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Objectives: Evaluate correlation between donor nerve semi-quantitative electromyography (sqEMG) and strength outcome in nerve transfer surgery., Methods: Retrospective review of pre-operative donor nerve semi-quantitative neurophysiology and post-operative recipient muscle force after at least one-year follow-up. The semi-quantitative technique is the average motor unit number estimate associated with needle recorded interference patterns in the donor muscle (IP-AMUNE), which was correlated with hand-held manometry, standardized as a percent of the contralateral arm, using multivariable linear regression with backward selection., Results: Twenty-eight nerve transfer cases were included. The correlation between the donor nerve IP-AMUNE and the recipient muscle strength was moderate to strong and highly significant (r = 0.67, p < 0.001). Medical Research Council (MRC) grading did not predict strength (p > 0.54)., Conclusions: IP-AMUNE is a good predictor of strength after nerve transfer surgery and should be considered in the evaluation and planning of patients undergoing nerve transfer to aid in donor nerve selection., Significance: IP-AMUNE may significantly benefit those undergoing nerve transfer surgery for the restoration of movement., (Copyright © 2019 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

A successful nerve transfer surgery can provide a wealth of benefits to a patient with cervical spinal cord injury. The process of surgical decision making ideally uses all pertinent information to produce the best functional outcome. Reliance on clinical examination and imaging studies alone can miss valuable information on the state of spinal cord health. In this regard, neurophysiological evaluation has the potential to effectively gauge the neurological status of even select pools of anterior horn cells and their axons to small nerve branches in question to determine the potential efficacy of their use in a transfer. If available preoperatively, knowledge gained from such an evaluation could significantly alter the reconstructive surgical plan and avoid poor results. The authors describe their institution's approach to the assessment of patients with cervical spinal cord injury who are being considered for nerve transfer surgery in both the acute and chronic setting and broadly review the neurophysiological techniques used.

Unlabelled: The use of transcranial motor evoked potentials (TCMEPs) to detect and hopefully prevent injury to the brain, spinal cord, and peripheral nerves intraoperatively has increased greatly in recent years. It is well established that in addition to certain anesthetic agents, patient factors such as advanced age, obesity, diabetes, hypertension, and a collection of neurological and neuromuscular diseases and disorders can greatly reduce or completely eliminate the ability to monitor TCMEPs effectively. One such disease, poliomyelitis (polio), is a highly contagious viral disease that has been mostly forgotten since its near-eradication through vaccination. Over the past three decades there has been increasing recognition of late onset neurological deterioration in individuals who were afflicted by, and apparently recovered from, paralytic poliomyelitis much earlier in life. This condition is known as post-poliomyelitis syndrome (PPS). Patients that appear to have fully recovered from polio, and those with PPS, may require special anesthetic considerations to facilitate effective TCMEP monitoring., Case Report: We report the rapid loss of only lower extremity TCMEPs bilaterally during a C6-C7, C7-T1 ACDF in a 67-year-old female to treat left-sided C7-C8 radiculopathy and C6-T1 foraminal stenosis. The general anesthetic maintenance regimen of 0.3 MAC sevoflurane and 100 microg/kg/min propofol was paused, and a wake-up test was initiated. Full upper and lower extremity motor function was observed. A thorough review of the patient's medical history revealed the potential risk factor of full recovery from poliomyelitis as a child. The sevoflurane was removed from the anesthetic regimen, and the lower extremity TCMEPs returned and were present for the remainder of the surgery.

Surgical interventions to improve function following nervous system injury have been in development since the early 1900s. Only recently these have been assimilated into a coherent approach which can be applied to injuries of the brain, spinal cord and peripheral nerves. In addition to pharmacological and stimulation based interventions, surgical manipulation of the peripheral nerves and muscles of the extremity can offer functional enhancement for a variety of limb impairments. In order to plan an effective surgical intervention, neurophysiological assessment of the injury and residual motor control is essential. Effective implementation of these surgical interventions can enhance function and quality of life for many individuals whose activity has been limited as a result of nervous system injury., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Quantification in clinical, diagnostic electromyography (EMG) currently includes motor unit action potential (MUAP) analysis and interference pattern analysis. Early efforts to examine the frequency/power spectra of the interference pattern showed modest value but the technique was not developed further. This paper re-examines spectral analysis, extending it into the time-varying domain, which has never been studied in diagnostic needle EMG. Time-frequency and time-scale analysis employing wavelet and non-wavelet techniques were applied to short trains of MUAPs. The results show that time-varying analysis produces clear visual representations of the energy content of individual MUAPs within an interference pattern. The time frequency representations allow easy, qualitative distinction between normal and neurogenic MUAPs. Furthermore, the quantified MUAP energy correlates well with the current morphological standard and the quantification process is substantially faster. Time-varying analysis links classical power spectral analysis in the realm of interference patterns with quantitative MUAP analysis. In addition to morphological classification, MUAPs might also be classified by energy content, which more closely reflects the physical and physiological consequences of neuromuscular pathology on the motor unit., (Published by Elsevier Ltd.)

Objective: Motor unit action potentials (MUAPs) recorded by needle electrode reflect the functional state of the motor unit and its force-generating capacity, and are usually described morphologically (e.g. amplitude, duration). However, since the purpose of motor unit activation is force generation, MUAP energy seems a more physically meaningful measurement., Methods: MUAPs were obtained by multi-MUAP decomposition of real interference patterns taken from human patients with neurological diseases. The energy content of each MUAP was measured from a time-frequency representation (TFR), specifically the Choi-Williams distribution, and compared with the standard MUAP morphological measure, the Size Index. The sample included normal, neurogenic, and myopathic MUAPs, from 11 patients., Results: There is an exponential distribution of energy within a sample of MUAPs and a strong exponential relationship between the Size Index and MUAP energy was observed., Conclusions: The energy content of a MUAP can be quantified and corresponds very well with the current quantitative standard. Energy is a possible addition to MUAP quantification., Significance: MUAPs could be classified as having normal, large (neurogenic), or low (myopathic) energy. MUAP energy has direct physical and physiological meaning that reflects the force-generating capacity of the motor unit. Time-frequency analysis could also be used to study the specific frequency content of MUAPs and the energy of MUAPs within an interference pattern, without the need for decomposition., (Copyright © 2011 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

Botulinum toxin type A (Dysport) has been shown in European studies to be a safe and effective treatment for cervical dystonia. This multicenter, double-blind, randomized, controlled trial assessed the safety and efficacy of Dysport in cervical dystonia patients in the United States. Eighty patients were randomly assigned to receive one treatment with Dysport (500 units) or placebo. Participants were followed up for 4 to 20 weeks, until they needed further treatment. They were assessed at baseline and weeks 2, 4, 8, 12, 16, and 20 after treatment. Dysport was significantly more efficacious than placebo at weeks 4, 8, and 12 as assessed by the Toronto Western Spasmodic Torticollis Rating Scale (10-point vs. 3.8-point reduction in total score, respectively, at week 4; P < or = 0.013). Of participants in the Dysport group, 38% showed positive treatment response, compared to 16% in the placebo group (95% confidence interval, 0.02-0.41). The median duration of response to Dysport was 18.5 weeks. Side effects were generally similar in the two treatment groups; only blurred vision and weakness occurred significantly more often with Dysport. No participants in the Dysport group converted from negative to positive antibodies after treatment. These results confirm previous reports that Dysport (500 units) is safe, effective, and well-tolerated in patients with cervical dystonia., (Copyright 2005 Movement Disorder Society.)

Clinical experience seems to indicate that botulinum toxin injections can, in selected patients with upper motor neurone syndrome, reduce spasticity and improve voluntary movement and active function. However, double-blind placebo-controlled trials have had difficulty showing active functional improvement, despite the clear ability of botulinum toxin to reduce spasticity. This prompts a re-analysis of the basic assumption that spasticity impairs voluntary movement and a review of the methodology of the clinical trials. Motor dysfunction is usually caused by weakness and the other "negative" features of upper motor neurone syndrome, rather than muscle overactivity. Recent research has explored the pathophysiological basis of the voluntary movement disorder, in particular the role of the various forms of motor overactivity, which might be amenable to botulinum toxin treatment. The failure of double-blind placebo-controlled clinical trials to show improvement in active function is, to a large extent, a result of their methodology, especially patient selection, injection protocols, and the choice of outcome measures. Clinical trials need to be re-designed and based upon expert experience and a better understanding of the pathophysiology of the motor disorder.

We studied the electrophysiological parameters of motor performance in eight patients with multiple sclerosis and troublesome fatigue, before and after treatment with 3,4-diaminopyridine. Symptomatic fatigue was evaluated by the Krupp Fatigue Severity Score and motor performance of adductor pollicis by transcranial magnetic stimulation, rapid voluntary movements and a fatiguing exercise test of a sustained 45-s isometric contraction. The motor tests revealed baseline abnormal motor function and substantial central fatigue. After a 3-week course of 3,4-diaminopyridine (25-60 mg/day), six out of the eight patients reported substantial improvement in fatigue and the group showed slightly less fatigue on the exercise test. Other electrophysiological tests of motor function were unchanged. The findings suggest that 3,4-diaminopyridine may play a role in the symptomatic treatment of fatigue in multiple sclerosis. However, the mechanism behind such a benefit in fatigue remains unclear and the discrepancy between subjective and more objective responses underlines the probable multifactorial nature of the pathogenesis of this symptom in multiple sclerosis.

Objective: A previous study of botulinum toxin type F (BTX-F) treatment for torticollis had shown a dose of 520 MU to be effective, but for a much shorter duration than is usual with botulinum toxin type A (BTX-A). The objective was to assess the effect of a higher dose of BTX-F., Methods: Four of the previously treated patients, plus an additional patient, were treated with a higher dose of 780 MU BTX-F. All were secondary nonresponders to BTX-A due to neutralising antibodies. A test injection of 40 MU BTX-F was also given into the extensor digitorum brevis muscle (EDB), to examine the time course of the biological effect of the toxin electrophysiologically. Patients were followed up at two, four, eight, and 12 weeks., Results: All patients reported subjective improvement lasting from seven to 11 (mean 8.6) weeks accompanied by a significant reduction in mean clinical severity scores at two weeks. Four patients had pain which was substantially reduced. The electrophysiological studies confirmed biological sensitivity to the toxin in all patients, showing a significant change beginning at two weeks and returning to baseline at 12 weeks. The time course of this effect paralleled roughly that of the clinical response. The four patients who had previously received 520 MU BTX-F reported that the response was better and longer in duration with 780 MU. Dysphagia was more common than reported with the lower dose., Conclusion: Better results are possible with higher doses of BTX-F but the duration of benefit is still shorter than with BTX-A, seemingly due to a shorter duration of neuromuscular junction blockade.

The aim of this study was to examine the pathophysiological mechanisms underlying co-contraction in patients with dystonia (n = 6) and writer's cramp (n = 5). Multi-unit needle and surface EMGs were recorded from extensor carpi radialis (ECR) and flexor carpi radialis (FCR) muscles during motor tasks that elicited dystonia or writer's cramp. The EMGs from ECR and FCR were recorded simultaneously and analysed using cross-correlation analysis. Similar recordings were obtained from healthy age- and sex-matched control subjects (n = 8). Despite co-contraction of the muscles, cross-correlograms from the healthy subjects did not reveal evidence of motor unit synchronization. Cross-correlograms from the dystonic subjects revealed a central peak with a median duration of 37 ms, indicating broad-peak motor unit synchronization. Cross-correlograms from patients with writer's cramp were either flat or modulated by a 11-12-Hz tremor. Frequency-domain analysis of ECR and FCR EMGs demonstrated significant coherence in the patients with dystonia and writer's cramp. These results indicate that co-contraction in dystonia is neurophysiologically distinct from voluntary co-contraction and is produced by abnormal synchronization of presynaptic inputs to antagonist motor neuron pools. ECR and FCR co-contraction in writer's cramp may be a compensatory process under voluntary control.

Fatigue is a common and disabling symptom in multiple sclerosis but is poorly understood. We investigated 'physiological' fatigue in 21 patients with multiple sclerosis who complained of disabling fatigue by measuring the decline in strength during a 45 s maximal contraction of the adductor pollicis muscle. The results were compared with those from a control group of 19 healthy subjects. The strength of control subjects declined by approximately 20% during the contraction; twitch interpolation showed central drive remained almost maximal throughout, and therefore that the fatigue was peripheral in origin. Patients had normal baseline strength, but developed greater fatigue (approximately 45%), which was central in origin. In both cases, the decline in strength followed a roughly linear time course suggesting that the patients, like the normals, were trying to maintain a maximum voluntary effort. Evidence for frequency-dependent conduction block (FDCB) in the patients' central motor pathways was sought by measuring the EMG responses to single and paired transcranial magnetic stimuli. Fatigue had no effect on the latency or size of EMG responses to transcranial magnetic stimulation, suggesting that FDCB was unlikely to have occurred. This was supported by measurements of the maximum speed of voluntary muscle contraction; although the patients showed relatively slow speeds before exercise, the decline in speed after fatigue was no greater than in normal subjects. We conclude that excessive 'physiological' fatigue contributes to the symptom of fatigue in multiple sclerosis and is central in origin. However, since the degree of exercise-induced fatigue did not correlate with the baseline complaint of fatigue, other factors must also be operating to produce the full range of clinical symptoms. We found no conclusive evidence that central fatigue is related to increased dysfunction in the primary central motor pathways and no evidence that FDCB is the pathophysiological mechanism. We postulate that central fatigue in multiple sclerosis is due to impaired drive to the primary motor cortex and several lines of evidence strongly suggest that this is not due to a lack of motivation.

We report the results of treatment of 16 patients (14 women, two men; 18-81 years old) with nonoccupational limb and trunk dystonia with botulinum toxin A (BTX; Dysport). A total of 18 clinical problems were identified. Outcomes were assessed in terms of pain relief and improvement in posture and function by the combined observations of the patient and physician. Patients' satisfaction with treatment was high--the benefit in 15 of 18 problems was rated as good to excellent. Reduction in pain was achieved in nine of 10 painful problems, with total relief in four cases. Some normalisation of posture was obtained in 17 of 18; it was complete in three cases. Functional improvement was less common (10 of 18). Excessive weakness was the most common side effect, affecting five patients, but it was disabling in only two. We conclude that BTX can provide substantial benefit with minimal side effects in the majority of patients with these conditions, particularly with pain relief and postural improvements.

We examined 66 hands referred with suspected carpal tunnel syndrome (CTS) using the second lumbrical-interosseous distal motor latency difference (2LI-DML) as well as standard tests: Forty-nine cases of CTS were diagnosed by the standard tests, 48 of whom had an abnormal median-ulnar palmar velocity comparison and 48 an abnormal 2LI-DML. The results of these 2 tests were closely correlated. The 2LI-DML supported the diagnosis of CTS in all cases except one, where the result was borderline. In one suspected case the 2LI-DML was the only abnormality. In 9 severe cases no median palmar responses could be obtained but an abnormal 2LI-DML was found. We conclude that the 2LI-DML is as sensitive as the palmar comparison and thus will support the diagnosis of CTS made by standard tests by providing an additional abnormality but that its routine use is unlikely to increase the diagnostic yield. Its value therefore may be in mild cases where the median-ulnar palmar comparison is normal or equivocal and in severe cases where standard test responses are unobtainable. It has also proved useful as a quick and simple screening test for CTS on the asymptomatic side.

Two reports have shown a Japanese preparation of botulinum toxin type F (BTX-F) to be an effective alternative for patients with torticollis who develop clinical resistance to botulinum toxin type A (BTX-A). A group of patients with torticollis, comprising five secondary non-responders and one primary non-responder, were treated with a preparation of BTX-F produced in the UK (Speywood Pharmaceuticals). A low dose of BTX-F (220 mouse units (MU) in total) was given into clinically affected neck muscles, followed six weeks later by an injection of a total of 520 MU. Antibodies to BTX-A (mouse protection assay) were present in all secondary non-responders but not in the primary non-responder. No patients developed atrophy after injection of Dysport BTX-A (40 MU) into the left extensor digitorum brevis muscle whereas pronounced atrophy occurred in all patients after injection of 40 MU of BTX-F into the right extensor digitorum brevis muscle. Three patients improved subjectively after treatment with 220 MU BTX-F and five (all secondary non-responders) after the subsequent dose of 520 MU (two considerably), with reduced Tsui scores, but group scores were only significantly changed after the higher dose. The primary non-responder remained unchanged after both doses of BTX-F. One patient reported mild dysphagia with 520 MU BTX-F. Mean duration of improvement with 520 MU BTX-F was five (range 4-6)weeks. Thus BTX-F provides benefit for BTX-A non-responders with few side effects but for a shorter period than BTX-A, possibly due to relative underdosing. As with BTX-A, biological sensitivity to BTX-F does not necessarily predict a clinical response.

Attempts to increase the diagnostic yield and reproducibility of electrophysiological investigation of peripheral nerve disorders have led to the development of new techniques, such as measurement of nerve refractoriness, as well as the re-evaluation, refinement and modification of more conventional nerve conduction tests. Other studies have characterized the clinical and electrophysiological features of the subtypes of polyneuropathies associated with monoclonal gammopathies and have documented their natural history, providing important diagnostic and prognostic information. Techniques originating in basic neuroscience that study the excitability of neurons and nerve membranes have been applied to clinical conditions, such as motor neuron disease and cramps, and have provided considerable insight into their pathogenesis.

We describe two cases of a syndrome resembling neuralgic amyotrophy that occurred in limbs injected with botulinum toxin for writer's cramp. In both cases, one of the injections was followed by pain and the next, 7-10 weeks later, by weakness. We believe that unexplained pain in the shoulder region after an injection of botulinum toxin contraindicates a second injection.

Lithium is potentially toxic to many parts of the central and peripheral nervous systems. Clinical lithium neurotoxicity may appear at any time during therapy and probably often goes unrecognised, at least for a time. Acute lithium toxicity has a mortality of 15%, and 10% of survivors suffer permanent neurological sequelae that are largely unpredictable though persons with the longest and most clinically severe intoxication are probably at highest risk. Even rapidly effective treatment with haemodialysis will not always protect against permanent residual neurological deficits. Lithium may also produce neurotoxic syndromes which develop chronically. There is a large variation among patients in relation to what constitutes a toxic serum lithium level. Both acute and chronic toxicity can occur with therapeutic range serum lithium levels. Failure to appreciate this fact may lead to delays in diagnosis and treatment, placing the patient at risk of permanent neurological damage or death. The diagnosis of lithium intoxication is largely clinical though the EEG may help if typical though non-specific EEG changes are present. If available, the red cell:plasma lithium ratio may be a sensitive indicator of intoxication. Prompt and effective treatment is indicated once the diagnosis of lithium intoxication is made. Prevention of intoxication, which requires the active involvement of both the doctor and patient, is crucial.