However, the rate of muscle with fibs-sw between these groups did not differ significantly. Conclusion: The rate of muscle showing FPs may assist in predicting the clinical prognosis.

P7-4 Reproducibility of the motor unit number index (MUNIX) in normal and ALS subjects

Therefore, we investigated differences in electrophysiological findings and clinical symptoms of ALS by comparing patients with rapid and slow clinical courses. In 40 ALS patients, 14 patients showed a clinical course lasting less than one year and 13 patients showed a clinical course lasting more than three years. The mean interval from onset to diagnosis of ALS patients with a rapid clinical course was shorter than that of patients with a slow clinical course (4.3 months vs. 20 months P< 0.05). Patients with a rapid clinical course showed FPs in almost all muscles in the examined extremities. The muscle number showing FPs was more than that of patients with a slow clinical course (96.3% vs. 53.1% P< 0.05). However, the rate of muscle with fibs-sw between these groups did not differ significantly. Conclusion: The rate of muscle showing FPs may assist in predicting the clinical prognosis.

P2-11 Quantitative sensory test: normal range in Korean adults and application to diabetic polyneuropathy

Purpose: It is believed that small peripheral nerve disorders are present from an earlier stage in diabetic patients. In this study, in order to assess the functions of the Ad nerve fibers of diabetic patients, we examined the pain threshold using intraepidermal electrical stimulation. Subjects: The subjects comprised 26 diabetic patients. A 23 healthy subjects without diabetes served as controls. Method: Using a surface stimulation device and stimulating electrode NM990 manufactured by the Nihon Kohden and measured the pain threshold in the inner side of the legs and the knees on both sides based on the method of Inui, et al. (Pain 96; 247, 2002). Results: The mean pain threshold values in the patient group were 0.03±0.02 mA in the both legs, 0.05±0.04 mA in the right knee, and 0.05±0.03 mA in the left knee, while the mean pain threshold values of the control group were 0.03±0.01 mA in the both legs, 0.03±0.01 mA in the right knee, and 0.04±0.02 mA in the left knee, and a statistically significant difference was observed only in the right knee (p < 0.05). Discussion and Conclusion: No abnormalities were observed in the pain thresholds of the diabetic patients except in the right knee. In patients with diabetic peripheral neuropathy, small fibers such as C and Ad fibers become impaired first, but there are also reports that, in immunopathological studies, impaired Ad fibers recover faster than the C fibers. We showed that no abnormalities in both legs were observed in the pain thresholds in th present study reflected the result that the function of legs was maintained through the continuous regeneration of Ad fibers in patients with diabetes. The reason for satistically difference of right knee threshold in patient group remains unclarified.

PO5.38 Electrophysiological Analysis between Chronic Inflammatory Demyelinating Polyradiculoneuropathy with and without Diabetes Mellitus

nerves respectively. The average number of plantar nerves with nerve conduction abnormalities was 2.4 for each patient. The most common abnormalities were low amplitude in the compound nerve action potential, suggesting that early diabetic sensory polyneuropathy is due to axonal degeneration. Conclusions: Plantar nerve study is useful for detecting nerve conduction abnormalities in the early stage of diabetic sensory polyneuropathy. It is a simple, sensitive, and objective means even in the patients with normal routine NCS.

PO5.39 Clinical and Electrophysiological Analysis of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

nerves respectively. The average number of plantar nerves with nerve conduction abnormalities was 2.4 for each patient. The most common abnormalities were low amplitude in the compound nerve action potential, suggesting that early diabetic sensory polyneuropathy is due to axonal degeneration. Conclusions: Plantar nerve study is useful for detecting nerve conduction abnormalities in the early stage of diabetic sensory polyneuropathy. It is a simple, sensitive, and objective means even in the patients with normal routine NCS.

PO5.36 The Value of Measuring Current Perception Threshold in Diabetic Sensory Polyneuropathy with Normal Routine NCS

hands according to neurophysiological criteria. Mean nerve conduction velocities from digit to C1, C2, C3 and C4 were 53.0±10.2 ms, 47.9±7.3, 38.2±8.6 ms, and 38.0±8.8 ms respectively. Mean segmental nerve conduction velocities of C1 C2, C2 C3 and C3 C4 are 41.8±16.9 m/s, 23.4±13.3 and 36.5±11.0 m/s respectively with a minimum value from C2 C3; which represents the segment of median nerve across the carpal ligament. When compared with C2-C3 segment there is a significant difference of nerve conduction velocities of C1 C2 (p < 0.001) and C3 C4 (p = 0.001). Median sensory nerve action potentials (MSNAP) at C1, C2, C3 and C4 were 1.3±8.5, 6.6±3.8, 8.1±5.1mV and 9.7±5.5mV respectively. Median motor distal latency is negatively correlated (r = .552) to NCV of C2 C3 segment (p < 0.01). Conclusions: The maximum slowing of median sensory nerve conduction velocity was obtained across the distal segment of carpal ligament.

PO5.21 Hypertrophy of Spinal Nerve Root in Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Background: Lewis Sumner syndrome (LSS) is a dysimmune neuropathy characterized by multifocal motor and sensory demyelinating neuropathy with conduction blocks. Although the controversy still remains as to whether LSS is multifocal variant of chronic inflammatory demylinating polyneuropathy (CIDP) or transitional form between CIDP and multifocal motor neuropathy (MMN), the recognition of LSS is important for determining appropriate therapeutic modalities. We report a patient with chronic relapsing LSS, who dramatically responded only to plasma exchange (PE). Case report: A 32-year old man, who had experienced an episode of motor weakness and paresthesia in left hand, admitted with the second attack of sensory disturbance in right hand. The nerve conduction study showed sensorimotor mononeuropathy multiplex with multiple conduction blocks. Third attack was diplopia suggestive of left abducens nerve palsy and fourth attack has come to right peroneal nerve. Nerve biopsy on right superficial peroneal nerve revealed marked interfascicular variation of demyelination. Nerve hypertrophy on semithin sections suggested onion-bulb formations. Under the diagnosis of LSS, various immune modulating therapy was tried including steroid, azathioprine, and intravenous immunoglobulins (IVIg), but all of them were not effective. PE was finally tried and made a dramatic effect both on clinical and electrophysiological findings. Later, this patient had several additional attacks of focal neuropathy with an interval of 8~10 months. All the subsequent attacks were also resolved by PE. Conclusions: LSS is generally responsive to immune-modulating therapies, such as high dose of steroid, azathioprine and IVIg. Among treated patients, two-thirds responded to these therapies and one-third of responders experience prolonged remission. Although PE is not usually considered for first-line therapy in LSS, it can be used as an effective alternative therapy in some cases of refractory LSS.