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(BQ) Part 1 book Oh''s intensive care manual has contents: Organisation aspects, palliative care, multiple organ dysfunction syndrome, haemodynamic monitoring, adult cardiopulmonary resuscitation, acute coronary care,.... and other contents.
OH’S INTENSIVE CARE MANUAL SEVENTH EDITION Content Strategist: Michael Houston Content Development Specialist: Nani Clansey Content Coordinator: Sam Crowe, Humayra Rahman Project Manager: Umarani Natarajan Design: Miles Hitchen Illustration Manager: Jennifer Rose Illustrator: Kinesis Illustration Marketing Manager: Abigail Swartz Seventh Edition OH’S INTENSIVE CARE MANUAL Edited by Andrew D Bersten MB BS MD FCICM Director, Intensive Care Unit, Flinders Medical Centre Professor and Head, Department of Critical Care Medicine Flinders University Adelaide, SA, Australia Neil Soni MB ChB MD FANZCA FRCA FCICM FFICM Consultant in Intensive Care Chelsea and Westminster Hospital Honorary Senior Lecturer Imperial College Medical School London, United Kingdom For additional online content visit expertconsult.com © 2014 Elsevier Ltd All rights reserved First edition 1979 Second edition 1985 Third edition 1990 Fourth edition 1997 Fifth edition 2003 Sixth edition 2009 Seventh edition 2014 The right of Andrew D Bersten and Neil Soni to be identified as authors of this work has been asserted by them in accordance with the Copyright, Designs and Patents Act 1988 No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein) ISBN: 978-0-7020-4762-6 Ebook ISBN: 978-1-4557-5013-9 British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Cataloging in Publication Data A catalog record for this book is available from the Library of Congress Notices Knowledge and best practice in this field are constantly changing As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein The publisher’s policy is to use paper manufactured from sustainable forests Printed in China Last digit is the print number:â•… 9â•… 8â•… 7â•… 6â•… 5â•… 4â•… 3â•… 2â•… Contents List of Contributorsâ•… xiii Prefacê•… xxi Acknowledgementsâ•… xxii Part One – Organisation Aspects Design and organisation of intensive care unitsâ•… Vineet V Sarode and Felicity H Hawker Critical care outreach and rapid response systemsâ•… 10 John R Welch and Christian P Subbe Severity of illness and likely outcome from critical illnessâ•… 16 Mark Palazzo Transport of critically ill patientsâ•… 27 Evan R Everest and Matthew R Hooper Physiotherapy in intensive carê•… 38 Fiona H Moffatt and Mandy O Jones Critical care nursingâ•… 47 John R Welch Ethics in intensive carê•… 55 Raymond F Raper and Malcolm M Fisher Common problems after ICUâ•… 61 Carl S Waldmann and Evelyn Corner Clinical information systemsâ•… 69 David Fraenkel 10 Clinical trials in critical carê•… 75 Simon Finfer and Anthony Delaney 11 Palliative carê•… 85 Sarah Cox and Neil Soni 12 ICU and the elderlyâ•… 90 Richard Keays 13 Health care team in intensive care medicinê•… 98 Gerry O’Callaghan 14 Preparing for examinations in intensive care medicinê•… 107 Carole Foot and Liz Hickson Part Two – Shock 15 Overview of shockâ•… 115 Matthew J Maiden and Sandra L Peake vi Contents 16 Haemodynamic monitoringâ•… 122 David J Sturgess 17 Multiple organ dysfunction syndromê•… 138 Matthew J Maiden and Marriane J Chapman 18 Monitoring oxygenationâ•… 146 Thomas J Morgan and Balasubramanian Venkatesh 19 Lactic acidosisâ•… 158 D James Cooper, Alisa M Higgins and Alistair D Nichol Part Three – Acute Coronary Care 20 Acute cardiac syndromes, investigations and interventionsâ•… 167 Bradley Power 21 Adult cardiopulmonary resuscitationâ•… 191 Peter T Morley 22 Management of cardiac arrhythmiasâ•… 200 Andrew Holt 23 Cardiac pacing and implantable cardioverter defibrillatorsâ•… 260 Aaisha Opel and Oliver R Segal 24 Acute heart failurê•… 271 Nicholas Ioannou, Pratik Sinha and David Treacher 25 Valvular and congenital heart disease and bacterial endocarditisâ•… 289 Mary White and Susanna Price 26 Intensive care after cardiac surgeryâ•… 299 Raymond F Raper 27 Echocardiography in the intensive care unitâ•… 308 Guido Tavazzi and Susanna Price Part Four – Respiratory Failure 28 Oxygen therapyâ•… 327 Adrian J Wagstaff 29 Airway management and acute airway obstructionâ•… 341 Gavin M Joynt and Gordon YS Choi 30 Acute respiratory failure in chronic obstructive pulmonary diseasê•… 354 Matthew T Naughton and David V Tuxen 31 Mechanical ventilationâ•… 364 Andrew D Bersten 32 Humidification and inhalation therapyâ•… 375 Steven T Galluccio and Andrew D Bersten 33 Acute respiratory distress syndromê•… 382 Andrew D Bersten 34 Pulmonary embolismâ•… 392 Andrew R Davies and David V Pilcher 35 Acute severe asthmâ•… 401 David V Tuxen and Matthew T Naughton Contents 36 Pneumoniâ•… 414 Kai Man Chan and Charles D Gomersall 37 Non-invasive ventilationâ•… 429 Graeme J Duke and Andrew D Bersten 38 Respiratory monitoringâ•… 436 Andrew D Bersten 39 Imaging the chestâ•… 445 Simon PG Padley 40 Ultrasound in the ICUâ•… 461 Ubbo F Wiersema 41 Extracorporeal membrane oxygenation (ECMO)â•… 472 Vincent Pellegrino 41.1â•… ECMO for respiratory failurê•… 472 41.2â•… ECMO for cardiac failurê•… 479 Part Five – Gastroenterological Emergencies and Surgery 42 Acute gastrointestinal bleedingâ•… 487 Joseph JY Sung 43 Severe acute pancreatitisâ•… 495 Duncan LA Wyncoll 44 Liver failurê•… 501 Christopher Willars and Julia Wendon 44.1â•… Acute hepatic failurê•… 501 44.2â•… Cirrhosis and acute-on-chronic liver diseasê•… 512 45 Abdominal surgical catastrophesâ•… 520 Stephen J Streat 46 Solid tumours and their implications in the ICUâ•… 526 Timothy Wigmore and Pascale Gruber Part Six – Acute Renal Failure 47 Acute kidney injuryâ•… 535 Rinaldo Bellomo 48 Renal replacement therapyâ•… 540 Rinaldo Bellomo Part Seven – Neurological Disorders 49 Disorders of consciousnessâ•… 549 Balasubramanian Venkatesh 50 Status epilepticusâ•… 560 Helen I Opdam 51 Acute cerebrovascular complicationsâ•… 568 Bernard Riley and Thearina de Beer vii viii Contents 52 Cerebral protectionâ•… 580 Victoria Heaviside and Michelle Hayes 53 Brain deathâ•… 591 Martin Smith 54 Meningitis and encephalomyelitisâ•… 597 Angus M Kennedy 55 Tetanusâ•… 607 Jeffrey Lipman 56 Deliriumâ•… 611 Timothy M Alce, Valerie Page and Marcela P Vizcaychipi 57 Neuromuscular diseases in intensive carê•… 617 George Skowronski and Manoj K Saxena Part Eight – Endocrine Disorders 58 Diabetic emergenciesâ•… 629 Richard Keays 59 Diabetes insipidus and other polyuric syndromesâ•… 637 Alastair C Carr 60 Thyroid emergenciesâ•… 652 Jonathan M Handy and Alexander M Man Ying Li 61 Adrenocortical insufficiency in critical illnessâ•… 660 Balasubramanian Venkatesh and Jeremy Cohen 62 Acute calcium disordersâ•… 666 Balasubramanian Venkatesh Part Nine – Obstetric Emergencies 63 Preeclampsia and eclampsiâ•… 677 Wai Ka Ming and Tony Gin 64 General obstetric emergenciesâ•… 684 Winnie TP Wan and Tony Gin 65 Severe pre-existing disease in pregnancyâ•… 692 Jeremy P Campbell and Steve M Yentis Part Ten – Infections and Immune Disorders 66 Anaphylaxisâ•… 699 Malcolm M Fisher 67 Host defence mechanisms and immunodeficiency disordersâ•… 703 Steven McGloughlin and Alexander A Padiglione 68 HIV and acquired immunodeficiency syndromê•… 710 Alexander A Padiglione and Steve McGloughlin 69 Severe sepsisâ•… 716 A Raffaele De Gaudio 70 Nosocomial infectionsâ•… 724 James Hatcher and Rishi H-P Dhillon Contents 71 Severe soft-tissue infectionsõ 733 Ilker Uỗkay, Hugo Sax, Pierre Hoffmeyer, Daniel Lew and Didier Pittet 72 Principles of antibiotic usê•… 738 Jeffrey Lipman 73 Tropical diseasesâ•… 743 Ramachandran Sivakumar and Michael E Pelly ix Part Eleven – Severe and Multiple Trauma 74 Severe and multiple traumâ•… 755 James A Judson and Li C Hsee 75 Severe head injuriesâ•… 762 John A Myburgh and Manoj K Saxena 76 Faciomaxillary and upper-airway injuriesâ•… 777 Cyrus Edibam and Hayley Robinson 77 Chest injuriesâ•… 784 Ubbo F Wiersema 78 Spinal injuriesâ•… 795 Sumesh Arora and Oliver J Flower 79 Abdominal and pelvic injuriesâ•… 807 Colin McArthur and Pieter HW Lubbert Part Twelve – Environmental Injuries 80 Submersionâ•… 817 Cyrus Edibam and Tim Bowles 81 Burnsâ•… 821 David P Mackie and Jacqueline EHM Vet 82 Thermal disordersâ•… 829 Stephen W Lam and Richard Strickland 83 Electrical safety and injuriesâ•… 844 Lester AH Critchley 84 Envenomationâ•… 851 James Tibballs 85 Ballistic injuryâ•… 861 Michael C Reade and Peter D (Toby) Thomas 86 Background information on ‘biochemical terrorism’â•… 871 Munita Grover and Michael Pelly Part Thirteen – Pharmacologic Considerations 87 Pharmacokinetics, pharmacodynamics and drug monitoring in critical illnessâ•… 881 Christine Chung 88 Management of acute poisoningâ•… 892 David M Wood and Duncan LA Wyncoll 89 Sedation and pain management in intensive carê•… 903 Luke E Torre References 616.e1 REFERENCES American Psychiatric Association Diagnostic and Statistical Manual of Mental Disorders DSM-IV-TR, 4th ed (Text Revision) Washington DC: American Psychiatric Association; 2000 Page VJ, Navarange S, Gama S, et al Routine delir ium monitoring in a UK intensive care unit Crit Care 2009;13:R16 Ely EW, Shintani A, Truman B, et al Delirium as a predictor of mortality in mechanically ventilated patients in the intensive care unit JAMA 2004;291: 1753–62 McCusker J, Cole MG, Voyer P, et al Prevalence and incidence of delirium in long-term care Int J Geriatr Psychiatry 2011;26:1152–61 Milbrandt EB, Deppen S, Harrison PL, et al Costs associated with delirium in mechanically ventilated patients Crit Care Med 2004;32:955–62 Lemstra AW, Groen in’t Woud JC, Hoozemans JJ, et al Microglia activation in sepsis: a case-control study J Neuroinflammation 2007;4:4 Maclullich AM, Ferguson KJ, Miller T, et al Unravel ling the pathophysiology of delirium: a focus on the role of aberrant stress responses J Psychosom Res 2008;65:229–38 Field RH, Gossen A, Cunningham C Prior pathology in the basal forebrain cholinergic system predisposes to inflammation-induced working memory deficits: reconciling inflammatory and cholinergic hypothe ses of delirium J Neurosci 2012;32:6288–94 Trzepacz PT Update on the neuropathogenesis of delirium Dement Geriatr Cogn Disord 1999;10: 330–4 10 Leung JM, Sands LP, Wang Y, et al Apolipoprotein E e4 allele increases the risk of early postoperative delirium in older patients undergoing noncardiac surgery Anesthesiology 2007;107:406–11 11 Abelha FJ, Fernandes V, Botelho M, et al Apolipo protein E e4 allele does not increase the risk of early postoperative delirium after major surgery J Anesth 2012;26:412–21 12 Ely EW, Margolin R, Francis J, et al Evaluation of delirium in critically ill patients: validation of the Confusion Assessment Method for the Intensive Care Unit (CAM-ICU) Crit Care Med 2001;29: 1370–9 13 van den Boogaard M, Pickkers P, Slooter AJ, et al Development and validation of PRE-DELIRIC (PRE diction of DELIRium in ICu patients) delirium pre diction model for intensive care patients: observational multicentre study BMJ 2012;344:e420 14 Spronk PE, Riekerk B, Hofhuis J, et al Occurrence of delirium is severely underestimated in the ICU during daily care Intensive Care Med 2009;35: 1276–80 15 Bergeron N, Dubois MJ, Dumont M, et al Intensive Care Delirium Screening Checklist: evaluation of a new screening tool Intensive Care Med 2001;27: 859–64 16 Luetz A, Heymann A, Radtke FM, et al Different assessment tools for intensive care unit delirium: which score to use? Crit Care Med 2010;38:409–18 17 van Eijk MM, van den Boogaard M, van Marum RJ, et al Routine use of the confusion assessment method for the intensive care unit: a multicenter study Am J Respir Crit Care Med 2011;184:340–4 18 Inouye SK, Bogardus ST Jr, Charpentier PA, et al A multicomponent intervention to prevent delirium in hospitalized older patients N Engl J Med 1999;340: 669–76 19 Agarwal V, O’Neill PJ, Cotton BA, et al Prevalence and risk factors for development of delirium in burn intensive care unit patients J Burn Care Res 2010;31: 706–15 20 Skrobik Y, Ahern S, Leblanc M, et al Protocolized intensive care unit management of analgesia, seda tion, and delirium improves analgesia and subsyn dromal delirium rates Anesth Analg 2010;111: 451–63 21 Girard TD, Kress JP, Fuchs BD, et al Efficacy and safety of a paired sedation and ventilator weaning protocol for mechanically ventilated patients in intensive care (Awakening and Breathing Con trolled trial): a randomised controlled trial Lancet 2008;371:126–34 22 Shehabi Y, Bellomo R, Reade MC, et al; Sedation Practice in Intensive Care Evaluation (SPICE) Study Investigators and the ANZICS Clinical Trials Group Early intensive care sedation predicts long-term mor tality in ventilated critically ill patients Am J Respir Crit Care Med 2012;186:724–31 23 Naughton BJ, Saltzman S, Ramadan F, et al A mul tifactorial intervention to reduce prevalence of delir ium and shorten hospital length of stay J Am Geriatr Soc 2005;53:18–23 24 Wang W, Li HL, Wang DX, et al Haloperidol proph ylaxis decreases delirium incidence in elderly patients after noncardiac surgery: a randomized controlled trial Crit Care Med 2012;40:731–9 25 Kalisvaart KJ, de Jonghe JF, Bogaards MJ, et al Haloperidol prophylaxis for elderly hip-surgery patients at risk for delirium: a randomized placebocontrolled study J Am Geriatr Soc 2005;53:1658–66 26 Skrobik YK, Bergeron N, Dumont M, et al Olanzap ine vs haloperidol: treating delirium in a critical care setting Intensive Care Med 2004;30:444–9 27 Devlin JW, Roberts RJ, Fong JJ, et al Efficacy and safety of quetiapine in critically ill patients with delirium: a prospective, multicenter, randomized, double-blind, placebo-controlled pilot study Crit Care Med 2010;38:419–27 28 Kim SW, Yoo JA, Lee SY, et al Risperidone versus olanzapine for the treatment of delirium Hum Psy chopharmacol 2010;25:298–302 29 Hoy SM, Keating GM Dexmedetomidine: a review of its use for sedation in mechanically ventilated patients in an intensive care setting and for proce dural sedation Drugs 2011;71:1481–501 616.e2 Delirium 30 van Eijk MM, Roes KC, Honing ML, et al Effect of rivastigmine as an adjunct to usual care with haloperidol on duration of delirium and mortality in critically ill patients: a multicentre, double-blind, placebo-controlled randomised trial Lancet 2010; 376:1829–37 31 Lonergan E, Luxenberg J, Areosa Sastre A Benzodi azepines for delirium Cochrane Database Syst Rev 2009;4:CD006379 57 Neuromuscular diseases in intensive care George Skowronski and Manoj K Saxena A number of disorders producing generalised neuromuscular weakness can require admission to the ICU, or complicate the course of ICU patients These may involve: anterior horn cells: motor neuron (or neurone) • spinal disease, poliomyelitis peripheral nerve conduction: Guillain–Barré syn• drome (GBS) and related disorders the neuromuscular junction: myasthenia gravis, • botulism contraction: myopathies, periodic paralysis • muscle mixed disorders: intensive care acquired weakness • Box 57.1 lists a differential diagnosis of muscle weakness in critically ill patients GUILLAIN–BARRÉ SYNDROME AND RELATED DISORDERS In 1834 James Wardrop reported a case of ascending sensory loss and weakness in a 35-year-old man, leading to almost complete quadriparesis over 10 days, and complete recovery over several months.1 In 1859, Landry described an acute ascending paralysis occurring in 10 patients, of whom died Guillain, Barré and Strohl in 19162 reported two cases of motor weakness, paraesthesiae and muscle tenderness in association with increased protein in the cerebrospinal fluid – lumbar puncture for cerebrospinal fluid (CSF) examination was first described only in the 1890s The many variants of this syndrome and the lack of specific diagnostic criteria have previously resulted in confusion in nomenclature More recently clinical, electrical and laboratory criteria for the predominant variant – acute inflammatory demyelinating polyÂ� radiculopathy (AIDP) – are well described,3 though 10–15% of cases not fit these criteria GBS is best regarded as a heterogeneous group of immunologically mediated disorders of peripheral nerve function INCIDENCE Since the incidence of poliomyelitis has markedly declined due to mass immunisation programmes, GBS has become the major cause of rapid-onset flaccid paralysis in previously healthy people, with an incidence of approximately 1.7 per 100â•›000.4 Epidemics have occurred in large populations exposed to viral illness or immunisation but immunosuppression and concurrent autoimmune disease may also be predisposing factors.5–7 The disorder is commoner in males, and up to times commoner in the elderly No consistent seasonal or racial predilection has been demonstrated.4 AETIOLOGY Most recent evidence supports the proposition that GBS is caused by immunologically mediated nerve injury.8 Cell-mediated immunity, in particular, probably plays a significant role, and inflammatory cell infiltrates are often seen in association with demyelination, which is generally regarded as the primary pathological process Antibodies to a number of nervous system components have been demonstrated in GBS patients, with most interest in recent years focusing on anti-ganglioside antibodies The precise mechanism of sensitisation is not known, but clinical associations suggest that antecedent infections or immunisations are commonly involved and two-thirds of cases are preceded by symptoms suggestive of respiratory or gastrointestinal infection Infective agents implicated include influenza A, parainfluenza, varicella-zoster, Epstein–Barr, chickenpox, mumps, human immunodeficiency virus (HIV),9 measles virus and Mycoplasma Campylobacter jejuni gastroenteritis now appears to be the most common predisposing infection and may be associated with a more severe clinical course; 26–41% of GBS patients show evidence of recent C jejuni infection.10 Cytomegalovirus infection accounts for a further 10–22% of cases.11 Immunisations against viral infections, tuberculosis, tetanus12 and typhoid have all been reported to be associated with the onset of GBS, but most of these reports are anecdotal and of questionable aetiological significance; 65% of patients present within a few weeks of minor respiratory (43%) or gastrointestinal (21%) illness Surveillance data following the 2009 H1N1 influenza epidemic suggested that the risk of GBS following immunisation was only slightly increased over baseline.13 618 Neuromuscular diseases in intensive care Box 57.1 Differential diagnosis of muscle weakness in critically ill patients Brainstem Lower pontine hemorrhage or infarction (locked-in state) Spinal cord Transverse myelitis Compression by tumour, abscess, or haemorrhage Carcinomatous or lymphomatous meningitis Peripheral nerve Intensive care unit acquired neuropathy/neuromyopathy Phrenic nerve injury during thoracic surgery Guillain–Barré syndrome Ingested toxins, including arsenic, thallium, cyanide Neuromuscular junction Delayed reversal of neuromuscular blockade Myasthenia gravis Lambert–Eaton syndrome Botulism Pesticide poisoning Skeletal muscle Acute necrotising myopathy Steroid myopathy Severe hypokalaemia, hypophosphataemia, and/or hypomagnesaemia Acute alcoholic myopathy Polymyositis or dermatomyositis Toxic myopathy (colchicine, lovastatin, cocaine, bumetanide, amiodarone and others) Intensive care unit acquired myopathy/neuromyopathy Adapted with permission from Hansen-Flaschen J Neuromuscular disorders of critical illness UpToDate 2006; Ver 14.393 PATHOGENESIS The peripheral nerves of patients who have died of GBS show infiltration of the endoneurium by mononuclear cells, in a predominantly perivenular distribution The inflammatory process may be distributed throughout the length of the nerves, but with more marked focal changes in the nerve roots, spinal nerves and major plexuses Electron micrographs show macrophages actively stripping myelin from the bodies of Schwann cells and axons In some cases, Wallerian degeneration of axons is also seen, and failure of regeneration in these cases may correspond with a poor clinical outcome The underlying immune response is complex and poorly understood, but serum from GBS patients produces myelin damage in vitro when complement is present.14 Although antibodies to various glycolipids have been demonstrated in GBS, these are generally in low titre and can occasionally be seen in controls Patients with recent C jejuni infection have a high incidence of antibodies to the ganglioside GM1.10 Antibodies to GD1a and GQ1b gangliosides are associated with the rarer AMAN and AMSAN variants (see below).15 The basis of the effectiveness of plasma exchange and immunoglobulin therapy is likely to be blocking of demyelinating antibodies by several mechanisms.16 CLINICAL PRESENTATION The majority of patients describe a minor illness in the weeks prior to presentation, with a peak incidence weeks beforehand Approximately half the patients initially experience paraesthesiae, typically beginning in the hands and feet One-quarter complain of motor weakness, and the remainder have both.8 Motor weakness proceeds to flaccid paralysis, which becomes the predominant complaint Objective loss of power and reduction or loss of tendon reflexes usually commence distally and ascend, but a more haphazard spread may occur Cranial nerves are involved in 45% of cases, most commonly the facial nerve, followed by the glossopharyngeal and vagus nerves One-third of patients require ventilatory support In the Miller–Fisher syndrome, a variant of GBS,17 cranial nerve abnormalities predominate, with ataxia, areflexia and ophthalmoplegia as the main features This is strongly associated with recent C jejuni infection and with the presence of GQ1b antibodies Another subgroup of patients presents with a primarily axonal neuropathy – acute motor–sensory axonal neuropathy (AMSAN) In these cases motor and sensory axons appear to be the primary targets of immune attack, rather than myelin These patients have a more fulminant and severe course, and there is again a strong association with C jejuni infection.18 In typical GBS, sensory loss is generally mild, with paraesthesiae or loss of vibration and proprioception, but occasionally sensory loss, pain or hyperaesthesia can be prominent features Autonomic dysfunction is common, and a major contributor to morbidity and mortality in ventilator-dependent cases.19 Orthostatic or persistent hypotension, paroxysmal hypertension and bradycardia are all described, as are fatal ventricular tachyarrhythmias Sinus tachycardia is seen in 30% of cases Paralytic ileus, urinary retention and abnormalities of sweating are also commonly seen DIFFERENTIAL DIAGNOSIS Most of the important alternative diagnoses are listed in Box 57.2 In patients with prolonged illness, the possibility of chronic inflammatory demyelinating polyÂ� radiculopathy (CIDP) should be considered.20 In this condition, preceding viral infection is uncommon, the onset is more insidious and the course is one of slow worsening or stepwise relapses Corticosteroids and plasma exchange are possibly effective in this disorder, but adequate studies of immunosuppressive drugs have not been carried out An intermediate subacute polyradiculopathy (SIDP) as well as a recurrent form of GBS are also described, and all of these variants may be part of the spectrum of a single condition However, a purely motor axonal neuropathy (acute motor–axonal neuropathy, AMAN), which causes seasonal childhood epidemics mimicking classical GBS in China and elsewhere,21 appears to be a distinct entity Once again, this is strongly associated with C jejuni infection Guillain–Barré syndrome and related disorders Box 57.2 Diagnostic criteria for typical Guillain–Barré syndrome3 Features required for diagnosis Progressive weakness in both arms and both legs Areflexia Features strongly supportive of the diagnosis Progression over days to weeks Relative symmetry of symptoms Mild sensory symptoms or signs Cranial nerve involvement, especially bilateral weakness of facial muscles Recovery beginning 2–4 weeks after progression ceases Autonomic dysfunction Absence of fever at onset High concentration of protein in cerebrospinal fluid protein, with fewer than 10õìõ106 cells/L Typical electrodiagnostic features Features excluding diagnosis Diagnosis of botulism, myasthenia, poliomyelitis or toxic neuropathy Abnormal porphyrin metabolism Recent diphtheria History or evidence of lead intoxication Purely sensory syndrome, without weakness INVESTIGATIONS In over 90% of patients, CSF protein is increased (greater than 0.4╯g/L), within weeks of onset of symptoms The level does not correlate with the clinical findings A pleocytosis with lymphocytes and monocytes in the CSF may be seen in a small proportion of patients, especially later in the disease Nerve conduction studies typically demonstrate reduced conduction velocity and prolonged distal latencies,22 but there is no consensus on precise electrophysiological criteria for the various subtypes.22 Severely reduced distal motor amplitude and a predominantly axonal pattern are associated with more severe disease and a guarded prognosis MANAGEMENT Although the management of the patient with severe and protracted GBS provides a major challenge, the prognosis is generally good if complications can be treated early or avoided SPECIFIC THERAPY Plasma exchange (plasmapheresis) is of value in GBS and in two trials a reduction in patients requiring mechanical ventilation, reduced duration of mechanical ventilation for those who required it, reduced time to motor recovery and time to walking without assistance were demonstrated.23,24 Mortality, however, was not altered Plasma exchange was most effective when carried out within days of onset of symptoms The currently recommended plasma exchange schedules consist of four exchanges of 1–2 plasma volumes each, over 1–2 weeks.25 Adverse events are common, and 619 some relate to the disease itself.26 Fresh frozen plasma is reported to have more side-effects than albumin as the replacement fluid.26 Immunoglobulin therapy was as effective as plasmapheresis27 and previous concerns of higher recurrence rates are probably unfounded Because of its ease of use, many authorities now advocate immunoglobulin as the treatment of choice.28 A dose of 2╯g/kg body weight intravenously, over 2–5 days, is the current recommendation.29 About 10% of patients relapse after initial treatment with either plasmapheresis or immunoglobulin; most respond well to a further course There appears to be no benefit in combining plasmapheresis and immunoglobulin treatments, or in crossing over from one to the other.30 A Cochrane review confirms that low- or high-dose corticosteroids are of no value,31 and may even slow recovery The combination of high-dose steroids with immunoglobulin does not affect the long-term outcome.32 SUPPORTIVE CARE RESPIRATORY In the spontaneously breathing patient, chest physiotherapy and careful monitoring of respiratory function are of paramount importance Regular measurement of vital capacity is probably the best way to predict respiratory failure, and is more reliable than arterial blood gases.33 The latter nevertheless remain a useful guide Any patient with a vital capacity less than 15╯mL/kg or 30% of the predicted level, or a rising arterial PCO2 is likely to require mechanical ventilation Bulbar involvement should be carefully sought, as there is a significant risk of aspiration of upper airway secretions, gastric contents or ingested food The cough reflex may be inadequate, and airway protection by tracheal intubation or tracheostomy is then required Oral feeding should be stopped in any patient in whom bulbar involvement is suspected Mechanical ventilation is mandatory if coughing is inadequate, pulmonary collapse or consolidation develop, arterial blood gases are significantly abnormal, vital capacity is less than predicted tidal volume (approximately 10╯mL/kg), or the patient is dyspnoeic, tachypnoeic or appears exhausted Mechanical ventilation, if necessary, will probably be required for several weeks (although there is wide variation), and early tracheostomy should be considered CARDIOVASCULAR Cardiac rhythm and blood pressure should be monitored Sinus tachycardia is the commonest autonomic manifestation of GBS and usually requires no active treatment Induction of anaesthesia appears particularly likely to induce serious arrhythmias Use of suxamethonium may contribute significantly to this34 and, as with many other neuromuscular disorders, should be avoided Endotracheal suctioning has also been associated with serious arrhythmias Cardiovascular 620 Neuromuscular diseases in intensive care instability may also be exacerbated by a number of other drugs (Box 57.3) These, likewise, should be avoided or used with great care Mild hypotension and bradycardia may require no treatment, particularly if renal and cerebral functions are maintained However, blood volume expansion or inotropic drugs may be required in some cases Hypertension is often transient, but occasionally requires appropriate drug therapy Hypoxia, hypercarbia, pain and visceral distension should be excluded as causes FLUIDS, ELECTROLYTES AND NUTRITION Paralytic ileus is not uncommon, especially immediately following the institution of mechanical ventilation, and a period of parenteral nutrition may be required However, wherever possible, nasoenteric feeding should be instituted because of its significantly greater safety Energy and fluid requirements are considerably reduced in these patients SEDATION AND ANALGESIA In non-ventilated patients, sedation should be avoided because of the potential for worsening respiratory and upper airway function In ventilated patients, sedation becomes less necessary as the patient becomes accustomed to the ventilator, but night sedation may help to preserve diurnal rhythms Limb pain, particularly with passive movement, is very common and often quite severe Quinine, minor and non-steroidal analgesics and antidepressant drugs may all be tried, but the pain can be difficult to control and opioids are often required Methadone, transdermal fentanyl, gabapentin and tramadol have all been advocated GENERAL AND NURSING CARE A comprehensive programme of physiotherapy should be implemented by nurses and physiotherapists, with Box 57.3 Drugs associated with cardiovascular instability in Guillain–Barré syndrome94 Exaggerated hypotensive response Phentolamine Nitroglycerin Edrophonium Thiopentone Morphine Furosemide Exaggerated hypertensive response Phenylephrine Ephedrine Dopamine Isoprenaline Arrhythmias Suxamethonium Cardiac arrest General anaesthesia Modified from Dalos et╯al,94 with permission careful attention to pressure area care, the maintenance of joint mobility and pulmonary function Nosocomial infection should be actively sought with culture of urine and respiratory secretions at least twice weekly Sites of vascular access should be inspected frequently, and changed whenever necessary It may be possible to manage stable long-term patients without venous access Care should be taken to prevent corneal ulceration and faecal impaction Prophylaxis against venous thromboembolism should be given, and enterally administered low-dose warfarin may be preferable to twice-daily heparin injections in long-stay patients Psychological problems, especially depression, are common, and some patients are helped by antidepressant drugs Good communication and rapport between the patient and staff, involvement of allied health practitioners, the provision of television, radio and reading aids and, where possible, occasional trips out of the ICU are all of great value PROGNOSIS The nadir of the disease is reached within 2–4 weeks, and gradual resolution follows over weeks to months Of those who survive the acute illness, 70% are fully recovered within year, and a further 20% are left with only minor limitation Poor prognostic features35 include age over 60 years, rapid progression to quadriparesis in less than days, the need for mechanical ventilation (except for children),36 and a preceding diarrhoeal illness.37 Even in patients ventilated for more than months, gradual improvement may continue for 18 months to years.38 These severely affected patients require a protracted period of rehabilitation Death in up to 25% of GBS patients has been reported in those requiring intensive care.39 Many of these deaths were due to potentially avoidable problems such as respiratory arrest, ventilator malfunction and intercurrent sepsis, and considerably better results have been achieved.23 A more representative estimate of the overall mortality is 5–8%.35 WEAKNESS SYNDROMES COMPLICATING CRITICAL ILLNESS 40,41 A number of neuromuscular disorders specifically associated with critical illness have been described over the last 30 years They are probably much more common than previously appreciated and may occur in up to 46% (95% CI 43–49%) of patients who require prolonged mechanical ventilation, have sepsis, or multiorgan failure.42 These disorders include neuropathies, myopathies and combinations of both Variations in nomenclature, the lack of a pragmatic, simple diagnostic test, and confusion with other disorders, such as GBS and corticosteroid-induced myopathy, have further complicated this area There is also considerable overlap among the various subtypes Sepsis, neuromuscular- Weakness syndromes complicating critical illness blocking agents (NMBA), disuse atrophy, asthma, corticosteroids and the multiple organ dysfunction syndrome (MODS) have all been implicated Intensive care unit acquired weakness (ICUAW) is now the preferred term used to encompass this broad group of heterogeneous disorders and is potentially classifiable (based on electrophysiology and tissue biopsy) into the subcategories of critical illness neuropathy, myopathy and neuromyopathy.43 The diagnosis of ICUAW requires the clinical context of an acute process of high illness severity often requiring prolonged organ support, and is usually associated with a period of protracted immobilisation Key clinical signs that support a diagnosis of ICUAW include the presence of normal cognition and consciousness, sparing of the cranial nerves, and the presence of symmetrical flaccid weakness A common feature of ICUAW is the involvement of the respiratory musculature, which results in rapid shallow breathing with reduced clearance of respiratory secretions Although two subgroups of ICUAW are outlined below, a number of rarer variants have also been described CRITICAL ILLNESS POLYNEUROPATHY This acute, diffuse, mainly motor neuropathy is probably the commonest of these disorders It usually presents in the recovery phase of a severe systemic illness with persistent quadriparetic weakness, hyporeflexia and difficulty in weaning from respiratory support There appears to be a specific association with severe sepsis and MODS Histological and electrophysiological features are consistent with axonal degeneration The mortality in this group is high, presumably reflecting that of the underlying condition CRITICAL ILLNESS MYOPATHY This disorder is linked with asthma and with the use of corticosteroids, NMBA and, less convincingly, aminoglycosides and beta-adrenergic agonists Reflexes are preserved except in severe cases, as is sensation Elevated blood CPK concentrations are often seen A few patients have a more severe, fulminant form with very high CPK levels, frank rhabdomyolysis and, rarely, renal failure Electrophysiological findings are somewhat variable, though muscle necrosis is usually apparent on histology Although steroidal muscle relaxants (pancuronium or vecuronium)44 have been particularly implicated, the disorder has also been seen with other types of NMBA DIFFERENTIAL DIAGNOSIS (BOX 57.4) The influence of drugs (particularly anxiolytics and analgesics), metabolic abnormalities (electrolyte abnormalities, altered renal and hepatic function) and 621 hypothermia should always be excluded when unexplained neuromuscular weakness appears in an intensive care patient The possibility of a coincident illness such as the Eaton–Lambert syndrome, myasthenia gravis, vasculitis or GBS must also be carefully considered Severe catabolism and disuse atrophy are common in many of these patients and can themselves result in significant weakness MANAGEMENT No specific therapies are available, but minimisation of corticosteroids, sedatives and NMBA, with the aim of maintaining an awake, communicative patient, may be beneficial Patient evaluation ideally should include daily grading of muscle strength In a cooperative patient this may be performed using the Medical Research Council (MRC) score, which grades strength from to in three functional muscle groups in each limb This gives a sum score that ranges from (paralysis) to 60 (full strength), with ICUAW defined as a score of less than 48.43,45 Ancillary investigations (nerve conduction, electromyogram and tissue biopsy) assist with the classification of ICUAW, but remain primarily research tools because of technical difficulties and the lack of a specific therapeutic intervention PROGNOSIS The prognosis is generally good providing the underlying acute and chronic disorders can be addressed The influence of these factors is reflected in the variable extent and duration of recovery (from a few weeks to several months, with some symptoms and signs persisting long term) ICUAW has been associated with prolonged weaning from mechanical ventilation, increased duration of intensive care and hospital stay.46 Longterm associations include increased physical disability and increased mortality,47 but it remains difficult to Box 57.4 Clinical features suggesting intensive care unit acquired weakness Onset of weakness is after the acute presentation Clinical context includes acute, severe illness requiring either prolonged mechanical ventilation, or sepsis and multiorgan support Exclude direct effects of sedation or neuromuscular blockade Normal cognition presence of flaccid, symmetrical motor weakness (with muscle wasting) affecting limbs and respiratory muscles, but sparing cranial nerves Reflexes are absent if a primarily neuropathic pathology, or reduced/absent if primarily a myopathic pathology Sensation may be affected with primarily neuropathic lesions Muscle strength grade (using Medical Research Council sum score – see text) 750╯mg/day) and a preoperative vital capacity of less than 2.9 litres.75 In those cases requiring mechanical ventilation, some authors advocate temporary cessation of anticholinesterase drugs to reduce respiratory secretions,76 but generally they should be continued, though dosage requirements must be reassessed carefully and repeatedly MOTOR NEURON DISEASE (AMYOTROPHIC LATERAL SCLEROSIS, LOU GEHRIG’S DISEASE) 77 Motor neuron disease refers to a group of related disorders (Box 57.6), a few of which are clearly genetically determined, while most arise sporadically, are of completely unknown aetiology, and are generally untreatable The most common variant is the sporadic form known as amyotrophic lateral sclerosis (ALS), a relentlessly progressive degenerative disease which most commonly affects males over 50 years of age.78 In North America the term ALS is often used more generically, essentially equivalent to the broader term motor neuron disease PATHOGENESIS The disease affects both upper and lower motor neurons The involvement of either can predominate early on, giving rise to several clinically recognisable subgroups (see Box 57.6) The cerebral cortex as well as the anterior horns of the spinal cord are involved, with shrinkage, degenerative pigmentation and, eventually, disappearance of the affected cells accompanied by gliosis of the lateral columns (‘lateral sclerosis’) As muscles are denervated, there is progressive atrophy of muscle fibres (‘amyotrophy’), but, remarkably, sensory neurons as well as those concerned with autonomic function, coordination and higher cerebral function are all spared The precise cause remains unknown Postulated pathogenetic causes include oxygen free radicals, viral or prion infection, excess excitatory neurotransmitters and growth factors, and immunological abnormalities.79 Heavy metal exposure has also been implicated The only established clinical risk factors are age and family history CLINICAL PRESENTATION 80 The earliest symptoms are those of insidiously developing limb weakness, often asymmetrical, accompanied by obvious muscle wasting This classically affects the small muscles of the hand and may be accompanied by fasciculation As time passes, the disease becomes more generalised and more symmetrical, with a mixture of upper and lower motor neuron signs (i.e spasticity and hyperreflexia in addition to gross wasting), eventually involving bulbar and respiratory muscles Awareness and intellect were previously thought to be preserved, but it is now recognised that up to half the patients have evidence of cognitive dysfunction.81 Death occurs in 50% of cases within 3–5 years, usually due to Box 57.6 Degenerative motor neuron disease95 Amyotrophic lateral sclerosis Spinal muscular atrophy Bulbar palsy Primary lateral sclerosis Pseudobulbar palsy Heritable motor neuron diseases Autosomal recessive spinal muscular atrophy Familial amyotrophic lateral sclerosis Other Associated with other degenerative disorders Modified from Beal et╯al,95 with permission Rare causes of acute weakness in the ICU respiratory infection, aspiration or ventilatory failure from profound weakness However, there is wide variability, and a few patients may survive for many years DIAGNOSIS There are no specific investigations, and the diagnosis must be made on clinical grounds together with electromyogram (EMG) evidence of denervation in at least three limbs Experienced neurologists correctly diagnose the condition with 95% accuracy.82 The most important differential diagnosis is multifocal motor neuropathy The distinction is of clinical importance, as the latter is amenable to treatment Poliomyelitis can also result in a syndrome of progressive weakness, wasting and fasciculation, beginning many years after the initial illness (the post-polio syndrome), and leading occasionally to respiratory failure and death.83 MANAGEMENT Treatment is essentially symptomatic and supportive No benefit has been shown with antioxidants, growth factors and immunosuppressants.79 Current evidence suggests that non-invasive ventilation improves both quality of life and survival in patients who not have severe bulbar dysfunction,84 but the evidence favouring enteral tube feeding is much less convincing.85 The centrally acting glutamate antagonist riluzole has been shown to slow slightly the progression of ALS.86 Admission to ICU is sometimes requested when these patients present with an acute deterioration or intercurrent illness The intensivist may also be asked to assist with ambulatory or home respiratory support for gradually worsening chronic respiratory failure Respiratory support may be given by facemask, nasal mask or, rarely, by tracheostomy using simple, compact ventilators Some patients require only intermittent support, particularly at night or during periods of acute deterioration due to intercurrent illness Although home-based non-invasive ventilation has become routine, long-term invasive respiratory support outside the ICU is a major undertaking, requiring specific equipment and extensive liaison with the patient, the family and numerous specialised support services RARE CAUSES OF ACUTE WEAKNESS IN THE ICU PERIODIC PARALYSIS 87 This term describes a group of rare primary disorders, mostly inherited as autosomal dominant traits, producing episodic weakness They must be distinguished from other causes of intermittent weakness, including electrolyte abnormalities, MG and transient ischaemic attacks The inherited types are now grouped together 625 with the various forms of myotonia and susceptibility to malignant hyperthermia All are classified as congenital defects of skeletal muscle ion channels and there is an association with long QT syndrome and other cardiac channelopathies.88 Symptoms begin early in life (before age 25), and follow rest or sleep rather than exertion Alertness during attacks is completely preserved, and muscle strength between attacks is normal Treatment is usually successful in preventing both the attacks and the chronic weakness, which can develop after many years in untreated patients The hypokalaemic form of periodic paralysis is predominantly inherited, but can also present sporadically in association with thyrotoxicosis Involvement of bulbar or respiratory muscles occurs rarely The degree of hypokalaemia during attacks is mild, but patients rapidly respond to potassium administration Effective prophylaxis is conferred by acetazolamide, with potassium replacement Many patients eventually develop established myopathy Depolarising muscle relaxants should be avoided in these patients The hyperkalaemic form is milder, almost always inherited and rarely requires intensive care The serum potassium is modestly elevated at the beginning of attacks, but may be normal at other times CK may be elevated during attacks Attacks respond to carbohydrate administration, sympathomimetics (which activate the sodium/potassium pump) and acetazolamide Thiazide diuretics or acetazolamide provide effective prophylaxis Non-depolarising muscle relaxants should be avoided in these patients A normokalaemic form and several eponymously named congenital syndromes are also included in this group of disorders BOTULISM 89 Botulism is a widespread but very uncommon potentially lethal disease caused by exotoxins produced by Clostridium botulinum – an anaerobic, spore-forming Gram-positive bacillus The vast majority of botulism is foodborne and outbreaks are largely due to homepreserved vegetables (type A toxin), meat (type B) or fish (type E), but high-risk foods also include low-acid fruit and condiments Signs and symptoms are caused by toxin produced in vitro and then ingested The first recorded outbreak occurred in Germany in 1817, from ingestion of improperly preserved blood sausage (Botulus (Latin)â•›=â•›‘sausage’) Wound botulism arises rarely, when wounds (typically open fractures) are contaminated by soil containing type A or B organisms Intravenous drug abusers are an increasing source of this condition through infected injection sites Infantile botulism arises in infants under months of age, and is due to the active production of toxin by organisms in the gut rather than the direct ingestion of toxin 626 Neuromuscular diseases in intensive care Hidden botulism describes the adult equivalent of infantile botulism, and is a rare complication of various gastrointestinal abnormalities Inadvertent botulism is the most recently described form, and occurs as a complication of the medical or cosmetic use of botulinum toxin Inhalational botulism is the form that would occur as a result of aerosolised toxin released in the context of bioterrorism Botulinum toxin (BoNT) is the most potent known neurotoxin, and some authors have estimated that as little as gram of aerosolised BoNT could lead to the death of over 1.5 million people.90 In most cases, exogenously produced exotoxin is absorbed (primarily in the upper small intestine), and carried by the bloodstream to cholinergic nerves at the neuromuscular junction, postganglionic parasympathetic nerve endings and autonomic ganglia, to which it irreversibly binds The toxin enters the nerve endings to interfere with ACh release Most patients become ill about days after ingestion of toxin, with gastrointestinal symptoms (nausea, vomiting, abdominal pain, diarrhoea or constipation), dryness of the eyes and mouth, dysphagia and generalised weakness, which progresses in a symmetrical, descending fashion, with ventilatory failure in severe cases Cranial nerve dysfunction is a prominent early feature, manifested by ptosis and diplopia, facial weakness and impaired upper airway reflexes The pupils may be fixed and dilated in severe cases Patients are usually afebrile and have no sensory involvement The differential diagnosis includes food poisoning from other causes, MG and GBS Botulism can be confirmed by the presence of toxin (either in the patient’s serum or stool, or in contaminated food) in about twothirds of cases Contact tracing, in the case of foodborne botulism, is of great importance Treatment is mainly supportive, with airway protection and mechanical ventilation when required Mean duration of mechanical ventilation, when required, is weeks Clearance of toxin from the bowel with enemas and cathartics has been advocated Guanidine hydrochloride, which enhances the release of ACh from nerve terminals, has been reported to improve muscle strength, especially in ocular muscles, and may be useful in milder cases.91 Antibiotics have not been clearly shown to be useful Equine antitoxins are available, but side-effects are common and their efficacy is limited A human-derived antitoxin has been shown to be effective in infantile botulism,92 and the United States Defense Department has a pentavalent antitoxin, which is not available for public use Antitoxin must be given before the onset of paralysis in order to be effective In wound botulism, antibiotics (penicillin or metronidazole) and aggressive debridement are recommended Most patients begin to improve after a week or so, but hospitalisation is usually required for 1–3 months The mortality is low (5–8%) with good supportive care, including mechanical ventilation Mild weakness and constipation may persist for many months Access the complete references list online at http://www.expertconsult.com Sejvar JJ, Baughman AL, Wise M, et al Population incidence of Guillain–Barré syndrome: a systematic review and meta-analysis Neuroepidemiology 2011;36:123–33 27 Plasma Exchange/Sandoglobulin Guillain–Barré Syndrome Trial Group Randomised trial of plasma exchange, intravenous immunoglobulin and combined treatments in Guillain–Barré syndrome Lancet 1997;349:225–30 28 Patwa HS, Chaudhry V, Katzberg H, et al Evidencebased 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