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Multiple sclerosis is a complex disease which presents a unique challenge in clinical management. This decade has seen the advent of new therapies which are partially effective in modifying the disease course. However, current therapies have no impact on existing neurological deficits and so supportive measures, symptomatic treatment and comprehensive rehabilitation remain at the core of management. Historically clinical practice has often been empirical but management should now be evidence‐based and guided by consensus opinion if improvements in care are to be made. (Int J Clin Pract2000; 54(3): 161‐169)

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.

The role of an inpatient neurorehabilitation unit in the management of multiple sclerosis (MS) remains unclear. A necessary first step is the demonstration of benefit in those who are admitted. In order to address this question, 79 patients with MS admitted over a 16-month period for multidisciplinary rehabilitation were studied using assessment scales as measures of disability and handicap. Fourteen patients were recovering from a recent relapse and the remaining 65 were in the progressive phase of the disease. The patients were clinically assessed and rated on admission and discharge using Kurtzke's Disability Status Scale (DSS), the Barthel Index (BI) and the Environmental Status Scale (ESS). A statistically significant functional improvement was seen in 65% of patients as determined by the Barthel Index and 44% improved on the ESS. Improvement was most marked in those in whom a reduction in impairment had occurred during recovery from relapse but also occurred in 63% of those who had not changed neurologically. These results suggest a beneficial effect from admission to a neurorehabilitation unit for patients with MS, including those in the progressive phase of the disease, although its role in the long-term management of MS remains to be defined.

In multiple sclerosis (MS) myelin damage is the result of a chronic inflammatory process mediated by CD4 positive T helper/effector cells. In experimental allergic encephalomyelitis (EAE), the animal model of MS, treatment with anti-CD4 antibodies can prevent the onset of disease. Natural history studies have demonstrated that gadolinium enhanced magnetic resonance imaging (MRI) of the brain is more sensitive and objective in assessing inflammatory disease activity in MS than clinical monitoring, so that less patients and shorter studies suffice to reach the same statistical power as compared to trials using clinical outcome parameters. In this paper we describe the design of an exploratory trial of chimeric monoclonal anti-CD4 antibodies in the treatment of MS. For this study we chose the number of active MS lesions on monthly gadolinium enhanced MRI scans as the primary outcome measure.

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 tests. 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.Key words: multiple sclerosis; fatigue; amino pyridines; K+ channel blocker; conduction block

A definitive diagnosis of multiple sclerosis cannot be made at presentation on patients with a clinically isolated syndrome of the optic nerve, spinal cord or brainstem suggestive of demyelination, as dissemination in time is not established. To determine the long-term risk of abnormalities on brain MRI for the development of multiple sclerosis and disability we performed a 10-year follow-up on 81 such patients who had T2-weighted brain MRI at presentation. Initial brain MRI was abnormal in 54 (67%). Follow up of those patients with an abnormal MRI revealed progression to clinically definite multiple sclerosis in 45 out of 54 (83%), of whom 11 (20%) had relapsing/remitting disease (EDSS > 3), 13 (24%) secondary progressive and 21 (39%) benign (relapsing/remitting with EDSS  3) disease. For those with a normal MRI progression to clinically definite multiple sclerosis occurred in only three out of 27 (11%), all benign. There was a significant relationship between the number of lesions at presentation and both EDSS = 0.45, P < 0.001) and the type of disease at follow-up (P < 0.0001). Brain MRI at presentation with a clinically isolated syndrome is predictive of the long-term risk of subsequent development of multiple sclerosis, the type of disease and extent of disability.Keywords:clinically isolated syndromes; multiple sclerosis

We report the findings in 60 patients with secondary progressive multiple sclerosis who had monthly brain MRI studies for 4 months (one baseline and three follow-up scans). The purpose was to define the sort-term MRI natural history in a large cohort with secondary progressive disease and to ascertain its relationship with other clinical and MRI features. The patients were participating in either a natural history study or the placebo arm or non-treatment phase of a therapeutic trial. The cohort had clinical had clinical features typical of secondary progressive disease: thus, all had moderate or severe locomotor disabilities [Expanded Disability Status Scale (EDSS), score 3.5-8], with a median disease duration of 12 years. There was equal representation of males and females. During the 3 months of follow-up there was a total of 362 new enhancing lesions seen in 42 patients, and there were 24 relapses in 20 patients. There was no correlation between new enhancing lesions and age at study entry, age of disease onset, gender, disease duration or EDSS, but there was a strong correlation with the number of enhancing lesions on the baseline scan (r = 0.65, P <0.0001) and subsequent activity. There was a non-significant trend for higher numbers of new enhancing lesions in those having relapses during the 3 months of scanning (P = 0.14) or in the preceding 6 months (P = 0.06). The 34 patients who did not relapse in either period had significantly fewer new active lesions (P = 0.02) than those who relapsed at some stage during the 9 months. Nevertheless, considerable activity was seen in the non-relapsing cohorts: there was a mean of 3.5 (median 2) new enhancing lesions in those not relapsing during the 3 month study, and 5.5 (median 2) in those not relapsing in the previous 6 months. We conclude that short term MRI activity is generally high in secondary progressive disease, confirming a useful role for the technique in exploratory trials. Further work should concentrate on elucidating the mechanisms of secondary progression by longer term follow-up studies of lager cohorts using multiple MRI and clinical measurements.Key words: multiple sclerosis; secondary progressive; MRI; exploratory treatment trials

The role of magnetic resonance imaging (MRI) in predicting disability in multiple sclerosis (MS) remains unclear. In this study 21 patients with primary and secondary progressive MS were reviewed 5 years following a serial MRI study of 6 months duration. In the secondary progressive group (n=11) there was a significant relationship between the occurrence of enhancing lesions and clinical relapses during the initial 6 months and increase in diability 5 years later. For both groups change in disability over the initial study period was predictive of outcome. These results suggest that the presence and frequency of gadolinium enhancement (a marker of inflammation) and changes in disability over a short period are predictive of future deterioration in progressive patients.

AimsHierarchy and leadership are essential within any multidisciplinary team. However, to err is human and team leaders can make mistakes irrespective of seniority. It is important that everyone within the team feels confident in raising concerns to ensure patient safety. This can be particularly challenging for new staff joining established healthcare teams. The Advanced Paediatric Life Support (APLS) Group recommend the PACE approach as a structured method for raising concerns.Abstract G49 Table 1StageLevel of ConcernP - ProbeI think you need to know what is happeningA - AlertI think something bad might happenC - ChallengeI know something bad will happenE - EmergencyI will not let it happenWe aimed to improve the confidence of new children’s nursing staff in raising concerns by introducing teaching on this topic into their induction simulation session.MethodsNew nurses undertook a simulated clinical scenario in which the doctor was deliberately hesitant and reluctant to administer appropriate treatment. Teaching was then provided on the PACE approach. Each nurse then participated again in a similar scenario. Using pre and post questionnaires we asked the 23 participants to suggest on a Likert scale how confident they would feel in challenging a nursing colleague or doctor if they had concerns about their practice before and after the session. During the debrief of the first scenarios we asked participants how they had felt.ResultsThere was no difference in participants’ confidence between challenging a nurse or doctor (3.4/5) prior to training. Following the initial scenario the nurses reported feeling ‘frustrated’ and ‘scared’.One stated ‘this is my worst fear.’ After the PACE teaching and subsequent scenarios nursing staff stated that their confidence in challenging nurses and doctors had increased to 4.3/5 and 4.2/5 respectively. Free text comments included:‘Hearing from doctors that they would rather be challenged was reassuring.’‘Improved my confidence to speak up when querying a decision’ConclusionNursing staff reported improved confidence in their ability to raise concerns. This can only serve to improve patient safety. We have subsequently embedded PACE training within all our medical and nursing induction programmes.

AimsThe Royal College of Paediatrics and Child Health (RCPCH) provides a comprehensive curriculum for paediatric trainees. It details the knowledge, skills and behaviours expected from a trained paediatrician and highlights the core competences required of trainees. Achieving these competencies can be difficult for more uncommon conditions which trainees do not routinely encounter in clinical practice. We sought to address this by offering senior paediatric trainees the opportunity to participate in inter-professional, high-fidelity, simulated scenarios based on rare but important clinical conditions.MethodsWe surveyed all level three paediatric trainees (ST6– ST8) in our deanery. We listed the acute presentations as outlined in the RCPCH for general paediatrics and asked them to identify conditions which they had either never experienced or had only had very limited exposure to in clinical practice. We incorporated routine simulation teaching, focusing on these rare cases, into the postgraduate education programme in the deanery’s tertiary paediatric centre. Trainees who participated were asked to complete an online questionnaire to explore their experience of these teaching sessions.ResultsTrainees identified a number of conditions with which they lacked clinical experience. Table 1 highlights some of the conditions trainees mentioned.Abstract G349(P) Table 1Examples of rare cases as lsited by level three trainees:Acute liver failureInfected EndocarditisNeonatal thyrotoxicosisSuperior vena cava obstruction secondary to malignancySalt poisoning (Factitious of induced illness)Feedback on these sessions was very positive. Comments included:‘These sessions enabled me to develop my confidence in the management of conditions which I rarely see in clinical practice.’‘Rare case simulation training allows me to meet curriculum competencies which I have not previously been able to achieve.’‘Challenging and educational.’ConclusionsSimulation training focusing on rare cases allows trainees to develop their confidence in managing uncommon conditions and address areas of the curriculum which they have previously struggled to achieve. After a successful local pilot we have now also embedded rare case simulation training into the deanery’s regional paediatric teaching programme.