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FC38.4 Evoked potentials (EP) in patients with frontal and occipital type of childhood adrenoleukodystrophy (ALD)
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Oral Communications / Clinical Neurophysiology 117 (2006) S49–S111
Results: Conditioning peripheral stimulation significantly modulated MEP size in all muscles of normal controls and WC patients. Control subjects exhibited an inhibitory effect at the C-T interval of 200 ms after both MN and index finger stimulation, and a facilitatory effect at the CT interval of 50 ms only after MN stimulation. In WC patients, facilitatory effect was decreased in the symptomatic side. After rTMS, sensorimotor integration tended to be normalized. Conclusion: The processing of proprioceptive inputs is disturbed in WC. Premotor rTMS may restore a normal sensorimotor integration. doi:10.1016/j.clinph.2006.06.125
FC38.2 Depth median nerve SEPs recorded in human supplementary motor area C. Barba 1, G. Colicchio 2, F. Mauguie`re 3 1 2 3
IRCCS, S. Lucia Foundation, Italy Catholic University, Neurosurgery Department, Italy Hopital Neurologique, France
Background: The question whether short latency SEPs could be generated in motor areas and in particular in supplementary motor area still remains debated. Objective: To record median nerve (MN) SEPs by stereotactically electrodes implanted in supplementary motor area (SMA)of 14 epileptic patients (9 in pre-SMA, 3 in SMA-proper, 2 in both) in order to determine short and middle-latency SEPs sources eventually generated in this area. Methods: Short and middle-latency MN SEPs were recorded by chronically implanted electrodes in the fronto-temporal cortex and in particular in the mesial frontal region of 14 drug-resistant epileptic patients. MN stimulations of 100 ls were delivered by skin electrodes at the wrist; stimulus intensity was adjusted slightly above the motor threshold. Results: No early SEP was originated in the whole SMA. Conversely, middle-latency SEPs were originated in pre-SMA but not in SMA-proper as demonstrated by both referential and bipolar recordings. In particular offline computed bipolar traces between neighbouring contacts implanted in the pre-SMA and in the frontal external regions showed a phase reversal at the deepest contacts located in pre-SMA. Conversely, bipolar recordings between neighbouring contacts implanted in the SMA-proper and in the frontal external regions showed inversion recovery at more superficial contacts, implanted in area 6. Conclusions: We concluded that SEPs seem to originate in pre-SMA and area 6 but not in SMA-proper. doi:10.1016/j.clinph.2006.06.127
FC38.3 Characterization of somatosensory discrimination responses using scalp and intracranial recordings L.A. Spackman 1, S.G. Boyd 2, A. Towell 3 1
Institute of Child Health, UK Great Ormond Street Hospital, Department of Clinical Neurophysiology, UK 3 University of Westminster, Department of Psychology, UK 2
Background: The auditory mismatch negativity (aMMN) is an event-related potential (ERP) elicited by an infrequent change in a stream of continuous, repetitive stimuli. It is thought to result from a comparison between the physical features of the deviant stimulus and a neural sensory memory trace of the standard stimulus. A limited number of investigations have suggested an analogous somatosensory discriminatory response. Objective: To examine the effects of frequency and duration change on somatosensory discrimination responses using scalp and intracranial recordings. Methods: Intermittent vibration to the fingertips of either hand was presented in a 2-stimulus oddball paradigm (deviant p = 0.10). Stimulus pairs of 20/70 ms, 50/ 150 ms and 170/250 ms were presented at 70 Hz to one group (N = 12, 18–38 years), and frequency pairs of 200/ 70 Hz to a second group (N = 10, 19–34 years). Recordings were made from subdural electrodes to similar stimuli in a third group (N = 9, 6–16 years) undergoing presurgical evaluation for epilepsy. Results: A negative/positive shift was recorded in the response to the deviant stimuli to both frequency and duration increments/decrements. The initial negativity had a mean onset of 90–170 ms and the peak latencies of both components were dependent on stimulus duration. The negative component appeared maximal over the hemisphere contralateral to the side of stimulation and anterior to the maximum P50/N70 components. The positive component appears more posterior over the mid parietal region, suggesting a separate generator, possibly in the secondary somatosensory cortex. It was most prominent with frequency deviations. Three intracranial cases showed a negative shift over the frontal areas in response to the deviant stimuli, suggesting a third generator. Conclusion: We propose that these changes in the deviant responses reflect a somatosensory mismatch response with features similar to the aMMN. doi:10.1016/j.clinph.2006.06.128
FC38.4 Evoked potentials (EP) in patients with frontal and occipital type of childhood adrenoleukodystrophy (ALD) M. Kaga, M. Inagaki, S. Suzuki, A. Gunji, Y. Inoue, A. Ishiguro National Institute of Mental Health, National Center of Neurology and Psychiatry, Department of Developmental Disorders, Japan
Oral Communications / Clinical Neurophysiology 117 (2006) S49–S111
Background: ALD is a progressive metabolic disease. Their lesions usually begin at occipital lobe. However, in 15 percent of patients, subcortical white matter of frontal lobe is the first lesion. We have investigated the early signs and symptoms of neuropsychological and neurophysiological findings in ALD. Objectives: The purpose of our study is to clarify whether their EP show different pattern in their disease process. Methods: Five children with frontal type (10–18 years) and 18 occipital type (5–11 years) participated in this study. EPs were measured at rather early stage and they were auditory brainstem response (ABR), auditory middle latency response (MLR), slow vertex response (SVR), visual evoked potentials (VEP) and short latency sensory evoked potentials (SSEP). Methods are already reported elsewhere. The results of two groups were compared with our basic data of healthy children. Results: In frontal type of patients, central conduction time (CCT) of ABR was delayed in all five. MLR was normal in 3 among 3 patients examined. SVR was normal in 4 out of 5 patients. VEP was normal in all 4 patients examined. CCT of SSEP was delayed in 2 out of 3 patients. In occipital type patients, their ABRs were basically normal in 13 patients. CCT of ABR was delayed in 4 patients. Otological condition affected ABR in 5 patients. MLR was normal in 9 out of 10 patients. SVR was normal in 5 and delayed in 6 out of 11 patients examined. VEP was normal in 6 and delayed in 6 among 12 patients. SSEP was normal in 8. Delayed CCT was recorded in 4 patients. Conclusions: Frontal and occipital type of ALD showed different tendency of EP abnormalities. Frontal type patients tend to show prolonged CCT of ABR with normal VEP while occipital type do the vice versa at the early stage of the disease. This shows the different propagation of the disease process of the same disease and can contribute to presume their clinical prognosis and to evaluate therapeutic procedure. doi:10.1016/j.clinph.2006.06.129
FC39.1 The use of intravenous immunoglobulins for the treatment of diabetic lumbosacral radiculoolexus neuropathy S. Tamburin, A. Forgione, D. Idone, G. Zanette Section of Neurology, Hospital Pederzoli, Department of Neurological Sciences and Vision, Verona, Italy Background: Diabetic lumbosacral radiculoplexus neuropathy (DLRPN) is a rare painful condition that may occur in diabetic patients. At the moment there is no proven treatment for DLRPN. Objective: To evaluate the effect of intravenous immunoglobulin (IVIg) therapy in the treatment of DLRPN. Methods: We recruited four patients affected by type II diabetes mellitus and DLRPN. They complained of
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thoracic and abdominal pain and developed painful lower-limb proximal weakness. Clinical examination and instrumental findings were suggestive of lumbosacral plexopathy and bilateral distal neuropathy. Sural nerve biopsy showed degeneration and loss of fibers with perivascular inflammation. Treatment with gabapentin, amytriptiline, carbamazepine, clonazepam and tramadole, even at high dosages, could not alleviate truncal and lower-limb pain. All patients were treated with IVIg (0.4 g/Kg/day for 5 days). Results: Immediately after IVIg treatment 3 patients improved. In particular, pain was alleviated and patients could reduce the dosage of analgesic drugs. One of the patients needed a second treatment with IVIg after 6 months. Conclusions: IVIg treatment may improve symptoms of patients with DLRPN. Our data could represent a starting point for a double-blind study aimed to evaluate the role of IVIg in the treatment of patients affected by DLRPN. doi:10.1016/j.clinph.2006.06.130
FC39.2 Use of melatonin in paediatric sleep EEGs S. Goyal, R. Arunachalam, S. Becker, F. Brunnhuber King’s College Hospital, Department of Clinical Neurophysiology, UK Melatonin is being increasingly used in Clinical Neurophysiology departments worldwide to facilitate sleep EEG recordings. We studied its efficacy in 57 outpatient paediatric patients referred for sleep EEG and compared it with 28 children who received Trimeprazine (Vallergan). The medication was given just prior to application of electrodes. Successful sleep was defined as the attainment of stage II sleep based on Rechtschaffen and Kales criteria. The first 10-s epoch containing sleep spindles was taken as the onset of sleep. At least 20 min of stage II sleep was recorded in all patients. The mean age for the Melatonin group was 6.9 years (range 0.5–16) and 5.5 (range 1–11) years for the Vallergan group. The proportion of children with abnormal EEGs was similar in both groups – 26 (43%) in the Melatonin and 14(50%) in the Trimeprazine group. 47(81%) children in the Melatonin and 20 (71%) in the Trimeprazine group achieved stage II sleep. The mean time to fall asleep from the beginning of the recording was significantly shorter for the melatonin group (31 min) compared with Trimeprazine (42 min) (p < 0.03). The total duration of the test was significantly shorter in the Melatonin group (56 min vs. 87 min, p < 0.01). The results suggest that Melatonin is a very effective drug aiding sleep EEG recordings in children. The latency to achieve stage II sleep and time spent by the child in the EEG department is significantly shorter than with Trimeprazine, and children do not need to be checked by medical staff prior to discharge. The use of Melatonin in children shows a
Oral Communications / Clinical Neurophysiology 117 (2006) S49–S111
Results: Conditioning peripheral stimulation significantly modulated MEP size in all muscles of normal controls and WC patients. Control subjects exhibited an inhibitory effect at the C-T interval of 200 ms after both MN and index finger stimulation, and a facilitatory effect at the CT interval of 50 ms only after MN stimulation. In WC patients, facilitatory effect was decreased in the symptomatic side. After rTMS, sensorimotor integration tended to be normalized. Conclusion: The processing of proprioceptive inputs is disturbed in WC. Premotor rTMS may restore a normal sensorimotor integration. doi:10.1016/j.clinph.2006.06.125
FC38.2 Depth median nerve SEPs recorded in human supplementary motor area C. Barba 1, G. Colicchio 2, F. Mauguie`re 3 1 2 3
IRCCS, S. Lucia Foundation, Italy Catholic University, Neurosurgery Department, Italy Hopital Neurologique, France
Background: The question whether short latency SEPs could be generated in motor areas and in particular in supplementary motor area still remains debated. Objective: To record median nerve (MN) SEPs by stereotactically electrodes implanted in supplementary motor area (SMA)of 14 epileptic patients (9 in pre-SMA, 3 in SMA-proper, 2 in both) in order to determine short and middle-latency SEPs sources eventually generated in this area. Methods: Short and middle-latency MN SEPs were recorded by chronically implanted electrodes in the fronto-temporal cortex and in particular in the mesial frontal region of 14 drug-resistant epileptic patients. MN stimulations of 100 ls were delivered by skin electrodes at the wrist; stimulus intensity was adjusted slightly above the motor threshold. Results: No early SEP was originated in the whole SMA. Conversely, middle-latency SEPs were originated in pre-SMA but not in SMA-proper as demonstrated by both referential and bipolar recordings. In particular offline computed bipolar traces between neighbouring contacts implanted in the pre-SMA and in the frontal external regions showed a phase reversal at the deepest contacts located in pre-SMA. Conversely, bipolar recordings between neighbouring contacts implanted in the SMA-proper and in the frontal external regions showed inversion recovery at more superficial contacts, implanted in area 6. Conclusions: We concluded that SEPs seem to originate in pre-SMA and area 6 but not in SMA-proper. doi:10.1016/j.clinph.2006.06.127
FC38.3 Characterization of somatosensory discrimination responses using scalp and intracranial recordings L.A. Spackman 1, S.G. Boyd 2, A. Towell 3 1
Institute of Child Health, UK Great Ormond Street Hospital, Department of Clinical Neurophysiology, UK 3 University of Westminster, Department of Psychology, UK 2
Background: The auditory mismatch negativity (aMMN) is an event-related potential (ERP) elicited by an infrequent change in a stream of continuous, repetitive stimuli. It is thought to result from a comparison between the physical features of the deviant stimulus and a neural sensory memory trace of the standard stimulus. A limited number of investigations have suggested an analogous somatosensory discriminatory response. Objective: To examine the effects of frequency and duration change on somatosensory discrimination responses using scalp and intracranial recordings. Methods: Intermittent vibration to the fingertips of either hand was presented in a 2-stimulus oddball paradigm (deviant p = 0.10). Stimulus pairs of 20/70 ms, 50/ 150 ms and 170/250 ms were presented at 70 Hz to one group (N = 12, 18–38 years), and frequency pairs of 200/ 70 Hz to a second group (N = 10, 19–34 years). Recordings were made from subdural electrodes to similar stimuli in a third group (N = 9, 6–16 years) undergoing presurgical evaluation for epilepsy. Results: A negative/positive shift was recorded in the response to the deviant stimuli to both frequency and duration increments/decrements. The initial negativity had a mean onset of 90–170 ms and the peak latencies of both components were dependent on stimulus duration. The negative component appeared maximal over the hemisphere contralateral to the side of stimulation and anterior to the maximum P50/N70 components. The positive component appears more posterior over the mid parietal region, suggesting a separate generator, possibly in the secondary somatosensory cortex. It was most prominent with frequency deviations. Three intracranial cases showed a negative shift over the frontal areas in response to the deviant stimuli, suggesting a third generator. Conclusion: We propose that these changes in the deviant responses reflect a somatosensory mismatch response with features similar to the aMMN. doi:10.1016/j.clinph.2006.06.128
FC38.4 Evoked potentials (EP) in patients with frontal and occipital type of childhood adrenoleukodystrophy (ALD) M. Kaga, M. Inagaki, S. Suzuki, A. Gunji, Y. Inoue, A. Ishiguro National Institute of Mental Health, National Center of Neurology and Psychiatry, Department of Developmental Disorders, Japan
Oral Communications / Clinical Neurophysiology 117 (2006) S49–S111
Background: ALD is a progressive metabolic disease. Their lesions usually begin at occipital lobe. However, in 15 percent of patients, subcortical white matter of frontal lobe is the first lesion. We have investigated the early signs and symptoms of neuropsychological and neurophysiological findings in ALD. Objectives: The purpose of our study is to clarify whether their EP show different pattern in their disease process. Methods: Five children with frontal type (10–18 years) and 18 occipital type (5–11 years) participated in this study. EPs were measured at rather early stage and they were auditory brainstem response (ABR), auditory middle latency response (MLR), slow vertex response (SVR), visual evoked potentials (VEP) and short latency sensory evoked potentials (SSEP). Methods are already reported elsewhere. The results of two groups were compared with our basic data of healthy children. Results: In frontal type of patients, central conduction time (CCT) of ABR was delayed in all five. MLR was normal in 3 among 3 patients examined. SVR was normal in 4 out of 5 patients. VEP was normal in all 4 patients examined. CCT of SSEP was delayed in 2 out of 3 patients. In occipital type patients, their ABRs were basically normal in 13 patients. CCT of ABR was delayed in 4 patients. Otological condition affected ABR in 5 patients. MLR was normal in 9 out of 10 patients. SVR was normal in 5 and delayed in 6 out of 11 patients examined. VEP was normal in 6 and delayed in 6 among 12 patients. SSEP was normal in 8. Delayed CCT was recorded in 4 patients. Conclusions: Frontal and occipital type of ALD showed different tendency of EP abnormalities. Frontal type patients tend to show prolonged CCT of ABR with normal VEP while occipital type do the vice versa at the early stage of the disease. This shows the different propagation of the disease process of the same disease and can contribute to presume their clinical prognosis and to evaluate therapeutic procedure. doi:10.1016/j.clinph.2006.06.129
FC39.1 The use of intravenous immunoglobulins for the treatment of diabetic lumbosacral radiculoolexus neuropathy S. Tamburin, A. Forgione, D. Idone, G. Zanette Section of Neurology, Hospital Pederzoli, Department of Neurological Sciences and Vision, Verona, Italy Background: Diabetic lumbosacral radiculoplexus neuropathy (DLRPN) is a rare painful condition that may occur in diabetic patients. At the moment there is no proven treatment for DLRPN. Objective: To evaluate the effect of intravenous immunoglobulin (IVIg) therapy in the treatment of DLRPN. Methods: We recruited four patients affected by type II diabetes mellitus and DLRPN. They complained of
S101
thoracic and abdominal pain and developed painful lower-limb proximal weakness. Clinical examination and instrumental findings were suggestive of lumbosacral plexopathy and bilateral distal neuropathy. Sural nerve biopsy showed degeneration and loss of fibers with perivascular inflammation. Treatment with gabapentin, amytriptiline, carbamazepine, clonazepam and tramadole, even at high dosages, could not alleviate truncal and lower-limb pain. All patients were treated with IVIg (0.4 g/Kg/day for 5 days). Results: Immediately after IVIg treatment 3 patients improved. In particular, pain was alleviated and patients could reduce the dosage of analgesic drugs. One of the patients needed a second treatment with IVIg after 6 months. Conclusions: IVIg treatment may improve symptoms of patients with DLRPN. Our data could represent a starting point for a double-blind study aimed to evaluate the role of IVIg in the treatment of patients affected by DLRPN. doi:10.1016/j.clinph.2006.06.130
FC39.2 Use of melatonin in paediatric sleep EEGs S. Goyal, R. Arunachalam, S. Becker, F. Brunnhuber King’s College Hospital, Department of Clinical Neurophysiology, UK Melatonin is being increasingly used in Clinical Neurophysiology departments worldwide to facilitate sleep EEG recordings. We studied its efficacy in 57 outpatient paediatric patients referred for sleep EEG and compared it with 28 children who received Trimeprazine (Vallergan). The medication was given just prior to application of electrodes. Successful sleep was defined as the attainment of stage II sleep based on Rechtschaffen and Kales criteria. The first 10-s epoch containing sleep spindles was taken as the onset of sleep. At least 20 min of stage II sleep was recorded in all patients. The mean age for the Melatonin group was 6.9 years (range 0.5–16) and 5.5 (range 1–11) years for the Vallergan group. The proportion of children with abnormal EEGs was similar in both groups – 26 (43%) in the Melatonin and 14(50%) in the Trimeprazine group. 47(81%) children in the Melatonin and 20 (71%) in the Trimeprazine group achieved stage II sleep. The mean time to fall asleep from the beginning of the recording was significantly shorter for the melatonin group (31 min) compared with Trimeprazine (42 min) (p < 0.03). The total duration of the test was significantly shorter in the Melatonin group (56 min vs. 87 min, p < 0.01). The results suggest that Melatonin is a very effective drug aiding sleep EEG recordings in children. The latency to achieve stage II sleep and time spent by the child in the EEG department is significantly shorter than with Trimeprazine, and children do not need to be checked by medical staff prior to discharge. The use of Melatonin in children shows a