Effect of mexiletine on transitory depression of compound motor action potential in recessive myotonia congenita

2015 ◽  
Vol 126 (2) ◽  
pp. 399-403 ◽  
Author(s):  
Mauro Lo Monaco ◽  
Adele D’Amico ◽  
Marco Luigetti ◽  
Jean-François Desaphy ◽  
Anna Modoni
Neurosurgery ◽  
2009 ◽  
Vol 65 (suppl_4) ◽  
pp. A153-A159 ◽  
Author(s):  
Martijn J.A. Malessy ◽  
Willem Pondaag ◽  
J. Gert van Dijk

Abstract OBJECTIVE Obstetric brachial plexus lesions (OBPLs) are caused by traction to the brachial plexus during labor. Typically, in these lesions, the nerves are usually not completely ruptured but form a “neuroma-in-continuity.” Even in the most severe OBPL lesions, at least some axons will pass through this neuroma-in-continuity and reach the tubes distal to the lesion site. These axons may be particularly prone to abnormal branching and misrouting, which may explain the typical feature of co-contraction. An additional factor that may reduce functional regeneration is that improper central motor programming may occur. Surgery should be restricted to severe cases in which spontaneous restoration of function will not occur, i.e., in neurotmesis or root avulsions. A major problem is how to predict whether function will be best after spontaneous nerve outgrowth or after nerve reconstructive surgery. When a decision has been made to perform an early surgical exploration, what to do with the neuroma-in-continuity can be a problem. The intraoperative appraisal is difficult and depends on experience, but even in experienced hands, misjudgment can be made. METHODS We performed an observational study to assess whether early electromyography (at the age of 1 month) is able to predict severe lesions. Additionally, the value of intraoperative nerve action potential and compound motor action potentials was investigated. RESULTS Severe cases of OBPL can be identified at 1 month of age on the basis of clinical findings and needle electromyography of the biceps. This outcome needs independent validation, which is currently in progress. Nerve action potential and compound motor action potential recordings show statistically significant differences on the group level between avulsion, neurotmesis, axonotmesis, and normal. For the individual patient, a clinically useful cutoff point could not be found. Intraoperative nerve action potential and compound motor action potential recordings do not add to the decision making during surgery. CONCLUSION The absence of a “gold standard” for the assessment of the severity of the OBPL lesion makes prognostic studies of OBPL complex. The currently available assessment strategies used to obtain the best possible solutions are discussed.


2008 ◽  
Vol 109 (5) ◽  
pp. 946-954 ◽  
Author(s):  
Willem Pondaag ◽  
Lieven P. A. J. van der Veken ◽  
Paul J. van Someren ◽  
J. Gert van Dijk ◽  
Martijn J. A. Malessy

Object A typical finding in supraclavicular exploration of infants with severe obstetric brachial plexus lesions (OBPLs) is a neuroma-in-continuity with the superior trunk and/or a root avulsion at C-5, C-6, or C-7. The operative strategy in these cases is determined by the intraoperative assessment of the severity of the lesion. Intraoperative nerve action potential (NAP) and evoked compound motor action potential (CMAP) recordings have been shown to be helpful diagnostic tools in adults, whereas their value in the intraoperative assessment of infants with OBPLs remains to be determined. Methods Intraoperative NAPs and CMAPs were systematically recorded from damaged and normal nerves of the upper brachial plexus in a consecutive series of 95 infants (mean age 175 days) with OBPLs. A total of 599 intraoperative NAP and 836 CMAP recordings were analyzed. The severity of the nerve lesions was graded as normal, axonotmesis, neurotmesis, or root avulsion, based on surgical, clinical, histological, and radiographic criteria. Results The correlation of NAP and CMAP recordings with the severity of the lesion was assessed. The specificity of an absent NAP or CMAP to predict a severe lesion (neurotmesis or avulsion) was > 0.9. However, the sensitivity of an absent NAP or CMAP for predicting a severe lesion was low (typically < 0.3). The severity of the nerve lesion was related to CMAP and NAP amplitudes. Cutoff points useful for intraoperative decision making could not be found to differentiate between lesion types in individual patients. Conclusions Intraoperative NAP and CMAP recordings do not assist in decision making in the surgical treatment of infants with OBPLs. The authors' findings in infants cannot be generalized to adults.


2020 ◽  
Vol 2020 ◽  
pp. 1-8 ◽  
Author(s):  
Jihyeung Kim ◽  
Young Eun Choi ◽  
Jeong Hwan Kim ◽  
Seung Hak Lee ◽  
Sohee Oh ◽  
...  

Purpose. Although many surgeons have anecdotally described reversing the polarity of the autograft with the intent of improving regeneration, the optimal orientation of the autogenous nerve graft remains controversial. The aim of this study was to compare (1) the outcomes of orthodromic and antidromic nerve grafts to clarify the effect of nerve graft polarity and (2) the outcome of either form of nerve grafts with that of nerve repair. Methods. In 14 of the 26 rabbits used in this study, a 1 cm defect was made in the tibial nerve. An orthodromic nerve graft on one side and an antidromic nerve graft on the other were performed using a 1.2 cm long segment of the peroneal nerve. In the remaining 12 rabbits, the tibial nerve was transected completely and then repaired microscopically on one side but left untreated on the other. Electrophysiologic studies were performed in all animals at 8 weeks after surgery, and the sciatic nerves were harvested. Results. Compound motor action potential was visible in all rabbits treated by nerve repair but in only half of the rabbits treated by nerve graft. There was no significant difference in the compound motor action potential, nerve conduction velocity, or total number of axons between the orthodromic and antidromic nerve graft groups. However, in both groups, the outcome was significantly poorer than that of the nerve repair group. Conclusion. There was no significant difference by electromyographic or histologic evaluation between orthodromic and antidromic nerve grafts. Direct nerve repair with moderate tension may be a more effective treatment than nerve grafting.


1996 ◽  
Vol 82 (2) ◽  
pp. 317-320 ◽  
Author(s):  
Peter G. Atanassoff ◽  
Branko M. Weiss ◽  
Sorin J. Brull ◽  
Andreas Horst ◽  
Daniel Kulling ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document