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P225 Cases developing motor neuron symptoms after cadmium intoxication
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Abstracts of the 13th European Congress of Clinical Neurophysiology / Clinical Neurophysiology 119 (2008), S1–S131
P224 Electromyographic pattern in Becker muscular dystrophy in comparison with Duchenne muscular dystrophy Barbara Emeryk-Szajewska, Jerzy Kopec Department of Neurology, Medical University of Warsaw Purpose: the aim of this study was to compare the electromyographic pattern in Becker muscular dystrophy (BMD) with that found in Duchenne muscular dystrophy (DMD). Material and method: fourteen men with BMD and 51 boys with DMD were investigated. Proximal muscles were examined: m. biceps brachii (BB) and m. rectus femoris (RF). They were divided according to the clinical criteria in two groups: of those with slight changes (group AB) and of those with severe abnormalities (CD). Our own method of Functional-QEMG was applied in the CNEMG examination. Results: spontaneous activity (fibrillations, complex repetitive discharges) was equally frequent in BMD and DMD. Linked potentials were rather frequent in either group. Myopathic features such as MUAPs low amplitude and area, polyphasic shape were seen in either condition, but more marked in DMD than in BMD (p< 0,05). Evaluation of IP recordings revealed that IP amplitude (amplitude size) is low in DMD already at the early stage of lesion but normal or only slightly diminished in BMD. Conclusion: our results might perhaps suggest different degrees of lesion in type II MUs between the compared types of muscular dystrophy.
Method: Single fiber electromyography (SFEMG) during voluntary contraction of the extansor digitorum communis muscle, nerve conduction studies of upper and lower extremities and concentric needle electromyography of the extansor digitorum communis (EDC) were performed on 20 patients with MD and 12 age-matched normal controls. Mean jitter value was calculated for each subject and it was compared with the values recorded from the normal controls. Results: Both controls and patients with MD had normal nerve conduction studies. Concentric needle EMG studies of the EDC muscle were also normal. Mean jitter values of the MD group did not differ from the normal control subjects (25,4±3,4 µs versus 27,2±2,6 µs, respectively; p=0.06) Conclusion: NMT abnormalities have been detected in migraineurs and cluster headache. It was thought that some migraineurs share similar genetic abnormalities with familial hemiplegic migraine (FHM) patients and have abnormalities of P/Q-type Ca+2 -channels which are present in motor nerve endings and in the brain. MD has been mapped to the DFNA9 locus on chromosome 14, the COCH gene; probably encoding an extracellular matrix protein. Inner hair cells have at least two and outer hair cells have at least three types of K+ -channels. In the cochlea the hair cell channel is KCNQ4. Mutations in two other genes encoding K+ -channel subunits, KCNQ1 and KCNE1, have been found in syndromic hereditary deafness. Clinical features such as sensitivity to dietary ions and reduction of the vertigo attacks with acetazolamide are on behalf of the channelopathy hypothesis. Though it doesn’t rule out the channelopathy hypothesis, a NMT deficit haven’t been found in patients with MD.
P225 Cases developing motor neuron symptoms after cadmium intoxication Refah Sayin, Temel Tombul, Aysel Milanlio˘glu Yuzuncu Yil Üniversity, Medical Faculty of Neurology Department Introduction and Purpose: Toxic and essential metals have been implicated in the pathogenesis of motor neuron disease (MND). Cadmium is a potential candidate for a neurotoxin in MND since it has been shown to be retrogradely transported in motor axons in motor neuron cell bodies. In this study, we present three cases manifesting motor neuron findings associated with cadmium intoxication. Method and Results: Patients with a history of working in such industrial sectors as blacksmith shops, accumulating and lead plaque coverage refered to our clinic with various complaints after 2, 3 and 6 months of the period of their employment. The complaints of these male patients with ages of 17, 18 and 27 were respectively physical pain, fatigue, weakness in limbs, nausea, anorexia, weight loss and involuntary tissue pulsations. In the neurological examination, the first case revealed 4/5 level of motor strength in the upper extremities, +3/5 in the left lower extremity and 4/5 right lower extremity. In the upper extremities of the second case, a level of +4/5 was determined markedly in the distal. In both of the lower extremities 4/5 motor strength was found. In the third case no bilateral gag reflex could be detected; a slight nasonal speech also accompanied. The deep tendon reflexes in all of the patients were brisked. There were no pathological reflexes. In the first two cases the presence of significant hypotenar, tenar atrophy and fasiculation attracted our attention. In electromyographic examination axonal degeneration findings together with spontaneous denervations attracted our attention. Conclusion: The fact that these three cases worked in the same place and the higher level of plasma cadmium especially in the first case after investigation metal intoxication brought to our mind that neurologic findings were associated with cadmium toxicity. Since there is quite a limited number of reports in literature showing findings of motor neuron involvement associated with cadmium metal.
P226 Single-fiber EMG in Meniere’s disease Fikret Bademkiran, Nese Celebisoy, Zafer Colakoglu, Ahmet Acarer Ege University Medical School Department of Neurology, Bornova, Izmir, Turkey Purpose: To assess neuromuscular transmission (NMT) in patients with Meniere’s disease (MD), in which the precise etiology is unknown and a channelopathy hypothesis has been proposed.
P227 Transferrin protects neuronal functionality in acute ischemic stroke Giovanni Assenza 1 , Filippo Zappasodi 2 , Rosanna Squitti 3 , Claudia Altamura 1 , Mariacarla Ventriglia 3 , Matilde Ercolani 3 , Domenico Lupoi 3 , Francesco Passarelli 3 , Paolo Maria Rossini 1 , Franca Tecchio 2 1 Dipartimento di Neuroscienze, Università Campus Bio-Medico, Rome, Italy; 2 ISTC-CNR, Rome, Italy; 3 Dipartimento di Neuroscienze, AFaR-Osp. Fatebenefratelli, Rome, Italy Purpose: Oxidative stress is involved in the spreading of ischemiareperfusion neuronal damage initiated by stroke. Our aim was to investigate the relationship of metals and oxidative stress molecules with neurological damage, as assessed by clinical examination and honed by Magnetoencephalography (MEG), in acute stroke. Methods: 18 patients (age 73±12) affected by mono-hemispheric ischemic stroke and 20 matched controls were enrolled. NIH Stroke Scale score (NIHSS) and Rolandic regions’ MEG activity were collected within the first week and blood samples within 48 hours from stroke. Level of copper, iron, zinc, ceruloplasmin, transferrin, total peroxides and Total Anti-Oxidant Trapping Capacity were correlated with clinical status and MEG activity. Results: The regression model designed to confirm MEG reliability in explaining clinical worsening, revealed that an increase of rest neuronal activity in the delta frequency range together with a decrease of primary sensory cortex excitability of the affected hemisphere predicted 67% of NIHSS variability. This reliability was confirmed by the regression model taking into account all MEG and biological contributions to NIHSS variability which revealed that only delta power entered the model predicting 45% of NIHSS. The regression models evaluating the biological variables influence on the neurological severity showed the decrease of transferrin as the only predictor of both the rest delta activity increase (42%) and the cortical excitability decrease (31%). Transferrin reduction was also the best predictor of NIHSS increase (36%) explaining together with peroxides 60% of its variability. Moreover, transferrin inversely associated with the lesion volume, especially in patients with cortico-subcortical stroke. Conclusions: Neurological damage was at best quantified by slow rest cortical activity and primary sensory cortex excitability of the affected hemisphere as assessed by MEG. Our results suggest that transferrin could play a protective role in preserving neuronal survival and functional efficiency, possibly trapping circulating metals limiting peroxides production.
Abstracts of the 13th European Congress of Clinical Neurophysiology / Clinical Neurophysiology 119 (2008), S1–S131
P224 Electromyographic pattern in Becker muscular dystrophy in comparison with Duchenne muscular dystrophy Barbara Emeryk-Szajewska, Jerzy Kopec Department of Neurology, Medical University of Warsaw Purpose: the aim of this study was to compare the electromyographic pattern in Becker muscular dystrophy (BMD) with that found in Duchenne muscular dystrophy (DMD). Material and method: fourteen men with BMD and 51 boys with DMD were investigated. Proximal muscles were examined: m. biceps brachii (BB) and m. rectus femoris (RF). They were divided according to the clinical criteria in two groups: of those with slight changes (group AB) and of those with severe abnormalities (CD). Our own method of Functional-QEMG was applied in the CNEMG examination. Results: spontaneous activity (fibrillations, complex repetitive discharges) was equally frequent in BMD and DMD. Linked potentials were rather frequent in either group. Myopathic features such as MUAPs low amplitude and area, polyphasic shape were seen in either condition, but more marked in DMD than in BMD (p< 0,05). Evaluation of IP recordings revealed that IP amplitude (amplitude size) is low in DMD already at the early stage of lesion but normal or only slightly diminished in BMD. Conclusion: our results might perhaps suggest different degrees of lesion in type II MUs between the compared types of muscular dystrophy.
Method: Single fiber electromyography (SFEMG) during voluntary contraction of the extansor digitorum communis muscle, nerve conduction studies of upper and lower extremities and concentric needle electromyography of the extansor digitorum communis (EDC) were performed on 20 patients with MD and 12 age-matched normal controls. Mean jitter value was calculated for each subject and it was compared with the values recorded from the normal controls. Results: Both controls and patients with MD had normal nerve conduction studies. Concentric needle EMG studies of the EDC muscle were also normal. Mean jitter values of the MD group did not differ from the normal control subjects (25,4±3,4 µs versus 27,2±2,6 µs, respectively; p=0.06) Conclusion: NMT abnormalities have been detected in migraineurs and cluster headache. It was thought that some migraineurs share similar genetic abnormalities with familial hemiplegic migraine (FHM) patients and have abnormalities of P/Q-type Ca+2 -channels which are present in motor nerve endings and in the brain. MD has been mapped to the DFNA9 locus on chromosome 14, the COCH gene; probably encoding an extracellular matrix protein. Inner hair cells have at least two and outer hair cells have at least three types of K+ -channels. In the cochlea the hair cell channel is KCNQ4. Mutations in two other genes encoding K+ -channel subunits, KCNQ1 and KCNE1, have been found in syndromic hereditary deafness. Clinical features such as sensitivity to dietary ions and reduction of the vertigo attacks with acetazolamide are on behalf of the channelopathy hypothesis. Though it doesn’t rule out the channelopathy hypothesis, a NMT deficit haven’t been found in patients with MD.
P225 Cases developing motor neuron symptoms after cadmium intoxication Refah Sayin, Temel Tombul, Aysel Milanlio˘glu Yuzuncu Yil Üniversity, Medical Faculty of Neurology Department Introduction and Purpose: Toxic and essential metals have been implicated in the pathogenesis of motor neuron disease (MND). Cadmium is a potential candidate for a neurotoxin in MND since it has been shown to be retrogradely transported in motor axons in motor neuron cell bodies. In this study, we present three cases manifesting motor neuron findings associated with cadmium intoxication. Method and Results: Patients with a history of working in such industrial sectors as blacksmith shops, accumulating and lead plaque coverage refered to our clinic with various complaints after 2, 3 and 6 months of the period of their employment. The complaints of these male patients with ages of 17, 18 and 27 were respectively physical pain, fatigue, weakness in limbs, nausea, anorexia, weight loss and involuntary tissue pulsations. In the neurological examination, the first case revealed 4/5 level of motor strength in the upper extremities, +3/5 in the left lower extremity and 4/5 right lower extremity. In the upper extremities of the second case, a level of +4/5 was determined markedly in the distal. In both of the lower extremities 4/5 motor strength was found. In the third case no bilateral gag reflex could be detected; a slight nasonal speech also accompanied. The deep tendon reflexes in all of the patients were brisked. There were no pathological reflexes. In the first two cases the presence of significant hypotenar, tenar atrophy and fasiculation attracted our attention. In electromyographic examination axonal degeneration findings together with spontaneous denervations attracted our attention. Conclusion: The fact that these three cases worked in the same place and the higher level of plasma cadmium especially in the first case after investigation metal intoxication brought to our mind that neurologic findings were associated with cadmium toxicity. Since there is quite a limited number of reports in literature showing findings of motor neuron involvement associated with cadmium metal.
P226 Single-fiber EMG in Meniere’s disease Fikret Bademkiran, Nese Celebisoy, Zafer Colakoglu, Ahmet Acarer Ege University Medical School Department of Neurology, Bornova, Izmir, Turkey Purpose: To assess neuromuscular transmission (NMT) in patients with Meniere’s disease (MD), in which the precise etiology is unknown and a channelopathy hypothesis has been proposed.
P227 Transferrin protects neuronal functionality in acute ischemic stroke Giovanni Assenza 1 , Filippo Zappasodi 2 , Rosanna Squitti 3 , Claudia Altamura 1 , Mariacarla Ventriglia 3 , Matilde Ercolani 3 , Domenico Lupoi 3 , Francesco Passarelli 3 , Paolo Maria Rossini 1 , Franca Tecchio 2 1 Dipartimento di Neuroscienze, Università Campus Bio-Medico, Rome, Italy; 2 ISTC-CNR, Rome, Italy; 3 Dipartimento di Neuroscienze, AFaR-Osp. Fatebenefratelli, Rome, Italy Purpose: Oxidative stress is involved in the spreading of ischemiareperfusion neuronal damage initiated by stroke. Our aim was to investigate the relationship of metals and oxidative stress molecules with neurological damage, as assessed by clinical examination and honed by Magnetoencephalography (MEG), in acute stroke. Methods: 18 patients (age 73±12) affected by mono-hemispheric ischemic stroke and 20 matched controls were enrolled. NIH Stroke Scale score (NIHSS) and Rolandic regions’ MEG activity were collected within the first week and blood samples within 48 hours from stroke. Level of copper, iron, zinc, ceruloplasmin, transferrin, total peroxides and Total Anti-Oxidant Trapping Capacity were correlated with clinical status and MEG activity. Results: The regression model designed to confirm MEG reliability in explaining clinical worsening, revealed that an increase of rest neuronal activity in the delta frequency range together with a decrease of primary sensory cortex excitability of the affected hemisphere predicted 67% of NIHSS variability. This reliability was confirmed by the regression model taking into account all MEG and biological contributions to NIHSS variability which revealed that only delta power entered the model predicting 45% of NIHSS. The regression models evaluating the biological variables influence on the neurological severity showed the decrease of transferrin as the only predictor of both the rest delta activity increase (42%) and the cortical excitability decrease (31%). Transferrin reduction was also the best predictor of NIHSS increase (36%) explaining together with peroxides 60% of its variability. Moreover, transferrin inversely associated with the lesion volume, especially in patients with cortico-subcortical stroke. Conclusions: Neurological damage was at best quantified by slow rest cortical activity and primary sensory cortex excitability of the affected hemisphere as assessed by MEG. Our results suggest that transferrin could play a protective role in preserving neuronal survival and functional efficiency, possibly trapping circulating metals limiting peroxides production.