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P1020: Acute idiopathic sensory neuronopathy with posterior reversible encephalopathy syndrome
S320
Abstracts of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339
(SSPE) using Magneto encephalography (MEG) and Electroencephalography (EEG). Methodology: Simultaneous MEG (306 channels) and EEG (64 channels) in five patients of SSPE (M:F = 3:2; age: 10.8±3.2 years; duration: 6.2±10 months) was carried out using Neuromag-Triux system. Qualitative analysis of 150 PCs/patient was performed. Fifty classical PCs of similar morphology and significant field topography were analysed, at the “onset” and “earliest significant peak” of the burst by discrete and distributed source imaging methods for MEG & EEG independently. Results: MEG background was asymmetrical in 2 and slow in 3patients. PCs were periodic (3) or quasi-periodic (2), occurring every 4-16 seconds and monomorphic in a patient but varied in morphology across patients. Major source localization of the onset of bursts by Magnetic Source Imaging (MSI) was in thalami (43%) & peri-central sulci (33%) while by Electric Source Imaging (ESI) it was in orbito/inferior frontal (35%) & peri-central (34%) regions. Major source localization of the earliest significant burst peak by MSI was in parietal (40%) and anterior/inferior frontal (42%) regions while by ESI it was in orbito/inferior frontal (45%), pre-central (28%) regions. With ESI 6-20% of activities were smeared/non-focal. Further analysis revealed that PCs were generated in thalami and propagated to posterior cingulate and to motor-sensory cortex in 3 patients. Conclusions: This is first ever study of simultaneous MEG-EEG recording of periodic complexes. It has provided insight into the understanding of subcortical myoclonus and generator and propagation of PCs in SSPE.
active HIT compared to passive non-predictive HIT. In patients predictive passive head movements elicited a VOR gain increase of 24% but this increase was significantly larger with active HIT (148%). Increase of VOR gain during predictive (4%) and active HIT (8%) was much smaller in healthy participants.
Figure 1
P1016 Electrophysiological study of distal axonopathic, proximal demylinating polyneuropathy in a case of Addison’s disease only recieved steroid therapy for 30 years M. Khalil, I. Khalil University of Alexandria, Physical Medicine, Rheumatology and Rehabilitation, Alexandria, Egypt Question: What is the underlying cause of weakness of Leg and forearm muscles in a case of 36 years old male patient diagnosed as Addison’s disease only received steroid therapy since aged 6 years. Methods: Clinical and electrophysiological study involving sensory and motor conduction in lower and upper limbs, F waves, H reflex, Needle EMG, SEP, ABR and PR-VEP where performed three times during the last 10 years. Results: The patient is presented with bilateral pescavus, weak dorsiflexos, wasting of leg muscles feet parathesia, which progressed slowly during the last 20 years. Two month ago he experienced progressive weakness of the wrist and finger extensorsr on both sides. MRI study of the brain and spinal cord was unremarkable. Nerve conduction study and needle EMG revealed findings consistent with distal axonopathic peripheral neuropathy involving distal segments of the sensory and motor fibers of the common peroneal, posterior tibial and radial nerves but demylinating neuropathy involving both Femoral and axillary nerves with significant delay of the F waves and H reflex. The study revealed normal SEP, ABR and PR-VEP. Conclusion: Follow up electrophysiological studies during the last 10 years revealed findings of mixed distal axonopathic neuropathy associated with proximal demylinating neuropathy with no evidence of Myelopathy or central Lesion.
P1018 Predictive mechanisms improve the vestibulo-ocular reflex in bilateral vestibular failure patients J.F. Wojak, N.-M. Jandl, S. Hertel, C. Helmchen, A. Sprenger Universitaet zu Luebeck, Klinik fuer Neurologie, Luebeck, Germany Patients with unilateral (UVF) or bilateral vestibular failure (BVF) suffer from blurred vision during head movements as an impaired vestibuloocular reflex (VOR) cannot sufficiently stabilize visual images on the fovea. In contrast to UVF, BVF patients often do not recover. The aim of this study was to test whether extravestibular mechanisms can help to improve the VOR. Eye and head movements were recorded during predictive and nonpredictive passive and active head movements (head impulse test, HIT) in patients with bilateral vestibular failure (N=31) and age-matched healthy participants (N=31). In both groups VOR gain was higher in predictive passive HIT as well as in
Figure 2
Improved eye stabilisation during active HIT is likely to be caused by non-vestibular predictive mechanisms using the efference copy of the neck and head motor command and of the proprioceptive feedback signal. This prediction probably includes the anticipation of the own gaze error in BVF providing a strong rationale for vestibular rehabilitation. The intact contralesional VOR of UVF patients with increased VOR gain during active HIT is unlikely to account for the improvement in BVF ipsilesional VOR gain, i.e. in UVF active HIT benefit from extravestibular information and not from central compensation of remaining vestibular function.
P1020 Acute idiopathic sensory neuronopathy with posterior reversible encephalopathy syndrome K. Lwin, S.Z. Tun Calderdale & Huddersfield NHS Foundation Trust, Clinical Neuophysiology, Halifax, United Kingdom A previously healthy, 20 yr-old mother who delivered a baby six weeks earlier had progressive tingling and numbness from feet to hands in a week. Her balance was off. She was unwell and lethargic but afebrile. Her BP was 143/94 mmHg. Unsteady gait, heel to shin ataxia and generalized hyporeflexia were seen. She felt dizzy and collapsed in next 24 hours. There was a brief period of unresponsiveness. Her BP was 71/41 mmHg. Investigations were made for a possible GBS. CSF studies were normal. EMG/NCS were obtained 10 days after symptoms onset. Sural, peroneal, tibial medial plantar sensory NCS are normal while median, ulnar, and radial SNAPs are low in amplitude. Motor nerve conduction studies are normal. A sensory neuronopathy diagnosis was made. Various immunological tests were done and none were revealing. On 6th day of admission patient had multiple generalized tonic clonic seizures with loss of consciousness. EEG revealed encephalopathy with occipital area attenuation. MRI brain scan is consistent with posterior reversible encephalopathy syndrome (PRES). NCS were repeated nine days, 7 weeks and a year later. She was treated with IV IgG and she went home two weeks later. This is a case of acute sensory neuronopathy of idiopathic nature with
Abstracts of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339
S321
Abstract P1020 – Figure 1
P1022 Increased cortical excitability in patients with stiff person syndrome assessed by paired-pulse transcranial magnetic stimulation. A case study of two patients J. Wilenius, S. Vaalto, J. Toppila, J. Partanen Hospital District of Helsinki and Uusimaa, Department of Clinical Neurophysiology, Helsinki, Finland
Figure 2
PRES in a six weeks post-partum patient. This is a first case report of such combination. Follow up NCS revealed losses of SNAPs appeared in arms followed by legs. Peroneal nerves are affected before sural nerves. Despite the clinical improvement, NCS failed to reveal the parallel changes.
P1021 Thoracic outlet syndrome – the proposition of new diagnostic procedures J. Pigonska, A. Bogucki Medicla University, Lodz, Department of Extrapyramidal Diseases, Lodz, Poland Question: Thoracic outlet syndrome (TOS) is a rare disorder affecting the lower trunk of the brachial plexus and subclavian vessels between the neck and axilla. Several types of TOS are distinguished: vascular (venous or arterial) and neurogenic (true or non-specific). The diagnosis of TOS is based on the clinical examination (including vascular tests) as well as on results of electroneurophysiological tests, angiography, classical radiology, MRI and USG-D. Methods and results: We present a history of 30-year-old-woman with confirmed arterial TOS who was complaining of the pain and the weakness in the right upper extremity. On clinical examination we found only reduced tendon reflexes of the right upper limb and positive vascular tests. The electroneurography (ENeG) of the brachial plexus showed only the reduced conduction volume between the Erb point and the armpit during the ulnar conduction test. The electromyography (EMG) of the upper limb muscles showed no denervation and reinervation. The F wave in normal position of the right hand and in during Addson, Allen test showed no difference. The laser evoked potentials evaluated the proper functions of small fibres’ sensory pathways. The contraleral test were also conducted. Conclusion: According to our clinical and neurophysiologiacal examination we did not find any evidence concerning the neurogenic lesion of the brachial plexus.
Question: Stiff person syndrome (SPS) is a rare neurological condition characterized by stiffness and spasms of the axial and limb muscles. Cortical and spinal hyperexcitabilities are thought to be important pathophysiological mechanisms. We describe intracortical inhibitory and excitatory balance in two patients with the SPS diagnosis. Methods: The function of intracortical inhibitory and excitatory interneurons was studied by using paired-pulse transcranial magnetic stimulation (ppTMS). Motor threshold (MT) was determined and thereafter baseline motor evoked potentials (MEPs) were recorded from the abductor pollicis brevis muscle using single-pulses and 120% MT intensity. Short-interval intracortical inhibition (SICI) was assessed using ppTMS with interstimulus interval (ISI) of 3 ms (pulse intensities 80% and 120% MT). Intracortical facilitation (ICF) was studied using 13 ms ISI (90% and 120% MT pulse intensities). Silent period -recordings were also conducted using single pulses (120% MT pulse intensity). Results: Patient 1 had pronounced ICF (MEP increase 400% when compared to baseline MEP) and decreased cortical inhibition (ppMEP amplitude 92% of the baseline MEP). The silent period was found to be abnormally long (158 ms). MT was normal. Patient 2 demonstrated pronounced ICF (ppMEP amplitude three times higher than baseline MEP) and absent SICI (ppMEP amplitude same as baseline MEP amplitude). The silent period and MT were normal. Conclusions: SPS patients showed signs of decreased SICI and increased ICF in ppTMS-study. TMS is a promising tool in diagnosing stiff person syndrome.
P1023 Clinical and electrophysiologic findings in Schwartz-Jampel syndrome L. Baysal Kirac 1 , E. Kocasoy Orhan 1 , M.B. Baslo 1 , U. Altunoglu 2 , H. Kayserili 2 , A.E. Oge 1 , J. Yazıcı 1 , S. Nicole 3 1 Istanbul University, Istanbul Medical Faculty, Neurology, Istanbul, Turkey; 2 Istanbul University, Istanbul Medical Faculty, Institute of Child Health, Istanbul, Turkey; 3 Centre de Recherche de l’Institut du Cerveau et de la Moelle Épinière, Paris, France Question: Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disorder characterized by generalized myotonic myopathy, joint contractures, skeletal anomalies, and short stature, caused by mutations in the HSPG2 gene encoding perlecan. The aim of this study is to present the electrophysiologic manifestations and underline the clinical heterogeneity of patients with SJS. Method: We presented clinical features, immunohistochemical, molecular and electromyographic (EMG) findings of six patients with SJS.
Abstracts of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339
(SSPE) using Magneto encephalography (MEG) and Electroencephalography (EEG). Methodology: Simultaneous MEG (306 channels) and EEG (64 channels) in five patients of SSPE (M:F = 3:2; age: 10.8±3.2 years; duration: 6.2±10 months) was carried out using Neuromag-Triux system. Qualitative analysis of 150 PCs/patient was performed. Fifty classical PCs of similar morphology and significant field topography were analysed, at the “onset” and “earliest significant peak” of the burst by discrete and distributed source imaging methods for MEG & EEG independently. Results: MEG background was asymmetrical in 2 and slow in 3patients. PCs were periodic (3) or quasi-periodic (2), occurring every 4-16 seconds and monomorphic in a patient but varied in morphology across patients. Major source localization of the onset of bursts by Magnetic Source Imaging (MSI) was in thalami (43%) & peri-central sulci (33%) while by Electric Source Imaging (ESI) it was in orbito/inferior frontal (35%) & peri-central (34%) regions. Major source localization of the earliest significant burst peak by MSI was in parietal (40%) and anterior/inferior frontal (42%) regions while by ESI it was in orbito/inferior frontal (45%), pre-central (28%) regions. With ESI 6-20% of activities were smeared/non-focal. Further analysis revealed that PCs were generated in thalami and propagated to posterior cingulate and to motor-sensory cortex in 3 patients. Conclusions: This is first ever study of simultaneous MEG-EEG recording of periodic complexes. It has provided insight into the understanding of subcortical myoclonus and generator and propagation of PCs in SSPE.
active HIT compared to passive non-predictive HIT. In patients predictive passive head movements elicited a VOR gain increase of 24% but this increase was significantly larger with active HIT (148%). Increase of VOR gain during predictive (4%) and active HIT (8%) was much smaller in healthy participants.
Figure 1
P1016 Electrophysiological study of distal axonopathic, proximal demylinating polyneuropathy in a case of Addison’s disease only recieved steroid therapy for 30 years M. Khalil, I. Khalil University of Alexandria, Physical Medicine, Rheumatology and Rehabilitation, Alexandria, Egypt Question: What is the underlying cause of weakness of Leg and forearm muscles in a case of 36 years old male patient diagnosed as Addison’s disease only received steroid therapy since aged 6 years. Methods: Clinical and electrophysiological study involving sensory and motor conduction in lower and upper limbs, F waves, H reflex, Needle EMG, SEP, ABR and PR-VEP where performed three times during the last 10 years. Results: The patient is presented with bilateral pescavus, weak dorsiflexos, wasting of leg muscles feet parathesia, which progressed slowly during the last 20 years. Two month ago he experienced progressive weakness of the wrist and finger extensorsr on both sides. MRI study of the brain and spinal cord was unremarkable. Nerve conduction study and needle EMG revealed findings consistent with distal axonopathic peripheral neuropathy involving distal segments of the sensory and motor fibers of the common peroneal, posterior tibial and radial nerves but demylinating neuropathy involving both Femoral and axillary nerves with significant delay of the F waves and H reflex. The study revealed normal SEP, ABR and PR-VEP. Conclusion: Follow up electrophysiological studies during the last 10 years revealed findings of mixed distal axonopathic neuropathy associated with proximal demylinating neuropathy with no evidence of Myelopathy or central Lesion.
P1018 Predictive mechanisms improve the vestibulo-ocular reflex in bilateral vestibular failure patients J.F. Wojak, N.-M. Jandl, S. Hertel, C. Helmchen, A. Sprenger Universitaet zu Luebeck, Klinik fuer Neurologie, Luebeck, Germany Patients with unilateral (UVF) or bilateral vestibular failure (BVF) suffer from blurred vision during head movements as an impaired vestibuloocular reflex (VOR) cannot sufficiently stabilize visual images on the fovea. In contrast to UVF, BVF patients often do not recover. The aim of this study was to test whether extravestibular mechanisms can help to improve the VOR. Eye and head movements were recorded during predictive and nonpredictive passive and active head movements (head impulse test, HIT) in patients with bilateral vestibular failure (N=31) and age-matched healthy participants (N=31). In both groups VOR gain was higher in predictive passive HIT as well as in
Figure 2
Improved eye stabilisation during active HIT is likely to be caused by non-vestibular predictive mechanisms using the efference copy of the neck and head motor command and of the proprioceptive feedback signal. This prediction probably includes the anticipation of the own gaze error in BVF providing a strong rationale for vestibular rehabilitation. The intact contralesional VOR of UVF patients with increased VOR gain during active HIT is unlikely to account for the improvement in BVF ipsilesional VOR gain, i.e. in UVF active HIT benefit from extravestibular information and not from central compensation of remaining vestibular function.
P1020 Acute idiopathic sensory neuronopathy with posterior reversible encephalopathy syndrome K. Lwin, S.Z. Tun Calderdale & Huddersfield NHS Foundation Trust, Clinical Neuophysiology, Halifax, United Kingdom A previously healthy, 20 yr-old mother who delivered a baby six weeks earlier had progressive tingling and numbness from feet to hands in a week. Her balance was off. She was unwell and lethargic but afebrile. Her BP was 143/94 mmHg. Unsteady gait, heel to shin ataxia and generalized hyporeflexia were seen. She felt dizzy and collapsed in next 24 hours. There was a brief period of unresponsiveness. Her BP was 71/41 mmHg. Investigations were made for a possible GBS. CSF studies were normal. EMG/NCS were obtained 10 days after symptoms onset. Sural, peroneal, tibial medial plantar sensory NCS are normal while median, ulnar, and radial SNAPs are low in amplitude. Motor nerve conduction studies are normal. A sensory neuronopathy diagnosis was made. Various immunological tests were done and none were revealing. On 6th day of admission patient had multiple generalized tonic clonic seizures with loss of consciousness. EEG revealed encephalopathy with occipital area attenuation. MRI brain scan is consistent with posterior reversible encephalopathy syndrome (PRES). NCS were repeated nine days, 7 weeks and a year later. She was treated with IV IgG and she went home two weeks later. This is a case of acute sensory neuronopathy of idiopathic nature with
Abstracts of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339
S321
Abstract P1020 – Figure 1
P1022 Increased cortical excitability in patients with stiff person syndrome assessed by paired-pulse transcranial magnetic stimulation. A case study of two patients J. Wilenius, S. Vaalto, J. Toppila, J. Partanen Hospital District of Helsinki and Uusimaa, Department of Clinical Neurophysiology, Helsinki, Finland
Figure 2
PRES in a six weeks post-partum patient. This is a first case report of such combination. Follow up NCS revealed losses of SNAPs appeared in arms followed by legs. Peroneal nerves are affected before sural nerves. Despite the clinical improvement, NCS failed to reveal the parallel changes.
P1021 Thoracic outlet syndrome – the proposition of new diagnostic procedures J. Pigonska, A. Bogucki Medicla University, Lodz, Department of Extrapyramidal Diseases, Lodz, Poland Question: Thoracic outlet syndrome (TOS) is a rare disorder affecting the lower trunk of the brachial plexus and subclavian vessels between the neck and axilla. Several types of TOS are distinguished: vascular (venous or arterial) and neurogenic (true or non-specific). The diagnosis of TOS is based on the clinical examination (including vascular tests) as well as on results of electroneurophysiological tests, angiography, classical radiology, MRI and USG-D. Methods and results: We present a history of 30-year-old-woman with confirmed arterial TOS who was complaining of the pain and the weakness in the right upper extremity. On clinical examination we found only reduced tendon reflexes of the right upper limb and positive vascular tests. The electroneurography (ENeG) of the brachial plexus showed only the reduced conduction volume between the Erb point and the armpit during the ulnar conduction test. The electromyography (EMG) of the upper limb muscles showed no denervation and reinervation. The F wave in normal position of the right hand and in during Addson, Allen test showed no difference. The laser evoked potentials evaluated the proper functions of small fibres’ sensory pathways. The contraleral test were also conducted. Conclusion: According to our clinical and neurophysiologiacal examination we did not find any evidence concerning the neurogenic lesion of the brachial plexus.
Question: Stiff person syndrome (SPS) is a rare neurological condition characterized by stiffness and spasms of the axial and limb muscles. Cortical and spinal hyperexcitabilities are thought to be important pathophysiological mechanisms. We describe intracortical inhibitory and excitatory balance in two patients with the SPS diagnosis. Methods: The function of intracortical inhibitory and excitatory interneurons was studied by using paired-pulse transcranial magnetic stimulation (ppTMS). Motor threshold (MT) was determined and thereafter baseline motor evoked potentials (MEPs) were recorded from the abductor pollicis brevis muscle using single-pulses and 120% MT intensity. Short-interval intracortical inhibition (SICI) was assessed using ppTMS with interstimulus interval (ISI) of 3 ms (pulse intensities 80% and 120% MT). Intracortical facilitation (ICF) was studied using 13 ms ISI (90% and 120% MT pulse intensities). Silent period -recordings were also conducted using single pulses (120% MT pulse intensity). Results: Patient 1 had pronounced ICF (MEP increase 400% when compared to baseline MEP) and decreased cortical inhibition (ppMEP amplitude 92% of the baseline MEP). The silent period was found to be abnormally long (158 ms). MT was normal. Patient 2 demonstrated pronounced ICF (ppMEP amplitude three times higher than baseline MEP) and absent SICI (ppMEP amplitude same as baseline MEP amplitude). The silent period and MT were normal. Conclusions: SPS patients showed signs of decreased SICI and increased ICF in ppTMS-study. TMS is a promising tool in diagnosing stiff person syndrome.
P1023 Clinical and electrophysiologic findings in Schwartz-Jampel syndrome L. Baysal Kirac 1 , E. Kocasoy Orhan 1 , M.B. Baslo 1 , U. Altunoglu 2 , H. Kayserili 2 , A.E. Oge 1 , J. Yazıcı 1 , S. Nicole 3 1 Istanbul University, Istanbul Medical Faculty, Neurology, Istanbul, Turkey; 2 Istanbul University, Istanbul Medical Faculty, Institute of Child Health, Istanbul, Turkey; 3 Centre de Recherche de l’Institut du Cerveau et de la Moelle Épinière, Paris, France Question: Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disorder characterized by generalized myotonic myopathy, joint contractures, skeletal anomalies, and short stature, caused by mutations in the HSPG2 gene encoding perlecan. The aim of this study is to present the electrophysiologic manifestations and underline the clinical heterogeneity of patients with SJS. Method: We presented clinical features, immunohistochemical, molecular and electromyographic (EMG) findings of six patients with SJS.