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P194. Nerve sonography guided diagnosis of painful peroneal palsies caused by intraneural ganglia – A case series
Society Proceedings / Clinical Neurophysiology 126 (2015) e63–e170
partial facial hypohidrosis or no hypohidrosis at all. We suspect a damage of the second order preganglionic sympathetic neuron, because gustatory sweating is most frequently described in preganglionic lesions. Despite the normal MRI scan, an association with the cervical surgery is probable. This case underlines the usefulness of ‘‘old’’ examination procedures like the starch-iodine sweat test in the topodiagnosis of focal dysautonomias like Horner‘s syndrome. doi:10.1016/j.clinph.2015.04.286
P192. Small hairpin RNA against PMP22 is effective in vitro but fails to improve the phenotype of a CMT1A rat model in vivo—B. Gess, K. Friedrich, D. Röhr, H. Halfter, P. Young (Universitätsklinikum Münster, Klinik für Schlafmedizin und neuromuskuläre Erkrankungen, Münster, Germany) CMT1A is the most common form of hereditary neuropathies, accounting for about 30–40% of all patients. A causative treatment is not available as of yet. CMT1A is based on a duplication of the gene PMP22. Thus, lowering PMP22 expression represents a promising avenue for treatment studies. In this pilot study, we used plasmid-vector encoded shRNA against PMP22 for posttranscriptional silencing of PMP22 in vitro and in vivo.
e169
Cell lines 3T3, HEK 293, and MSC80 (Schwann cell-derived) were used to test the effects of PMP22-shRNA in vitro. In vitro assays confirmed knockdown of PMP22 mRNA and protein by PMP22-shRNA. The CMT1A rat model carrying a PMP22-transgene (CPMP rats) were injected with PMP22-shRNA in vivo. Concecutively, we were able to detect the construct in the peripheral nerves by PCR for up to 7 days after injection. However, shRNA injections did not significantly knock-down PMP22 mRNA or protein in peripheral nerves. Functional and behavioral tests as well as nerve conduction studies did not show significant improvements in PMP22-shRNA versus scramble-shRNA or NaCl treated rats. In conclusion, the shRNA constructs in this study were effective in reducing PMP22 in vitro, but not in vivo with the treatment protocol used. Likewise, a functional benefit could not be achieved (Fig. 1).
doi:10.1016/j.clinph.2015.04.287
P193. Small fiber neuropathy after lightning injuries—R. Backhaus a, L. Kirzinger a, S. Platen a, I. Kleiter b, W. Schulte-Mattler a, B. Schalke a (a Universität Regensburg, Regensburg, Germany, b Ruhr-Universität, Bochum, Germany) Introduction: Survivors of lightning injuries suffer from several acute or delayed neurological symptoms. As a delayed complication chronic pain syndrome and signs of polyneuropathy are common. Methods: We examined 3 patients (2 male, 1 female) with clinical symptoms of parenthesis, chronic pain syndrome or other sensible sensations appearing after lightning injury in healthy patients before. Added to complete neurological work up, including electrophysiological examination and quantitative sensory testing (QST), biopsy of the skin in affected area was performed and prepared for histological examination. Results: Affection of peripheral nervous system was frequent, as were chronic pain syndroms. Reduction of temperature reception could be seen in quantitative sensory testing. Results correlated with founded consistent histological pattern of a small fiber neuropathy with reduction of density of intraepidermal nerve fiber in all patients. Conclusion: Next to electrophysiological examination biopsy of affected skin can help to objectify polyneuropathic sensations as a common effect of lightning strike. doi:10.1016/j.clinph.2015.04.288
P194. Nerve sonography guided diagnosis of painful peroneal palsies caused by intraneural ganglia – A case series—L. Schilg, A. Felbecker, S. Haegele-Link, B. Gers, J. Weber, B. Tettenborn, T. Hundsberger (Kantonsspital St. Gallen, St. Gallen, Switzerland)
Fig. 1.
Background: Acute peroneal palsy is frequently caused by compression, trauma and surgery. Risk factors are weight loss, sitting with crossed legs, wrong positioning during surgery and diabetes mellitus. Focal demyelination is more frequent than axonal damage which in turn is related with an inferior prognosis. Rarely, acquired peroneal palsy is caused by intraneural ganglia. Methods: We here report of three patients with untypical peroneal palsy caused by intraneural ganglia which were detected by nerve sonography (15 MHz Linear array transducer, GE Healthcare LOGIQ E9 Ultrasound). Results and conclusions: A careful clinical history as well as a profound clinical and electrophysiological examination is required to
e170
Society Proceedings / Clinical Neurophysiology 126 (2015) e63–e170
disclose unusual findings. These are common in non-typical peroneal palsy. In this situation high resolution nerve sonography is a fast and sensitive method to detect intraneural ganglia. In contrast to the much more frequent ‘‘loco typico’’ lesion intraneural ganglia can be treated by microscopic nerve surgery as part of primary
treatment strategy. Hence, detection of intraneural ganglia is meaningful for prognosis and guides therapy. doi:10.1016/j.clinph.2015.04.289
partial facial hypohidrosis or no hypohidrosis at all. We suspect a damage of the second order preganglionic sympathetic neuron, because gustatory sweating is most frequently described in preganglionic lesions. Despite the normal MRI scan, an association with the cervical surgery is probable. This case underlines the usefulness of ‘‘old’’ examination procedures like the starch-iodine sweat test in the topodiagnosis of focal dysautonomias like Horner‘s syndrome. doi:10.1016/j.clinph.2015.04.286
P192. Small hairpin RNA against PMP22 is effective in vitro but fails to improve the phenotype of a CMT1A rat model in vivo—B. Gess, K. Friedrich, D. Röhr, H. Halfter, P. Young (Universitätsklinikum Münster, Klinik für Schlafmedizin und neuromuskuläre Erkrankungen, Münster, Germany) CMT1A is the most common form of hereditary neuropathies, accounting for about 30–40% of all patients. A causative treatment is not available as of yet. CMT1A is based on a duplication of the gene PMP22. Thus, lowering PMP22 expression represents a promising avenue for treatment studies. In this pilot study, we used plasmid-vector encoded shRNA against PMP22 for posttranscriptional silencing of PMP22 in vitro and in vivo.
e169
Cell lines 3T3, HEK 293, and MSC80 (Schwann cell-derived) were used to test the effects of PMP22-shRNA in vitro. In vitro assays confirmed knockdown of PMP22 mRNA and protein by PMP22-shRNA. The CMT1A rat model carrying a PMP22-transgene (CPMP rats) were injected with PMP22-shRNA in vivo. Concecutively, we were able to detect the construct in the peripheral nerves by PCR for up to 7 days after injection. However, shRNA injections did not significantly knock-down PMP22 mRNA or protein in peripheral nerves. Functional and behavioral tests as well as nerve conduction studies did not show significant improvements in PMP22-shRNA versus scramble-shRNA or NaCl treated rats. In conclusion, the shRNA constructs in this study were effective in reducing PMP22 in vitro, but not in vivo with the treatment protocol used. Likewise, a functional benefit could not be achieved (Fig. 1).
doi:10.1016/j.clinph.2015.04.287
P193. Small fiber neuropathy after lightning injuries—R. Backhaus a, L. Kirzinger a, S. Platen a, I. Kleiter b, W. Schulte-Mattler a, B. Schalke a (a Universität Regensburg, Regensburg, Germany, b Ruhr-Universität, Bochum, Germany) Introduction: Survivors of lightning injuries suffer from several acute or delayed neurological symptoms. As a delayed complication chronic pain syndrome and signs of polyneuropathy are common. Methods: We examined 3 patients (2 male, 1 female) with clinical symptoms of parenthesis, chronic pain syndrome or other sensible sensations appearing after lightning injury in healthy patients before. Added to complete neurological work up, including electrophysiological examination and quantitative sensory testing (QST), biopsy of the skin in affected area was performed and prepared for histological examination. Results: Affection of peripheral nervous system was frequent, as were chronic pain syndroms. Reduction of temperature reception could be seen in quantitative sensory testing. Results correlated with founded consistent histological pattern of a small fiber neuropathy with reduction of density of intraepidermal nerve fiber in all patients. Conclusion: Next to electrophysiological examination biopsy of affected skin can help to objectify polyneuropathic sensations as a common effect of lightning strike. doi:10.1016/j.clinph.2015.04.288
P194. Nerve sonography guided diagnosis of painful peroneal palsies caused by intraneural ganglia – A case series—L. Schilg, A. Felbecker, S. Haegele-Link, B. Gers, J. Weber, B. Tettenborn, T. Hundsberger (Kantonsspital St. Gallen, St. Gallen, Switzerland)
Fig. 1.
Background: Acute peroneal palsy is frequently caused by compression, trauma and surgery. Risk factors are weight loss, sitting with crossed legs, wrong positioning during surgery and diabetes mellitus. Focal demyelination is more frequent than axonal damage which in turn is related with an inferior prognosis. Rarely, acquired peroneal palsy is caused by intraneural ganglia. Methods: We here report of three patients with untypical peroneal palsy caused by intraneural ganglia which were detected by nerve sonography (15 MHz Linear array transducer, GE Healthcare LOGIQ E9 Ultrasound). Results and conclusions: A careful clinical history as well as a profound clinical and electrophysiological examination is required to
e170
Society Proceedings / Clinical Neurophysiology 126 (2015) e63–e170
disclose unusual findings. These are common in non-typical peroneal palsy. In this situation high resolution nerve sonography is a fast and sensitive method to detect intraneural ganglia. In contrast to the much more frequent ‘‘loco typico’’ lesion intraneural ganglia can be treated by microscopic nerve surgery as part of primary
treatment strategy. Hence, detection of intraneural ganglia is meaningful for prognosis and guides therapy. doi:10.1016/j.clinph.2015.04.289