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Biomedical subjects

G L Sheean

Publications and source records attributed to G L Sheean.

18 recordsLinked to original sources

Botulinum treatment of spasticity: why is it so difficult to show a functional benefit?

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.

Botulinum Toxins, Type A↗

Abnormal motor unit synchronization of antagonist muscles underlies pathological co-contraction in upper limb dystonia.

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.

Adolescent↗

An open-labelled clinical and electrophysiological study of 3,4 diaminopyridine in the treatment of fatigue in multiple sclerosis.

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.

4-Aminopyridine↗

Further studies using higher doses of botulinum toxin type F for torticollis resistant to botulinum toxin type A.

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.

Adult↗

Myasthenic hand.

Explore the source record for details and available documents.

Aged↗

An electrophysiological study of the mechanism of fatigue in multiple sclerosis.

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.

Adult↗

The effect of botulinum toxin on hand function after incomplete spinal cord injury at the level of C5/6: a case report.

OBJECTIVE: To investigate the benefits of the focal use of botulinum toxin in spasticity in the forearm seen after incomplete spinal cord injury. DESIGN: A single case study with standardized assessment before and at three-week intervals after injection. INTERVENTION: EMG-guided selective injection of botulinum toxin. SUBJECT: A 23-year-old man, 18 months post injury. MEASURES: Rivermead Motor Assessment; grip strength; Jebsen hand tests; visual analogue scale; Ashworth spasticity scale. RESULTS: Weakness was seen as expected with some functional losses, but the patient made gains in the areas of concern: shaking hands, typing, using the hand to drink. These gains were sustained at 12 weeks. CONCLUSION: Selective use of botulinum toxin to weaken muscles can lead to functional benefit.

Adult↗

Treatment of nonoccupational limb and trunk dystonia with botulinum toxin.

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.

Adolescent↗

Pain and remote weakness in limbs injected with botulinum toxin A for writer's cramp.

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.

Adult↗

Lumbrical-interosseous latency comparison in the diagnosis of carpal tunnel syndrome.

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.

Adult↗

Electrodiagnosis.

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.

Electrodiagnosis↗

Botulinum toxin F in the treatment of torticollis clinically resistant to botulinum toxin A.

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.

Adult↗

Lithium neurotoxicity.

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.

Acute Disease↗