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P164 Neurophysiologic evaluation in monomelic amyotrophy
Poster Session: Electromyography and Neurography
314
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EVALUATIONOF NERVE MOTORAND SENSORY CONDUCTIONVELOCmES IN EPILEPTICPATIENTS TREATEDWITH CARBAMAZEPINE(CBZ) IN MONO-AND POLYTHERAPY
R. Slusarczyk, E. Motta. Dept. of Neurology Silesian Medical Acamedy.
Zabrze, Poland Phenytoin is commonly believed to be the cause of peripheral neuropathy. In the present study median, ulnar, peroneal and tibial nerve motor conduction velocities (MCVs) and median, ulnar and sural nerve sensory conduction velocities (SCVs) were performed in 26 epileptic patients (mean age 32.0 + 7.4 years) treated only with CBZ (mean treatment duration 10.9 + 8,7 years) and in 16 patients (mean age 32.6 + 6:9 years) receiving CBZ in polytherapy (mean treatment duration 14.8 + 9.9 years). The control group consisted of 16 healthy volunteers in the same range of age. The average MCV values in both groups were similar and did not differ from control group. The average SCV values were significantly slower in both groups than those of normal subjects. There was no relationship between conduction velocities and dally dosage of CBZ, serum drug levels and treatment duration in both groups. None of the patients had clinical signs of polyneuropathy. It may be concluded that CBZ applied in monotherapy as well as in polytherapy may caused subclinical sensory peripheral neuropathy.
~6-~
INTERMEDIATEFORM OF SPINAL MUSCULARATROPHY
B. Ishpekova, M. Rasheva, S. Novachkova, E. Vassileva, P. Stamenova.
University Hospital "Tzaritza Joanna ", Sofia The purpose of the study is to establish the diagnostic and prognostic impact of EMG in II type of spinal muscular atrophy, as well as the degree of clinical and electrophysiological variability. Out of 67 patients suffering from spinal muscular atrophy 21 cases with SMA II type have been established, never being able to stand or walk and an onset of the symptoms before 18 months of age. The follow-up period ranges from 1 to 20 years (mean 10.8 yrs). The gravity of the neurological symptoms depends on the duration of the disease. The onset of SMA II type is important as an earlier onset corresponds to more severe symptoms. EMG has been performed on 18 of these patients. The highest percentage of fibrillation and positive sharp waves was observed in the proximal muscles of the lower limbs. "Bizzare" discharges were found mostly in patients with a greater duration of the disease. We established slowed conduction velocity along the motor fibres, M-response was absent in 5 patients, mostly in those with a longer duration of the disease. Sensory conduction was within the normal limits. It can be concluded that EMG is extremely valuable in determining the diagnosis and prognosis of SMA 11 type cases, as well as establishing the degree of variability.
~ P ' ~ - ~ TRAUMATICOUADRIPLEGIAWITH PHRENICNERVE INJURY ~P-~
NEUROPHYSIOLOGICEVALUATIONIN MONOMELIC AMYOTROPHY
M. Gratac6s, N. Raguer, N. Gonzalez, F. Crespo, C. Cervera, J. Gamez.
Hospital de la Vail d'Hebrdn, Barcelona, Spain Monomelic amyotrophy (MA) is a rare form of motorneuron disease, usually presenting as painless asymmetric weakness and atrophy of the distal muscles at the upper extremities, without sensory deficiencies. MRI of the cervical spine show in some of this patients segmental cervical cord atrophy, localized at the same root levels of the muscle paresis. This study has been undertaken to evaluate the neurophysiological performance of 4 patients with MA, two of them with typical MRI lesions. Results: a.-Normal sensory nerves action potentials, b.-Redueed amplitudes of the compound muscle potentials, with normal or slightly decreased velocities, and increased distal latencies in the most denervated muscles in three patients, c.-Features of acute and chronic denervation in distal upper extremities muscles of C7-DI root levels, without leg involvement, d.Reduced incidence, and increased latency of F-waves, in the most afected nerves, e.-Two patients showed central motor conduction time in the high range of normality at the upper extremities (abductor digiti minimi) elicited by magnetic stimulation and normal in the anterior tibialis. Cervical magnetic stimulation demonstrated enlarged latencies in the most affected muscles, f.-Normal somatosensory evoked potentials stimulating ulnar and posterior tibialis nerves. All these neurophysiological values demonstrate the almost exclusively damage at the second motorneuron in MA, and rules out central motor and sensory involvement.
ELECTROMYOGRAPHICCHARACTERISTICSOF KLIPPEL-TRENAUNAY-WEBERSYNDROME B. Jocid, D. Goci&Stankovid, V. Disid, J. Vukidevid. Clinic of Mental
Health and Child Neuropsychiatry Medical Faculty of NiL Yugoslavia We will describe a 10 years old boy with this syndrome. When he was 3 years old the diagnosis of aortic disease and arterial hypertension had established. He has hyperpigmented nevus on the neck which continue on the chin and lip. The similar findings there are on the left side of thorax and on the left hand also. He has also kyphoscoliosis, scapulae alatae and hypotrophyo of proximal muscles on the both upper and down limbs. Both visual and auditory evoked potentials were normal. Somatosensory EP revealed mild conduction disturbances. CT scan discerned a normal findings. EMG examination showed signs of insufficient of lower motor innervation with high compensatory action potential of motor units. The authors suggest a possibility of association Klippel-TrenaunayWeber syndrome with disease of motor neuron.
Kjeld Andersen, A. S~eterhaug, T. Skandsen, A. Stolt-Nielsen. University
Hospital, Trondheira, Norway Bilateral peripheral nerve injury of the phrenic nerves is seldom. A young man with a C4 fracture and tetraplegia, dependent on respirator was evaluated for diaphragm pacing. EMG and nerve conduction from 5 to 21 months after the accident showed a reinnervation pattern compatible with a nearly total loss of axons in the phrenic nerve. Diaphragm pacing with a four-pole sequential phrenic nerve stimulation (Atrostim, Tampere, Finland) was established after 11 months. This method combines low frequency stimulation with a four times higher fusion frequency and a smooth contraction. On the left side pacing was tolerated after 2 years for some hours daily, on the right side reinnervation was too insufficient to provide ventilation. In cases of high spinal cord lesion complicated by unexplained need of respiratory assistance neurophysiological evaluation may be necessary to establish the function of phrenic nerves and the right time for a possible implantation of a functional electrical stimulator.
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ELECTROPHYSIOLOGICFEATURESOF CONTINUOUS MUSCLE FIBER ACTIVITY(NEUROMYOTONIA)
S. Pavlovic, R. Trikic. Institute for Neurology, Clinical Centre of Serbia,
Belgrade, Yugoslavia The syndrome of continuous muscle fiber activity is associated with a variety of clinical manifestations and electromyographic discharges. We present three patients with muscle stiffness, cramps and exercise intolerance. Neurologic examinations showed generalized fasciculations and myokimia. Painful cramps were provoked by voluntary muscle contractions. Mild distal atrophy and diminished or absent tendon reflexes were present in two patients and distal sensory loss was found in one. Electromyographic examination showed fasciculations and myokimic discharges at rest in all three patients. In one patient, continuous motor unit activity was present in the calf muscles at rest. The number of highfrequency discharges increased greatly following voluntary contraction of the gastrocnemius muscle. Peroneal nerve block with 1% lidocalne at the fibula head did not affect spontaneous activity in the appropriate muscles. In another patient, continuous muscle fiber activity was registered only after voluntary contractions. Distal tibial nerve block with 2% lidocalne significantly reduced the amplitudes and frequency of neuromyotonic discharges, but did not abolish them. These findings demonstrate hyperexcitability of the terminal motor axons and the heterogenity of electrophysiologic features of the syndrome of continuous muscle fiber activity.
314
~
EVALUATIONOF NERVE MOTORAND SENSORY CONDUCTIONVELOCmES IN EPILEPTICPATIENTS TREATEDWITH CARBAMAZEPINE(CBZ) IN MONO-AND POLYTHERAPY
R. Slusarczyk, E. Motta. Dept. of Neurology Silesian Medical Acamedy.
Zabrze, Poland Phenytoin is commonly believed to be the cause of peripheral neuropathy. In the present study median, ulnar, peroneal and tibial nerve motor conduction velocities (MCVs) and median, ulnar and sural nerve sensory conduction velocities (SCVs) were performed in 26 epileptic patients (mean age 32.0 + 7.4 years) treated only with CBZ (mean treatment duration 10.9 + 8,7 years) and in 16 patients (mean age 32.6 + 6:9 years) receiving CBZ in polytherapy (mean treatment duration 14.8 + 9.9 years). The control group consisted of 16 healthy volunteers in the same range of age. The average MCV values in both groups were similar and did not differ from control group. The average SCV values were significantly slower in both groups than those of normal subjects. There was no relationship between conduction velocities and dally dosage of CBZ, serum drug levels and treatment duration in both groups. None of the patients had clinical signs of polyneuropathy. It may be concluded that CBZ applied in monotherapy as well as in polytherapy may caused subclinical sensory peripheral neuropathy.
~6-~
INTERMEDIATEFORM OF SPINAL MUSCULARATROPHY
B. Ishpekova, M. Rasheva, S. Novachkova, E. Vassileva, P. Stamenova.
University Hospital "Tzaritza Joanna ", Sofia The purpose of the study is to establish the diagnostic and prognostic impact of EMG in II type of spinal muscular atrophy, as well as the degree of clinical and electrophysiological variability. Out of 67 patients suffering from spinal muscular atrophy 21 cases with SMA II type have been established, never being able to stand or walk and an onset of the symptoms before 18 months of age. The follow-up period ranges from 1 to 20 years (mean 10.8 yrs). The gravity of the neurological symptoms depends on the duration of the disease. The onset of SMA II type is important as an earlier onset corresponds to more severe symptoms. EMG has been performed on 18 of these patients. The highest percentage of fibrillation and positive sharp waves was observed in the proximal muscles of the lower limbs. "Bizzare" discharges were found mostly in patients with a greater duration of the disease. We established slowed conduction velocity along the motor fibres, M-response was absent in 5 patients, mostly in those with a longer duration of the disease. Sensory conduction was within the normal limits. It can be concluded that EMG is extremely valuable in determining the diagnosis and prognosis of SMA 11 type cases, as well as establishing the degree of variability.
~ P ' ~ - ~ TRAUMATICOUADRIPLEGIAWITH PHRENICNERVE INJURY ~P-~
NEUROPHYSIOLOGICEVALUATIONIN MONOMELIC AMYOTROPHY
M. Gratac6s, N. Raguer, N. Gonzalez, F. Crespo, C. Cervera, J. Gamez.
Hospital de la Vail d'Hebrdn, Barcelona, Spain Monomelic amyotrophy (MA) is a rare form of motorneuron disease, usually presenting as painless asymmetric weakness and atrophy of the distal muscles at the upper extremities, without sensory deficiencies. MRI of the cervical spine show in some of this patients segmental cervical cord atrophy, localized at the same root levels of the muscle paresis. This study has been undertaken to evaluate the neurophysiological performance of 4 patients with MA, two of them with typical MRI lesions. Results: a.-Normal sensory nerves action potentials, b.-Redueed amplitudes of the compound muscle potentials, with normal or slightly decreased velocities, and increased distal latencies in the most denervated muscles in three patients, c.-Features of acute and chronic denervation in distal upper extremities muscles of C7-DI root levels, without leg involvement, d.Reduced incidence, and increased latency of F-waves, in the most afected nerves, e.-Two patients showed central motor conduction time in the high range of normality at the upper extremities (abductor digiti minimi) elicited by magnetic stimulation and normal in the anterior tibialis. Cervical magnetic stimulation demonstrated enlarged latencies in the most affected muscles, f.-Normal somatosensory evoked potentials stimulating ulnar and posterior tibialis nerves. All these neurophysiological values demonstrate the almost exclusively damage at the second motorneuron in MA, and rules out central motor and sensory involvement.
ELECTROMYOGRAPHICCHARACTERISTICSOF KLIPPEL-TRENAUNAY-WEBERSYNDROME B. Jocid, D. Goci&Stankovid, V. Disid, J. Vukidevid. Clinic of Mental
Health and Child Neuropsychiatry Medical Faculty of NiL Yugoslavia We will describe a 10 years old boy with this syndrome. When he was 3 years old the diagnosis of aortic disease and arterial hypertension had established. He has hyperpigmented nevus on the neck which continue on the chin and lip. The similar findings there are on the left side of thorax and on the left hand also. He has also kyphoscoliosis, scapulae alatae and hypotrophyo of proximal muscles on the both upper and down limbs. Both visual and auditory evoked potentials were normal. Somatosensory EP revealed mild conduction disturbances. CT scan discerned a normal findings. EMG examination showed signs of insufficient of lower motor innervation with high compensatory action potential of motor units. The authors suggest a possibility of association Klippel-TrenaunayWeber syndrome with disease of motor neuron.
Kjeld Andersen, A. S~eterhaug, T. Skandsen, A. Stolt-Nielsen. University
Hospital, Trondheira, Norway Bilateral peripheral nerve injury of the phrenic nerves is seldom. A young man with a C4 fracture and tetraplegia, dependent on respirator was evaluated for diaphragm pacing. EMG and nerve conduction from 5 to 21 months after the accident showed a reinnervation pattern compatible with a nearly total loss of axons in the phrenic nerve. Diaphragm pacing with a four-pole sequential phrenic nerve stimulation (Atrostim, Tampere, Finland) was established after 11 months. This method combines low frequency stimulation with a four times higher fusion frequency and a smooth contraction. On the left side pacing was tolerated after 2 years for some hours daily, on the right side reinnervation was too insufficient to provide ventilation. In cases of high spinal cord lesion complicated by unexplained need of respiratory assistance neurophysiological evaluation may be necessary to establish the function of phrenic nerves and the right time for a possible implantation of a functional electrical stimulator.
~
ELECTROPHYSIOLOGICFEATURESOF CONTINUOUS MUSCLE FIBER ACTIVITY(NEUROMYOTONIA)
S. Pavlovic, R. Trikic. Institute for Neurology, Clinical Centre of Serbia,
Belgrade, Yugoslavia The syndrome of continuous muscle fiber activity is associated with a variety of clinical manifestations and electromyographic discharges. We present three patients with muscle stiffness, cramps and exercise intolerance. Neurologic examinations showed generalized fasciculations and myokimia. Painful cramps were provoked by voluntary muscle contractions. Mild distal atrophy and diminished or absent tendon reflexes were present in two patients and distal sensory loss was found in one. Electromyographic examination showed fasciculations and myokimic discharges at rest in all three patients. In one patient, continuous motor unit activity was present in the calf muscles at rest. The number of highfrequency discharges increased greatly following voluntary contraction of the gastrocnemius muscle. Peroneal nerve block with 1% lidocalne at the fibula head did not affect spontaneous activity in the appropriate muscles. In another patient, continuous muscle fiber activity was registered only after voluntary contractions. Distal tibial nerve block with 2% lidocalne significantly reduced the amplitudes and frequency of neuromyotonic discharges, but did not abolish them. These findings demonstrate hyperexcitability of the terminal motor axons and the heterogenity of electrophysiologic features of the syndrome of continuous muscle fiber activity.