Se Ho Oh

Idiopathic Spinal Cord Herniation as a Treatable Cause of Progressive Brown-Sequard Syndrome

Idiopathic spinal cord herniation is a rare spinal cord disorder caused by spinal cord prolapse through a adural defect. It is a curable disease, so early detection is of particular importance. We report a 38-year-old woman with Brown-Sequard syndrome which was caused by the thoracic spinal cord herniation. Her weakness was almost completely resolved after surgical management, which emphasizes the importance of early diagnosis and surgical management in this rare disease entity.

PO6.10 A Case of Motor Neuron Disease Associated with Multiple Myeloma

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.

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.

PO5.37 Plantar Nerve Study in Patients with Diabetic Sensory Polyneuropathy and 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.

PO5.36 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.

PO14.18 Transient Global Amnesia with Both Temporal Spikes and Hippocampal High Signal Intensities on the Diffusion Weighted Images

Hirotaka Kosaka1,2 *, Masao Omori3, Toshio Munesue4, Makoto Ishitobi1, Yukiko Matsumura1, Tetsuya Takahashi1, Kousuke Narita1,5, Tetsuhito Murata1, Hidehiko Okazawa2, Norihiro Sadato2,6,7, Yuji Wada1 1Dept. of Neuropsychiatry, University of Fukui, Japan, 2Biological Imaging Research Center, University of Fukui, Japan, 3Dept. of Social Welfare Science, Fukui Prefectural University, Japan, 4Research Center for Child Mental Development, Kanazawa University, Japan, 5Dept. of Psychiatry and Human Behavior, Gunma University Graduate School of Medicine, Japan, 6Dept. of Cerebral Research, National Institute for Physiological Sciences, Japan, 7Japan Science and Technology Agency and Technology for Society (RISTEX), Japan E-mail address: hirotaka@u-fukui.ac.jp

PO5.10 A Case of Complete Ophthalmoplegia with the Antibody to GT1a Ganglioside

Background: To analyze the clinical and electrophysiological features of the painful diabetic peripheral neuropathy (PDPN). Methods: Thirty-two patients with PDPN, whose history was more than 1 year and visual analog scale (VAS) was more than 4, were recruited and investigated. Twenty well-matched healthy volunteers as controls. Conventional motor and sensory nerve conduction velocity (NCV) and quantitative sensory testing (QST) were performed. The relations between VAS and clinic, eletrophysiology were analyzed. Results: NCV of 13 patients showed normal, but in11 of them QST revealed abnormal. The abnormality rates of NCV, QST and NCV+QST were 59.4%, 87.5% and 93.7%, respectively. The relations between VAS and heat pain threshold (HP) of QST, VAS and tibial sensory nerve conduction velocity (SCV) were found. Conclusions: Both small nerve fibers (SNF) and thick myelin fibers were involved, specially SNF in early stage of PDPN. QST is sensitive means for the early diagnosis of PDPN. VAS could be associated with C-nerve fibers and tibial sensory nerve. Combining QST with NCV can give us a comprehensive evaluation of peripheral nerve function.

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.

PO1.15 The Changes in the Mixed Nerve Conduction with Age in the Medial and Lateral Plantar Nerves

Background: Anterior interosseous nerve (AIN) syndrome is a clinical entity characterized by paresis of the pronator quadratus, the flexor pollicis longus (FPL), and the flexor digitorum profundus. Previous study described a nerve conduction study using surface electrode by recording compound muscle action potentials from FPL. The purpose of this study was to determine the normal motor conduction values for the AIN in the Korean. Methods: Nerve conduction studies of the AIN were performed on 100 healthy subjects (51 women and 49 men, aged 26 to 60 years). The median nerve was stimulated supramaximally in antecubital fossa, just medial to the pulsation of the brachial artery. The active surface recording electrode was positioned over the belly of the flexor pollicis longus, 6 8 cm proximal to the radial styloid, and moved so as to obtain a negative takeoff. And, we applied the normative data to a patient with the AIN syndrome. Results: In 100 normal controls (200 different AIN studies), mean CMAP amplitude and area were 9.8±1.4 mV and 34.0±4.2 mV.ms. Mean CMAP onset latency was 3.7±0.4 ms. and mean velocity was 46.0±4.1 m/s. In patient with AIN syndrome, the CMAP amplitude and onset latency were 6.5 mV and 3.1 ms. (13.3 mV and 3.0 ms in asymptomatic side) Conclusions: Normative data has been established in the Korean and the CMAP amplitudes were larger than that of previous study and onset latency was similar. This normative data may be used to assess AIN syndrome.