Appendix Guillain - Barre Syndrome: A Short Guide for the Patient, Relative and Friend *

Introduction

This guide is written by a doctor for patients who have been told that they may have Guillain-Barre syndrome and their relatives and friends. It has to be honest and is meant to be reassuring. Unfortunately words cannot talk back and the writer cannot respond to the concern which this information may uninten• tionally arouse. Consequently if you do not understand or are worried by the information offered here, do ask your doctor to explain.

What is Guillain - Barre syndrome?

Guillain-Barre syndrome (GBS) is an uncommon illness causing weakness and loss of sensation which usually recovers completely after a few weeks or months. It is named after two French physicians, Guillain (pronounced Ghee-Yun) and Barre (pronounced Bah-Rray), who described it in 1916 in two soldiers who got better! It affects one person in 2000 each year, i.e. 1000 altogether each year in the United Kingdom. It can occur at any age from infancy onwards but is slightly more common in the old. It is more common in men than women. It is not hereditary: it is not passed on to your children. It is not infectious: it is not caught from or transmitted to anybody else. However, it does often develop a week or two after a throat or intestinal .

Early Symptoms

The first symptoms are usually either tingling (pins and needles) or loss of feeling (numbness) beginning in the toes and fingers or weakness so that legs feel heavy and wooden, arms feel limp and hands cannot grip or turn things properly. These symptoms may remain mild and clear up within a week or two without need for hospital admission. but most people need to be admitted to hospital. At the earliest stage it may be difficult for the patient to persuade the doctor

* Reproduced with permission of The British Guillain-Barre Syndrome Support Group. 296 Appendix: A Short Guide for the Patient, Relative and Friend that there is anything physical wrong, Within a few days it is all too obvious that something has gone wrong because legs simply will not bear weight, and arms become terribly weak and the doctor finds that the tendon reflexes have disappeared.

Investigations

The diagnosis of GBS is made on clinical grounds, not laboratory tests. This means that the doctor has to rely on the history and clinical examination fitting into the pattern of GBS. The doctor will particularly want to know of any recent possible toxin exposure (insecticides, solvents), alcohol intake, tick bites, family history of disease, or symptoms of any coincidental illnesses, such as diabetes (thirst, frequent urination, weight loss): any of these might lead to a different diagnosis. Investigations will include blood tests and usually a and electromyogram (EMG). The lumbar puncture involves lying on one side and having a needle inserted under local anaesthesia between the vertebrae into the sac of cerebrospinal fluid which surrounds the nerve roots. The idea is worse than the procedure really is and it does not usually hurt (the writer has had one). The cerebrospinal fluid often contains much more protein than usual while the cell content remains normal. If different changes are found the doctor has to review the diagnosis with even more care. The electromyogram or EMG is an electrical recording of the muscle activity. If a nerve is stimulated with a brief electrical pulse (felt like a sharp tap or jolt), muscle activity can be recorded and the speed of nerve conduction worked out. Often in GBS nerve conduction is slowed or even blocked. The test usually lasts about half an hour. It is only slightly uncomfortable and quite harmless.

Plateau Phase

The worst is usually reached within two or three weeks. After this begins the plateau phase, which usually lasts a few days or weeks, during which the course of the disease seems. stationary. Most people are so weak during this stage that they are confined to bed and rest is probably a good thing. However, it is very important to keep all the joints moving through a full range to stop them stiffening up. The physiotherapist is in charge of this physical therapy and may be pleased to supervise relatives and friends in what they can do to help. Unfortunately some patients get a lot of during GBS. This may come from the of the themselves, from the muscles which have tem• porarily lost their nerve supply, from stiff joints or simply because the patient is lying in an uncomfortable posture and is too weak to move into a more com• fortable position. To combat the pain, the doctors will prescribe pain-killers and the nurses and physiotherapists will help with repositioning and physical therapy. It helps to know that some pain is common in GBS, that the pain will disappear The Intensive Care Unit 297 as the condition improves, and that the occurrence of pain does not mean anything else is going wrong.

The Intensive Care Unit

This section is directed only towards the few patients that are so severely affected that they are transferred to an intensive care unit. About 20% of patients have weakness of the breathing muscles and have to be placed on a machine called a ventilator which will take over their breathing. This is less worrying than it sounds because it is absolutely routine for all intensive care units to take over breathing with an artificial ventilator. The connection to the ventilator is made under a short general anaesthetic to a tube in the windpipe (trachea) via the nose or mouth. This tube, called an endotracheal tube, can be left in place for a week or two. If artificial ventilation is required for longer a surgeon may make a small opening, called a tracheostomy, into the windpipe at the base of the neck which is much more comfortable and permits artificial ventilation for as long as necessary. The tracheostomy is also performed under a general anaesthetic. Fortunately in GBS artificial ventilation is rarely necessary for more than a few weeks, and most people, 80%, do not need artificial ven• tilation at all. When ventilation is no longer needed, the tracheostomy tube can be removed quite painlessly. The wound closes in a few days eventually leaving a faint scar below the line of a collar. Intensive care has become a very sophisticated part of medicine which has enormously improved the care of severe GBS. To make this possible pulse, blood pressure, temperature and blood chemistry have to be measured fre• quently. The pulse will be recorded by monitoring the heart beat, with an electrocardiogram, on a television screen to detect abnormalities which may need treatment. Patients may need infusions into veins to provide fluids and give drugs and a tube called a catheter in the bladder to drain the urine. A tube, called a nasogastric tube, may be passed from the nose into the stomach because swallowing may be difficult or impossible and food is still necessary. Constipa• tion can be a troublesome problem at first, but eventually nurses and patients invariably work out a regime of laxatives and suppositories which works. Com• munication can be a problem for a patient who is unable to talk but with blinks, nods, communication boards and above all patience it is almost always possible to get the message across. If the intensive care regime seems tedious, it is worth remembering that modern intensive care has made death from GBS a very rare event indeed.

Recovery Phase

Eventually the numbness begins to recede and strength begins to come back. Once it is clear that this is a genuine improvement rather than wishful thinking, there is cause for general rejoicing because improvement is likely to continue 298 Appendix: A Short Guide for the Patient, Relative and Friend steadily and two-thirds of patients recover completely. The time taken for recovery to occur is very variable. Sometimes it is only a week or two but most people remain affected for between three and six months. The one-third of patients who do not recover completely may be left with minor degrees of weakness, numbness and sometimes discomfort which do not usually seriously interfere with their lives. Some, however, are left so disabled that they cannot resume their former occupation. This is usually because of residual weakness of their hands and ankles so that strenuous manual work and walking are impaired. It is very rare to be left dependent on a wheelchair. Improvement is fastest during the first few months but some patients report continued gradual improvement even after a year has elapsed.

What Causes Guillain - Barre Syndrome?

The disease is due to inflammation of the peripheral nerves or "neuritis". The peripheral nerves connect the central nervous system to the skin and muscles. Because many nerves are inflamed GBS is called a "polyneuritis". Because it often occurs after an infection it is sometimes called "post-infective polyneuri• tis". The most likely explanation for the inflammation is that the immune cells, called lymphocytes, make a mistake and attack the nerves. They may have been tricked into making this mistake by the previous infection. Eventually the immune cells realise their mistake and stop attacking the nerves. A disease in which the immune system attacks its own body is called an auto (self) immune disease. Fortunately the part of the nerve attacked, which is the insulating or myelin sheath, can be replaced by the myelin-forming cells, named Schwann cells after the doctor who described them. Usually the conducting core of the nerve, called the axon, is not damaged. If the axons are damaged too, as sometimes occurs, they can regrow but recovery takes longer. The way in which trick the lymphocytes into attacking the body and producing such an is still the subject of research. The lymphocytes may cause the formation of chemicals called antibodies which circulate in the blood and latch onto and damage the myelin. The lymphocytes also attract macrophages, cells which can attack and digest myelin: this digestion is not altogether bad because the damaged myelin has to be removed before new myelin can be formed. Eventually special suppressor lymphocytes are formed which are able to suppress the action of the harmful lymphocytes. We need to know more about what induces the formation of these suppressor lymphocytes.

How Can We Cure Guillain-Barre Syndrome?

Since GBS usually gets better on its own the most important treatment is ordinary nursing and medical care with physiotherapy, and if necessary intensive care. No drugs have been proven to make any difference to the speed of re• covery. Some doctors prescribe steroid hormones in the acute stage but others Chronic Idiopathic Demyelinating 299 do not. Several recent trials have demonstrated that on average plasma exchange is helpful for severely affected patients in the first week or two of the illness. Plasma exchange involves being connected to a machine which can separate the blood cells from the fluid or plasma: about 250 ml of blood is removed at a time, the plasma is discarded and the blood cells are returned to the patient with clean plasma. The procedure is repeated several times on each of about five days until sufficient plasma has been exchanged. The risks of the procedure are extremely small and modern sterilisation has, for practical purposes, eliminated the risk of transmitting unpleasant infections in the clean plasma. Plasma exchange is probably not worthwhile in mildly affected patients or in acute GBS after the first couple of weeks. Although plasma exchange does seem to shorten the duration of the illness, particularly the time on a ventilator and the time to walk unaided, it is a help rather than a cure and improved treatments are constantly being sought.

Chronic Idiopathic Demyelinating Polyradiculoneuropathy

Chronic idiopathic demyelinating polyradiculoneuropathy, or CIDP for short, is much less common than GBS and most people reading this leaflet need not bother with this section. Less than 3% of patients with GBS have a second attack. It is usually clear from soon after the onset that a patient has CIDP because of its chronic, i.e. gradually progressive, course evolving over months or years rather than days or weeks as in GBS. Some patients with CIDP do have periods of worsening and then improvement and individual relapses are some• times rather confusingly like GBS. The name CIDP is derived as follows: C stands for chronic which refers to the gradual course. Idiopathic means "of its own cause" and is an admission that we cannot identify a specific cause such as diabetes or toxin exposure. Some doctors call CIDP "chronic inflammatory demyelinating polyradiculoneuropathy" or "chronic idiopathic polyneuritis" names which imply that, like GBS, the nerves are damaged by inflammation. Demyelinating means that the damage is to the insulating myelin sheaths round each nerve fibre and the central "wire" or axon is more or less spared. "Poly" means many, "radiculo" means root, "neuro" means peripheral nerve and "opathy" means disease: so polyradiculoneuropathy means disease of many peripheral nerves and the spinal roots (which are the points of origin of the peripheral nerves from the spinal cord). CIDP is probably an autoimmune disease similar to GBS. Although CIDP is a chronic condition several different treatments are thought to be helpful. They all act by suppressing the damaging autoimmune response. Examples are steroids, azathioprine and plasma exchange. Obviously suppressing the immune response cannot be undertaken lightly because it runs the risk of suppressing normal immune responses to infections. The decision whether to try these treatments has to be tailored by the doctor to the individual needs of each patient. How• ever, it is reassuring to know that demyelination can be repaired, that treatment is available and that some patients get better without treatment. 300 Appendix: A Short Guide for the Patient, Relative and Friend Where to go for help

Guillain-Barre Syndrome Support Group International PO Box 262 Wynnewood, Pa 19096, USA Tel: (215) 642 6855 Mrs G Sanders Guillain-Barre Syndrome Support Group Foxley, Holdingham Sleaford, Lines NG34 8NR, UK Tel: 0529 304615

In the UK the following may be useful: Department of Employment. The Disablement Resettlement Officer (DRO) can be extremely helpful where there are employment problems. He (or she) can usually be contacted through a local lob Centre or Employment Office. Department of Health & Social Security. Local offices deal with National Insurance Benefits. National Insurance Benefits may include Sickness Benefit, Invalidity Benefit, Attendance Allowance and Mobility Allowance. Disablement Income Group and DIG Charitable Trust, Millmead Business Centre, Millmead Road, London N17 9QU. Tel: 081-801 8013. Founded to fight for adequate income to meet the extra costs of disability, the charitable trust has an advisory service to help disabled people with problems regarding obtaining allowances for which they may be eligible. Disabled Living Foundation, 346 Kensington High Street, London W14 8NS Occupational Therapists give advice on all types of aids and equipment to help with the tasks of everyday living. They can also advise on adaptations to a dwelling. They can be contacted at Social Services Departments in the Com• munity, or through referral at a hospital Occupational Therapy Department. Royal Association for Disability and Rehabilitation (RADAR) 25 Mortimer Street, London WIN 8AB. Sexual and Personal Relationships of the Disabled (SPOD), 286 Camden Road, London N7 OBl. Tel: 071-6078851. Advisory leaflets available. Social Services Department of the local authority is empowered to provide services for the handicapped when necessary. The services available vary from area to area. However, home helps, meals on wheels and advice from an occu• pational therapist are generally available. A social worker will also visit on request to discuss any problems.

Further Reading

Hughes RAe, Winer 1B (1984) Guillain-Barre syndrome. In: Matthews WB, Glaser G (eds) Recent advances in clinical . Blackwells, Oxford, pp 19-49 Further Reading 301

Editorial (1986) Plasma exchange for neurological disorders. Lancet ii:1313-1314 Guillain-Barre Syndrome Study Group (1985) and acute Guillain-Barre syndrome. Neurology 35:1096-1104 Heller J, Vogel S (1986) No laughing matter. Jonathan Cape, London Hughes RAC (1990) Guillain-Barre syndrome. Springer-Verlag, Berlin, London Ropper AH, Shahani BT (1984) Diagnosis and management of acute areftexic with emphasis on Guillain-Barre syndrome. In: Asbury AK, Gilliatt RW (eds) Peripheral nerve disorders: a practical approach. Butterworths, London, pp 21-45 Subject Index

Acquired immunodeficiency syndrome Aystole 133 (AIDS) 108-10, 284, 287, 288 Atropine 133 sensory in 288 Autoallergic basis 11 ACfH 14,161-2,234 Autoimmune disorders 178,179 Acute ascending paralysis 1-4 Autoimmune haemolytic anaemia 178,232 Acute autonomic dysfunction 130 Autonomic dysfunction 132-6 Acute encephalomyelitis 11 Autonomic nervous system 132-6 Acute febrile polyneuritis 4,132 experimental 60-1 Acute infective polyneuritis 4-5 Autopsy observations 84-90 Acute inflammatory demyelinating Axonal degeneration 147, 150, 154 polyradiculoneuropathy 143 Azathioprine 171,236,237,238,270 Acute transverse myelitis 126 Addison's disease 178,232 AIDS. See Acquired immunodeficiency B cells 42 syndrome (AIDS) Bacterial infections 111 AIDS-related complex (ARC) 109,284,288 Bence-Jones protein 274-5,280 Albumino-cytological dissociation 5-12 Benign monoclonal gammopathy 275-8 Allergic granulomatous 265-6 Betablockers 158 Amiodarone 127,214 Biopsy studies in GBS 90-4,97-8,141,210, 127 223,226,230 Amyloidosis 275 Bladder 134 Analgesia 159 Blood count 193 Animal idiopathic polyradiculoneuropathy Blood - CSF barrier 178 254-5 Blood-nerve barrier 31-2,54 Animal models 247-59 Borrelia 128 Antecedent events Borrelia burqdorferi 112, 128 mycoplasma, bacterial and other Botulism 126-7 infections as 111-12 Bronchial carcinoma 181 viral infections as 106-11 Antibodies 183-93 Calcium channel antagonists 159 complement-fixing, to myelin antigens Campylobacter 111-12 185-9 Captopril 113 in CIDP 232-4 Carcinoma 181 to cardiolipin 190 Cardiac arrest 159 to glycolipids 190-1 Cardiac arrhythmias 133,158 to myelin proteins 192 Cardiolipin 190 to Schwann cells 192-3 Cauda equina neuritis of the horse 255-7 to whole nerve preparations 189-90 CD3+ cells 193 Antibody-dependent cell-mediated toxicity CD4+ cells 97,108 (ADCC) 40-2 CD8+ cells 71,97,108,193 Anticardiolipin antibodies 190 Cell-mediated immunity Antidiuretic hormone (AD H) secretion 135 in CIDP 234 Antigen prosentation 32 to nerve antigens 196-7 Aspirin 283 Cellular immunity 193-7 304 Index

Central nervous system (CNS) 29 subcategories of 208 Cerebrospinal fluid (CSF) 4-5,7-9,13,64, treatment 234-40 110,124,126,128,155,177-8,206, Circulating cells 193 210,219-20,296 Clinical course 122-3 Chest physiotherapy 158 Clinical features 88, 121-40 Children with GBS 154 and outcome after 12 months 154 Chlorambucil 277 Clofazimine 283 Chloroquine 238 Clostridium botulinum 127 Chomel 1 Clostridium botulinum type F 112 Chronic axonal neuropathy 213 Coeliac disease 232 Chronic demyelinating neuropathy 212 Colitis 179 Chronic idiopathic demyelinating Communication 159,172 polyradiculoneuropathy. See CIDP Complement 36, 182, 233 Chronic inflammatory Complement-fixing antibodies to myelin polyradiculoneuropathy 14 antigens 36,185-9,233 Chronic relapsing polyneuritis 14 Compound muscle action potential amplitude Churg-Strauss syndrome 265-6 (CMAP) 142-3,149,150 CIDP 13-15,73,98,109,110,113,122,137, Coonhound paralysis 252-4 164,171,180,181,185,188,205-46, Cortisone 161-2,234 255,267,284,287,299 Corynebacterium 129 and putative autoimmune diseases 232 Corynebacterium diphtheriae 129 antibodies in 232-4 Coxsackie virus autopsy studies 210 Critical illness polyneuropathy 129 biopsies 226 Crow-Fukase syndrome 280 cell-mediated immunity in 234 Cushingoid side-effects 236 cerebrospinal fluid (CSF) in 219-20 Cyclophosphamide 237, 270 chronic progressive or relapsing forms Cyclosporin A in EAN 74,238 205,225 Cytomegalovirus (CMV) 108-11,288 clinical course 221 Cytotoxic drugs 171, 236 clinical features 220-9 CNS involvement in 215-16, 219 definition 205 Dapsone 283 diagnosis 212 Death in GBS, causes of 156, 159 diagnostic criteria 206-7 Definition of GBS 8, 12-13,295 differential diagnosis 212 Demyelinating neuropathy 129,143 diseases associated with 209 causes of 212 distinguishing from GBS 207 Demyelinating serum factors 183-5 drug effects 214 Demyelination 22,27,142,143,183-5,197, familial incidence 229 250-2 frequency of recurrence following GBS antibody-dependent cell-mediated 40-1 208 antibody-mediated 35-40 history 205-6 bystander 44-5 HLA association in 229-31 cell-mediated cytotoxicity causing 41-2 immunogenetics 229-31 delayed hypersensitivity to myelin immunology 229-34 antigens causing 42 immunosuppressive drugs in 236-8 immunologically mediated 33-4 immunoglobulin in 239-40 inCIDP 207 monophasic chronic progressive 225 in EAE 54,55,64 220-230 inEAN 84 neurophysiology 207,216-19 macrophage-mediated 87 onset of 220 morphological sequence of 54 ophthalmoplegia in 211 neuropathy 83 paraprotein in 214 neurophysiology 63-4 pathology 220 primary 27 plasma exchange in 238-9 Denervation 141 preceding events 209-10 Diagnosis 1,124,177,296 presentation 220 criteria for GBS 12-13 prognosis 211 criteria for CIDP 14 severity of 221 Differential diagnosis 124-30 steroids in 234-6 129 Index 305

Diphtheria toxoid 105 Gastrointestinal infections 107 Disability grade scale 164-5 GD1b 29,278 Drug administration 113 General medical and nursing care 156-61 Drug involvement 127 Glomerulonephritis 137,180 130 Glucocerebroside 37 Glue sniffing 127 Glycolipids, antibodies to 190-1 Electrodiagnostic evaluation 143 GMt 29,37,278 Electromyogram (EMG) 296 Guillain-Barre syndrome 42 153 causes of 298 Electron microscopic studies 90 causes of acute neuropathy resembling Electrophysiological evidence 148-50 125 Endoneurium 19,22 definition 8, 12-13,295 Epidemiology 101-19 diseases associated with 178-81 influenza A/New Jersey (Swine) vaccine distinguishing from ClOP 207 epidemic 101-5 first description 1-4 Epineurium 19 first use of term 8 Epstein-Barr virus (EBV) 108-9 history 15-16, 211 Erythema nodosum leprosum 283 subdivisions 9 Experimental allergic encephalomyelitis synonyms 12 (EAE) 29-30,35,49-51,64-5,67, see also Animal models; Clinical features; 69,71 Definition; Diagnosis; Differential comparison with EAN 60 diagnosis; Epidemiology; History; demyelination in 54,55,64 Immunology; Pathology; Presentation; Experimental allergic neuritis (EAN) 11,35, Prognosis; Recovery; Treatment 42,49-81,85,150,183,227,250,253, Guillain-Barre Syndrome Support Group 287 173,295,300 actively induced 71-2 analogy with 83 antibody production 69-70 Hashimoto's Disease 232 autoimmune reaction 57 Heparin 159 cell-mediated immunity 70-2 Hepatitis 111 cells infiltrating peripheral nerves in 58 chronic active 232 chronic relapsing 61-3 Hereditary motor and sensory neuropathy comparison with EAE 60 types I and III (HMSN I and III) 214, cyclosporin'A in 74,238 230 demyelination in 84 Herpes simplex infection 109 early stages of 53-5,59,74 Herpes virus infections 108-9 genetic factors 69 History of GBS 15-16,211 identification of antigen responsible for HLA associations in GBS 181-2 64-9 in ClOP 230-1 importance of macrophages 75 Hodgkin's disease 179, 181 morphological changes 54 Human immunodeficiency virus (HIV) neurological signs of 59-60 infection 97,109,110,179,180,284, pathology 51-9 285-8 treatment 73-5 Hypereosinophilic syndrome 266 Experimental autonomic neuropathy 60 Hypersensitivity vasculitis 261, 265 Hypertension 159 Hypertrophic polyneuropathy 14 F ratio 145 Hyponatraemia 135 F wave latency 144,146 Hypotension 159 Familial incidence 181-2 Fowl paralysis 249 Idiopathic polyneuritis 12 Idiopathic thrombocytopenia purpura 178 IgA 95,278 Galactocerebroside 37, 233 IgG 19,95,96,128,182,210,278 Galactocerebroside neuritis 72-3 IgM 38,95,96,108,128,182,232-3,277-9 Gammaglobulin 171, 239 Immune complexes 182-3 Ganglioside 278 Immunisation, GBS following 105 Gastric carcinoma 181 Immunisation programmes 129 306 Index

Immunogenetics 229-31 Muscle monitoring 172 Immunoglobulin, treatment with intravenous Muscle wasting 153 171,182,239-40 Myasthenia 179 Immunohistochemical studies 94-7, 228, 233 Myasthenia gravis 126, 179 Immunology 177-204,229-34 Mycobacterium· 60 Immunosuppressive drugs 171,236-8 280,283,286,287 Incidence. See Epidemiology Mycobacterium tuberculosis 280 Inflammatory diseases 11 Mycoplasma infection 111 Influenza 111 Myelin 28-31 Influenza A/New Jersey (swine) vaccine vesicular disruption 92 15-16, 101-5 vesicular dissolution 90 Intensive care 156-159, 297 Myelin antigens, complement-fixing Intercellular adhesion molecules 30 antibodies to 36, 185-9 Intravenous immunoglobulin 171, 239 Myelin associated glycoprotein (MAG) Intubation 157 30-1,38,94,277 Investigations 296 Myelin basic protein (MBP) 29-30,49-50, Isoniazid 127 94 Ixodes burgdorferi 128 Myelin ganglioside. See GMt Myelin oligodendrocyte glycoprotein (MOG) 38 K-cells 40 Myelin Pz protein 192 Krabbe's disease 28 Myelin sheath 22,28,33,57,253,299 Myelination 22 Myokymia 147-8 Landry-Guillain-Barre Syndrome 8 Myometer 172 Landry's acute ascending paralysis 1-4 Myxoedema 179 280-7 Lower motor neuron syndrome 278 LTB4 75 Nasogastric tube 160 LTBs 75 Necrotising adrenalitis 178 Lupus anticoagulant effect 190 Neoplastic disease 181, 238 Lymphocyte transformation tests 196 Nerve antigens 196 Lymphocytes 193 cell-mediated immunity to 196-7 Lysophosphatidylcholine 34 Nerve conduction abnormalities 141 time course of 143-4 Nerve fibres 22 McBain's disease 280 Neuroblastoma cells 193 Macrophage migration inhibition test 197 Neuromuscular paralysis 156 Macrophage procoagulant activity 197 Neurophysiological studies 141-52 Major histocompatibility (MHC) antigens Nitrofurantoin 127 32-3,41,58-9,97 Non-Hodgkin's lymphoma 237, 270 Marek's disease 247-52 Mast cells 54, 75 Measles 110 Oligoclonal bands 178, 210 Melphalan 277 Onion bulb formation 61-3,98,223-5,227 6-mercaptopurine 171, 236 Operation 112 Methylprednisolone 163,235 Ophthalmoplegia 130-2 Miller Fisher syndrome 125,130-2 Osler 4 MOG (myelin-oligodendrocyte glycoprotein) 38 MAG see myelin associated glycoprotein Po protein 29,36,39,68-9,94 Monoclonal antibodies 74,96 Pz peptides 66-8 Monoclonal gammopathy of undetermined Pz protein 29,30,36,39,64-6,71,83,94, significance (MGUS) 275-9 192 Motor nerve conduction abnormalities Pain relief 161 141-3 Pandysautonomia 130 Multiple myeloma 275,279-80 Papilloedema 136, 180 (MS) 179-81,215-16, Paraprotein 238 220,231,235,238 Paroxysmal autonomic disturbances 133-4 Mumps 111 Pathology of GBS 9-12,83-100 Index 307

Patient care in GBS 156-61 Schwann cells 22,27,30,33,34,36,49,57, Perhexiline 127,214 95,98,150,192-3,250,252,298 Periarteritis nodosa 179,261 Sedation 159 Perineurium 19 Sensory evoked potentials (SEPs) 148 Peripheral nerve anatomy 19 Sensory nerve action potential (SNAP) Peripheral nerve damage 9-12 146-7,150 Peripheral nervous system Sensory nerve conducti9n 146-7 antigen presentation 32-3 Sensory neuronopathy acute 131 immunobiologyof 19-48 Shiqella boydii 112 Pernicious anaemia 179, 232 Siccasyndrome 232 Peroneal nerve biopsies 92 Sjogren's syndrome 130,268 Plasma cell dyscrasias and neuropathy 274-9 Skin cancer 237 Plasma exchange 164-71,236,238-9,277, Smallpox vaccination 110 299 Soluble IL-2 receptors 195,234 Plateau phase 296 Spinal and brainstem form of GBS 9 POEMS syndrome 280 Spinal form of GBS 9 Poliomyelitis 126 Spinal root biopsy 91 Poliomyelitis vaccination 105 Staphylococcal osteomyelitis 8 Polyarteritis nodosa 261,265 Steroids 161-4,234-6,277,298 Polyneuropathy, classification 4 Streptokinase therapy 113 Polyradiculoneuritis with mental involvement Sulphate-3-glucuronyl-lacto-N- 9 neotetraosylceramide (SGN) 29 Porphyria 128 Sural nerve biopsy 92,97, 223 Post-mortem electron microscopic studies 90 Surgical procedures 112-13 Prednisolone 162-3,179,270,283 Sweating 134 Prednisone 235,236 Symptoms 295-6 Pregnancy 113,209 Systemic lupus erythematosus (SLE) 232, Presentation 121 266-7 Pressure sores 160 Prognosis 123, 148-50, 153-5 Progressive lumbosacral polyradiculopathy T cells 32,41,42,50,68,71,72,74,75,94, 288 193,234,249,251,252 Protcase inhibitors 181,231 T helper cells 42, 94 Psychopathic symptoms 9 Tachycardias 158 Pulmonary capillary wedge pressure 159 Takayasu's arteritis 261 Pulmonary embolism 159,179 Temporal arteritis 179 Putative autoimmune diseases and CIDP 232 Tetanus toxoid 105 Thalidomide 283 Thrombocytopenia 181 Rabies 126 Thyrotoxicosis 179,232 Rabies vaccination 105-6 Tick bite meningoradiculitis 128 Radiculoneuritis 5-12 Tick bite paralysis 127 with albumino-cytological dissociation Total parenteral nutrition 160 83 Toxin exposure 127 Reassurance 159 Treatment of GBS 153-76,298-9 Recovery process 123, 172-3,296-7 Typhoid vaccination, GBS following 105 Rehabilitation 172-3 Remyelination 58,97-8,250-1 Respiratory failure 156 Vaccinia virus 11 0 Respiratory infections 107 Varicella-zoster infection 109 Respiratory support 157 Vasculitic disorders 179 Rheumatoid arthritis 179,267-8 Vasculitic neuropathy 261-70 Rifampicin 283 histology 262-5 RNA 19 presentation 261-2 111 treatment of 269-70 without clinical evidence of other organ involvement 268-9 Sarcoid neuropathy 270-4 Vasculitis 128,215 Schick test 129 classification 261 Schmidt-Lantermann incisures 30-1,33 Venous thrombosis 159 308 Index

Ventilation 133,153,157-8 278-9 Vincristine 127 Wallerian degeneration 27 Viral infections as antecedent events 106-11 Wegener's granulomatosis 261,265,269 Vital capacity measurement 157

Zimeldine 113,127 Waldenstrom's macroglobulinaemia 275,