Microsoft word - abn short guidelines, sept 2002

The medical management of motor neurone disease – a UK perspective of current practice
The UK MND Interest group#
Guidance on the management of motor neurone disease (MND), sometimes also known as amyotrophic lateral sclerosis (ALS), has previously appeared in documents including the Practice Parameter on the Care of the Patient with Amyotrophic Lateral Sclerosis [1] and the Practice Advisory on the use of Riluzole in the Management of Amyotrophic Lateral Sclerosis [2], both developed under the auspices of the American Academy of Neurology. While these documents give an excellent overview of their respective topics, this paper is aimed at hospital doctors and general practitioners looking after MND sufferers, their carers and families, in the UK. It is intended to emphasise the opportunities for symptomatic treatments which can ease the burden of suffering in this most This document is evidence-based where possible and, where evidence is not available, it is based on expert opinion of best practice. It has been developed by the UK MND Interest Group, based on an earlier document endorsed by Council of the Association of British Neurologists in June 1999 which was widely circulated among neurologists and others but not formally published. This document has been updated to take account of developments in practice since that time as well as recent guidance from the National Institute for Clinical Excellence (NICE) [3] on the use of riluzole Current management of MND however does not depend solely on medical treatment. The variability of symptoms and the rapid development of significant neurological disability also necessitate timely intervention from a wide range of staff in other health care disciplines. The quality of life of MND sufferers is increased when patients are managed by a multi-disciplinary team with a specific commitment to MND. Although it is not possible to provide access to such teams everywhere, it is hoped that this paper will increase awareness of the opportunities for symptomatic, as well as disease modifying, therapies among clinical colleagues. The World Federation of Neurology (WFN) Research Group on Neuromuscular Diseases has [ criteria/]. Although widely accepted these criteria were designed as an aid to clinical trials and research. They might thus be considered more restrictive than the “burden of proof” used in routine neurological practice. They do however accommodate differing levels of certainty of diagnosis and their use is encouraged. • The diagnosis should be communicated by a senior doctor, in privacy, with the opportunity • Patients should be accompanied by a relative and/or health professional who would be able • Regular feedback is essential, to ensure patients have understood the information that they have been given [5] and they should be encouraged to express their concerns. • Written information should be available about the disease, the Motor Neurone Disease Association and other follow-up contacts. • The diagnosis should be communicated to the General Practitioner (and family if not present when patient informed of diagnosis) without delay. A co-ordinated multidisciplinary approach is required to meet the rapidly changing physical and psychosocial needs of patients and carers throughout the course of the disease. This should be underpinned by the following principles: • Care encompassing the whole person and those that matter to them. • Prompt provision of treatments to secure symptom control and quality of life. • Planning for the future and timely liaison e.g. with the palliative care team. The maintenance of optimal functional independence and well-being requires a multi-disciplinary • Drooling can be alleviated by anti-cholinergic drugs such as hyoscine (sublingual or transdermal), glycopyrrolate (subcutaneously), atropine (orally), tricyclic antidepressants (such as amitryptiline) and beta-blockers [6]. Non- pharmacological approaches such as salivary gland irradiation and duct ligation have been described. • Dysphagia is best managed in association with speech and language therapists as well as dieticians and, as it progresses, more invasive approaches such as PEG may need to be considered in consultation with patient and carer. Expert opinion favours early PEG [7]. • Speech and language therapists also help in the management of communication difficulties. When a communication aid is needed it is essential that it is provided promptly [8]. • Muscle cramps may be treated by quinine, diazepam, phenytoin and naftidrofuryl. Quinine, diazepam and phenytoin are often usefully given at bedtime. There is considerable individual variation in levels of response to these drugs. If one does not help, others might. • Spasticity can be helped by physiotherapy or drug treatment e.g. baclofen, dantrolene, • The impact of increasing limb weakness may be alleviated by specific interventions ranging from low-tech equipment such as simple splints and neck support collars designed for MND sufferers to environmental control systems and computer access technology [9, 10]. • Respiratory symptoms are distressing for patients and carers. Nocturnal hypoventilation may cause early morning headache and lethargy. There is a strong suggestion that non-invasive respiratory support improves quality of life [11] and should be considered at an early stage with appropriate specialist respiratory management in discussion with patients and carers [12]. Other symptomatic treatments such as opioids can relieve dyspnoea, coughing and choking. The *Breathing Space Kit contains medication which can be used by the carer, nurse or general practitioner for the emergency treatment of the acute episodes of respiratory distress which may occur in the terminal stages [13, 14]. The use of invasive ventilation is a complex issue which, if contemplated, requires early and careful discussion with patients • Depression should be differentiated from the natural sadness of disability. When present it may be treated with an appropriate antidepressant such as amitryptiline or a selective serotonin reuptake inhibiting drug (SSRI). These drugs may also relieve emotional lability and anxiety. Psychiatric advice may also be required. • Pain is common and may be managed at all stages with anti-spasticity drugs, non-steroidal agents and analgesics [16], using the principles of the WHO ladder including opioids such as oral morphine or fentanyl transdermal patches. Significant benefits can be achieved with Reviews of some of these therapies are currently registered with the Cochrane Collaboration []. It is hoped that coverage of this area will become more comprehensive in the future. These comments are based on clinical effectiveness only and do not Riluzole is the only drug currently licensed in the UK for the treatment of MND. Riluzole is a disease-modifying therapy. It does not cause symptomatic improvement and does not prevent death. Patients and carers need to be informed of the implications of the trial data before starting treatment. Evidence for its efficacy comes from two randomised placebo-controlled trials (RCT's) [17,18] see footnote+. The NICE (National Institute for Clinical Excellence) guidance [3] states that “1.1 Riluzole is recommended for the treatment of individuals with the amyotrophic lateral sclerosis (ALS) form of motor neurone disease (MND)” and “1.2 Riluzole therapy should be initiated by a neurological specialist with expertise in the management of MND. Routine supervision of therapy should be managed by locally agreed shared care protocols undertaken by general practitioners”. The Cochrane Systematic Review [19] concluded that “Riluzole 100mg per day is reasonably safe and probably prolongs survival by about two months in patients with ALS. More studies are needed, especially to clarify its effect in older patients (over 75 years) and those with more advanced disease.” Riluzole treatment is appropriately discussed with MND patients as soon as a working diagnosis has been made, although RCT evidence only exists currently for patients with definite or probable MND within the El Escorial Criteria [4]. Patient choice or adverse effects such as fatigue and nausea, may lead to cessation of therapy. Efficacy evidence from placebo-controlled randomised clinical trials only extends to 18 months of therapy. Nonetheless it is reasonable to continue therapy for longer than 18 months when considered appropriate by the clinician, patient and carers. However, it is not appropriate to start patients with advanced disease on riluzole or to prolong treatment into the terminal phase. . • Although there is published evidence from one placebo-controlled RCT [20] that insulin- like nerve growth factor (rhIGF-I) slows disease progression, this was not confirmed in a second RCT [21]. This drug is not licensed. • Many other putative disease-modifying therapies for MND have been investigated but have C Gardner-Thorpe, Consultant Neurologist, Exeter Neurosciences, Royal Devon and Exeter Hospital
and Peninsular Medical School, Barrack Road, EXETER, EX2 5DW: D Jefferson; Consultant
Neurologist, Queen’s Medical Centre, Nottingham, NG7 2UH: PN Leigh; Professor of Neurology,
Institute of Psychiatry, de Crespigny Park, London, SE5 8AF: JD Mitchell; Consultant Neurologist &
Honorary Professor of Clinical Neurology, Royal Preston Hospital, Sharoe Green Lane, Fulwood, PRESTON, PR2 9HT (Chairman): KE Morrison; Professor of Neurology, Medical School,
University of Birmingham, Edgbaston, BIRMINGHAM, B15 2TT: DJ Oliver; Consultant in
Palliative Medicine, Wisdom Hospice, St William Way, Rochester, Kent, ME1 2NU; Honorary Senior Lecturer in Palliative Care, Kent Institute of Medicine and Health Science, University of Kent at Canterbury: RW Orrell; Senior Lecturer and Consultant Neurologist, Royal Free & University
College Medical School, University College, London, NW3 2PF: H Pall; Senior Lecturer in
Neurology, University of Birmingham, B15 2TH: PJ Shaw; Professor of Neurology, E Floor, Medical
School, University of Sheffield, Beech Hill Road, SHEFFIELD, S10 2RX: M Swash; Consultant
Neurologist, The Royal London Hospital, London, E1 1BB: RJ Swingler; Consultant Neurologist,
Ninewells Hospital, DUNDEE, DD9 1SY: E Williams; Consultant in Rehabilitation Medicine,
Walton Centre for Neurology and Neurosurgery, LIVERPOOL, L9 7LJ: CA Young; Consultant
Neurologist, Walton Centre for Neurology and Neurosurgery, LIVERPOOL, L9 7LJ. The UK MND Interest Group is funded by unconditional educational grants from Aventis, the manufacturers of riluzole, and Amgen. Members of the group have accepted speakers honoraria from several pharmaceutical firms, including Aventis, the manufacturer of riluzole. Some members of the group were also investigators in the second large trial of riluzole in MND, but only PNL participated in the data analysis and manuscript preparation. The Association of British Neurologists has received financial support from the pharmaceutical industry including the manufacturers of medications for motor neurone disease. *Obtainable on medical request through the Motor Neurone Disease Association, David Niven House, PO Box 246, NORTHAMPTON, NN I 2PR. (Telephone 01604-250505, fax 01604-24726). Kit includes suggestions for the management of acute respiratory symptoms. It is then stocked with drugs through prescription by the GP. The following contents are suggested: enema (Stesolid) 10mg x 3 (for use by carers) Chlorpromazine 25mg x 3 for injection is suggested as a possible alternative to Midazolam. Glycopyrronium bromide 200µg x 3 is suggested as a possible alternative to Hyoscine +These RCT's report a modest decrease in risk of tracheostomy or death with riluzole (relative risk riluzole 100mg/day= 0.66 (95% confidence interval 0.42-1.02), p = 0.05 over 12 months treatment, [17]; relative risk, riluzole 100mg/day (Cox model) = 0.65 (95% confidence interval 0.50-0.85), p = 0.002 over 18 months treatment, [18]. It has been calculated [22] that the number of patients needed to treat with riluzole 100mg/day to prevent one death or tracheostomy after one year (based on [18]) is 9.2 (95% confidence interval 5.2-38). A post-hoc analysis of the data from the dose-ranging trial [23] has suggested that riluzole delays the progression of the disease to severe disability. The findings of this preliminary study require confirmation. 1. Miller RG, Rosenberg JA, Gelinas DF et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence based review). Neurology 1999; 52: 1311-23 2. Quality standards group of the American Academy of Neurology. Practice advisory on the treatment of amyotrophic lateral sclerosis with riluzole. Neurology 1997; 49: 657-9 3. National Institute for Clinical Excellence. Technology Appraisal Guidance No 20 – Guidance on the use of riluzole (Rilutek) for the treatment of motor neurone disease. 2001 4. World Federation of Neurology Research Group on Neuromuscular Diseases. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 5. Johnston M, Earll L, Mitchell E, Morrison I; Wright S. Communicating the diagnosis of motor neurone disease. Palliative Medicine 1996; 10: 23-34 6. Borasio GD, Oliver D. The control of other symptoms. In; Palliative Care in Amyotrophic Lateral Sclerosis. Oliver D, Borasio GD, Walsh D eds. Oxford, Oxford University Press; 2000: 72- 7. Wagner-Sonntag E, Allison S, Oliver D, Prosiegel M, Rawlings J, Scott A. Dysphagia. In; Palliative Care in Amyotrophic Lateral Sclerosis. Oliver D, Borasio GD, Walsh D eds. Oxford, 8. Scott A, Foulsom M. Speech and Language therapy. In; Palliative Care in Amyotrophic Lateral Sclerosis. Oliver D, Borasio GD, Walsh D eds. Oxford, Oxford University Press; 2000: 9. O’Gorman B. Physiotherapy. In; Palliative Care in Amyotrophic Lateral Sclerosis. Oliver D, Borasio GD, Walsh D eds. Oxford, Oxford University Press; 2000: 10. Kingsnorth C. Occupational Therapy. In; Palliative Care in Amyotrophic Lateral Sclerosis. Oliver D, Borasio GD, Walsh D eds. Oxford, Oxford University Press; 2000: 11. Lyall RA, Donaldson N, Fleming T, Wood C, Newsom-Davis I, Polkey MI, Leigh PN, Moxham J. Prospective study of quality of life in ALS patients treated with non-invasive ventilation. 12. Lyall R, Moxham J, Leigh N. Dyspnoea. In; Palliative Care in Amyotrophic Lateral Sclerosis. Oliver D, Borasio GD, Walsh D eds. Oxford, Oxford University Press; 2000: 13. Oliver D. The quality of care and symptom control – the effects on the terminal phase of ALS/MND. J Neurol Sci 1996; 139 (Suppl): 134-6 14. Sykes N. End of life care in ALS. In; Palliative Care in Amyotrophic Lateral Sclerosis. Oliver D, Borasio GD, Walsh D eds. Oxford, Oxford University Press; 2000: 15. Gelinas D. Amyotrophic lateral sclerosis and invasive ventilation. In; Palliative Care in Amyotrophic Lateral Sclerosis. Oliver D, Borasio GD, Walsh D eds. Oxford, Oxford University 16. Oliver D. Opioid medication in the palliative care of motor neurone disease. Palliative Medicine 17. Bensimon G, Lacomblez L, Meininger Vet al. A controlled trial of riluzole in amyotrophic lateral sclerosis. N Eng J Med 1994; 330: 585-91 18. Lacomblez L, Bensimon G, Leigh PN et al. Dose ranging study of riluzole in amyotrophic lateral sclerosis. Lancet 1996; 37: 1425-31 19. Miller RG, Mitchell JD, Lyon M and Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND) (Cochrane Review). In: The Cochrane Library, Issue 3, 2002. 20. Lai EC, Felice KJ, Festoff BW et al. Effect of recombinant human insulin-like growth factor-1 on progression of ALS. Neurology 1997; 49: 1621-30 21. Borasio G, Robberecht W, Leigh PN et al. A placebo-controlled trial of insulin-like growth factor-1 in amyotrophic lateral sclerosis. Neurology 1998; 51: 583-6 22. Anon. Riluzole. In Therapeutic Drugs, ed. Dollery C. Churchill Livingstone, Edinburgh 1999; 23. Riviere M, Meininger V, Zeisser P, Munsat T. An analysis of extended survival in patients with amyotrophic lateral sclerosis treated with riluzole. Arch Neurol 1998; 55: 526-8



Review Article Juvenile Dermatomyositis: A Review Geetha Chari, MD; Teresita A. Laude, MD, FAAP, FAAD Abstract acute and severe onset. It appears to have a seasonal predi-lection, occurring more frequently in the spring and summerJuvenile dermatomyositis is a systemic vasculopathy, affectingmonths. A history of antecedent illness is often obtained inmainly the skin and muscle. In the U

Microsoft word - mentor application 08 _2_.doc

Name: __________________________________________________ Street Address: ___________________________________________ City: _____________________________ Zip:___________________ Daytime Phone: _______________ Evening Phone: ___________ Email Address:____________________________________________ Highest Level of Education Completed: Current College students please fill out the following sectio

Copyright © 2010 Medicament Inoculation Pdf