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Whilst the advice and information in this book are believed to be true and accurate at the date of going to press, neither the author[s] nor the publisher can accept any legal responsibility or liability for any errors or omissions that may be made. In particular (but without limiting the generality of the preceding disclaimer) every effort has been made to check drug dosages; however it is still possible that errors have been missed. Furthermore, dosage schedules are constantly being revised and new side-effects recognized. For these reasons the reader is strongly urged to consult the drug companies’ printed instructions before administering any of the drugs recommended in this book.
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A catalogue record for this book is available from the British Library
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A catalog record for this book is available from the Library of Congress
ISBN 0 340 80814 4
1 2 3 4 5 6 7 8 9 10
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To the memory of Sheila Wallace and to my wife, Kate, and my children, Seonaid, Alastair and Nicholas, for their love and inspiration.
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Shunsuke Ohtahara, Yasuko Yamatogi and Yoko Ohtsuka
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Contributors
Jean Aicardi MDFRCP
Professor, Service de Pédiatrie, Hôpital Robert Debré, Paris, France
Joan K Austin DNSRNFAAN
Distinguished Professor, Department of Environments for Health, Indiana University School of Nursing, Indianapolis, USA
Frank MC Besag
Professor and Consultant Neuropsychiatrist, University of Luton, and Specialist Medical Department, Learning Disability Service, Twinwoods Health Resource Centre, Bedfordshire and Luton Community NHS Trust, Bedfordshire, UK
Paulo Rogério M de Bittencourt MDPhD
Clinical Director, Unidade de Neurologia Clínica, Curitiba, Brazil
Blaise FD Bourgeois MD
Professor of Neurology, Harvard Medical School, and Director, Division of Epilepsy and Clinical Neurophysiology, Children’s Hospital, Boston, USA
Harry T Chugani MD
Professor of Pediatrics, Neurology and Radiology, Wayne State University School of Medicine, and Chief, Division of Pediatric Neurology and Co-Director, Positron Emission Tomography Center, Children’s Hospital of Michigan, Detroit, Michigan, USA
Mary B Connolly MBMRCP(I) MRCP(UK) FRCP(C)
Head of Epilepsy Surgery Program and Clinical Associate Professor, Division of Neurology, Department of Paediatrics, British Columbia’s Children’s Hospital, Vancouver, BC, Canada
Paolo Curatolo
Professor of Pediatric Neurology and Chief, Division of Pediatric Neurology, Department of Neurosciences, Tor Vergata University of Rome, Italy
JoAnne Dahl PhD
Associate Professor, Department of Education and Psychology, University of Gävle, Sweden
Sharon Davies MB BS MRCPsych Specialist Registrar in Child and Adolescent Psychiatry, Great Ormond Street Hospital for Children, London, UK
Thierry Deonna
Professor, Unité de Neuropédiatrie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
Charlotte Dravet Centre Saint-Paul – Hôpital Henri Gastaut, Marseille, France
Olivier Dulac Professor, Service de Pédiatrie Neurologique, Hôpital Saint Vincent-de Paul, Paris, France
David W Dunn MD
Associate Professor, Departments of Psychiatry and Neurology, Indiana University School of Medicine, Indianapolis, USA
Kevin Farrell MBFRCP(E) FRCP(C) Professor, Division of Pediatric Neurology, University of British Columbia, and Director, Epilepsy Service, British Columbia’s Children’s Hospital, Vancouver, British Columbia, Canada
Natalio Fejerman Department of Neurology, Hospital de Pediatria, Prof. Juan P Garrahan Hospital, Buenos Aires, Argentina
Colin D Ferrie MDFRCPCH Department of Paediatric Neurology, The General Infirmary at Leeds, UK
Andrew Fisher PhD
Pharmacology and Therapeutics Unit, Department of Clinical and Experimental Epilepsy, Institute of Neurology, London, UK
Lars Forsgren MDPhD
Professor of Neurology, Department of Neurology, Umeå University Hospital, Sweden
Elena Gardella
Department of Neurological Sciences, University of Bologna, Bellaria Hospital, Bologna, Italy
R Mark Gardiner MDFRCPCHFMedSci
Professor, Department of Paediatrics and Child Health, Royal Free and University College Medical School University College London, The Rayne Institute, London, UK
Richard A Grünewald MADPhil FRCP
Consultant Neurologist and Honorary Senior Clinical Lecturer, Sheffield Teaching Hospitals NHS Trust and University of Sheffield, Royal Hallamshire Hospital, Sheffield, UK
Renzo Guerrini
Professor of Child Neurology and Psychiatry, University of Pisa, DUNPI–IRCCS Stella Maris, Calambrone, Pisa, Italy
The late Olaf Henriksen
Formerly of Statens Senter for Epilepsi, Sandvika, Norway
Isobel Heyman MBBSPhDMRCPsych
Consultant Child and Adolescent Neuropsychiatrist and Honorary Senior Lecturer, Maudsley Hospital/Institute of Psychiatry, and Great Ormond Street Hospital for Children, London, UK
Sara Hudson LPNCCRC
Executive Director, Pediatric Epilepsy Research Center, Children’s Mercy Hospital, Kansas City, Missouri, USA
John GR Jefferys FMEDSCI
Professor of Neuroscience and Head of Neurophysiology, University of Birmingham Medical School, Birmingham, UK
Csaba Juhász MDPhD
Assistant Professor, Department of Pediatrics, Division of Pediatric Neurology, Wayne State University School of Medicine, and Positron Emission Tomography Center, Children’s Hospital of Michigan, Detroit, Michigan, USA
Dorothée GA Kasteleijn-Nolst Trenité MDPhDMPH
Medical Center Alkmaar, The Netherlands
Rüdiger Köhling
Professor, Experimental Neurophysiology, Department of Epileptology, University of Bonn, Germany
Eric H Kossoff MD
The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA
Michael Koutroumanidis MD
Consultant and Honorary Senior Lecturer, GKT School of Medicine, Department of Clinical Neurophysiology and Epilepsies, Guy’s and St. Thomas’ NHS Trust, St. Thomas’ Hospital, London, UK
Ruben I Kuzniecky MD
Professor of Neurology, New York University Epilepsy Center, Department of Neurology, New York University, New York, USA
John H Livingston
Department of Paediatric Neurology, Leeds General Hospital, UK
Alain Malafosse
Professor, Hôpitaux Universitaires de Genève, Instituts Universitaires de Psychiatrie, Chêne-Bourg, Switzerland
Roberto Michelucci MD
Department of Neurosciences, University of Bologna, Bellaria Hospital, Bologna, Italy
Hajime Miyata MDPhD
Section of Neuropathology, Department of Pathology and Laboratory Medicine, UCLA Medical Center, Los Angeles, CA, USA and Department of Neuropathology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Tottori, Japan
Eli M Mizrahi MD
Head, Peter Kellaway Section of Neurophysiology, Professor of Neurology and Pediatrics, Director, Baylor Comprehensive Epilepsy Center, Baylor College of Medicine, and Chief, Neurophysiology Services, The Methodist Hospital and Texas Children’s Hospital, Houston, Texas
Jerome V Murphy MD
Principle Investigator Pediatric Epilepsy Research Center Children’s Mercy Hospital Kansas City MO USA
Shunsuke Ohtahara MDPhD
Professor emeritus, Department of Child Neurology, Okayama University Medical School, Shikatacho, Okayama, Japan
Yoko Ohtsuka MDPhD
Department of Child Neurology Okayama University Medical School, Shikatacho, Okayama, Japan
CP Panayiotopoulos MDPhDFRCP Department of Clinical Neurophysiology and Epilepsies, St Thomas’ Hospital, London, UK
Philip N Patsalos FRCPath PhD Professor of Clinical Pharmacology, Pharmacology and Therapeutics Unit, Department of Clinical and Experimental Epilepsy, Institute of Neurology, London, UK
Daniela T Pilz FRCPMD
Consultant in Medical Genetics, Institute for Medical Genetics, University Hospital of Wales, Cardiff, UK
Charles E Polkey MDFRCS
Emeritus Professor of Neurosurgery, Division of Clinical Neurosciences, Kings College Hospital, London, UK
Bwee Tien Poll-The MDPhD
Professor in Child Neurology, Academic Medical Center, University of Amsterdam, The Netherlands
Jean-Paul Rathgeb Service de Pédiatrie Neurologique, Hôpital Saint Vincent-de Paul, Paris, France
David Ravine FRACPMRCPath MD
Professor of Medical Genetics, University of Western Australia, Perth, Western Australia
Anna Lecticia Ribeiro Pinto MDPhD
Pediatric Neurologist, Clinica Neurológica de Joinville, Brazil
Raili Riikonen
Professor of Child Neurology, University of Kuopio, Head, Department of Child Neurology, Central Hospital of Kuopio University, Kuopio, Finland
Robert Robinson BA(Hons) MBBSMRCP
Department of Paediatrics and Child Health, Royal Free and University College Medical School, University College London, The Rayne Institute, London, UK
Guido Rubboli
Department of Neurological Sciences, University of Bologna, Bellaria Hospital, Bologna, Italy
Anne de Saint-Martin Service de Pédiatrie, CHU Hautepierre, Strasbourg, France
Matti Sillanpää MDPhD
Professor of Child Neurology, Departments of Child Neurology and Public Health, Turku University, Turku, Finland
John Stephenson
Professor in Paediatric Neurology, Fraser of Allander Neurosciences Unit, Royal Hospital for Sick Children, Glasgow, UK
Carlo Alberto Tassinari
Professor, Department of Neurological Sciences, University of Bologna, Bellaria Hospital, Bologna, Italy
Eileen PG Vining MD
Associate Professor of Neurology and Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA
Harry V Vinters MD
Departments of Pathology and Laboratory Medicine (Neuropathology and Neurology), and Brain Research Institute, Mental Retardation Research Center and Neuropsychiatric Institute, UCLA Medical Center Los Angeles, CA, USA
The late Sheila J Wallace Formerly Paediatric Neurologist, Department of Child Health, University Hospital of Wales, Cardiff, UK
Katherine Wambera
Division of Neurology, British Columbia’s Children’s Hospital, Vancouver, British Columbia, Canada
Kazuyoshi Watanabe MDPhD
Department of Paediatrics, Nagoya University Graduate School of Medicine, Showa-ku, Nagoya, Japan
William P Whitehouse
Senior Lecturer in Paediatric Neurology, The University of Nottingham and Queen’s Medical Centre, University Hospital, Nottingham, UK
Peter KH Wong MDFRCPC
Division of Pediatric Neurology, Department of Pediatrics, University of British Columbia, and Department of Clinical Neurophysiology, British Columbia’s Children’s Hospital, Vancouver, British Columbia, Canada
Yasuko Yamatogi MDPhD
Faculty of Health and Welfare Science, Okayama Prefectural University, Soja, Okayama, Japan
Sameer Zuberi
Consultant Paediatric Neurologist, Fraser of Allander Neurosciences Unit, Royal Hospital for Sick Children, Yorkhill, Glasgow, UK
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Foreword
At one time epilepsy was epilepsy,and the field of epilepsy was dominated by adult epileptologists who seemed to believe that children were only small adults, that adult epilepsy was identical to epilepsy in children of all ages and sizes.They appeared to think that the underlying pathology,pathogenesis,and treatment ofchildren with epilepsy were similar,only the dosing ofmedications was different.Sheila Wallace was one ofthe pioneers in pediatric epilepsy,and played a prominent role in beginning to change the perceptions ofadult epileptologists,helping them to recognize that epilepsy in children was different.
A limp may have many varied causes:from a nail in a shoe,a bad knee or hip,to a stroke or cerebral palsy.The differential diagnosis ofa limp and its pathogenesis and treatment will vary with the age ofthe patient and the course ofthe condition.However,all the patients hobble. Just so,seizures are a manifestation ofa dysfunction within the nervous system,but their manifestations, etiology,pathogenesis,and management will often differ by age,even within childhood,and will often be different from those ofseizures in adults.Children are not small
adults,and epilepsy in children is different than epilepsy in adults.
This second edition of Epilepsy in Children is testimony to those differences.A perusal ofthe table ofcontents reveals the breadth and depth to which the editors have gone to cover this increasingly extensive field.The authors ofchapters not only cover,in depth,the myriad aspects and multiple types ofepilepsy in children,but also further cover the many aspects by which children and their families are affected by epilepsy.Authors come from around the world,and represent many ofthe best, brightest,and most forward thinking in this field.The editors have chosen well.
As my mother would have said,“Ifonly Sheila could have lived to see it – she would have been justly proud.” Kevin Farrell,as the co-editor,should be proud as well,of having completed the editing ofa book that will add to the growing field ofepilepsy in children.
John M.Freeman,MD
October 2003
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Preface
This second edition of Epilepsy in Children reflects not only Sheila Wallace’s vision ofthe problem ofepilepsy in this age group but also her ability to translate the science ofepilepsy into a practical clinical approach.There have been many major advances in both the basic and clinical science ofepilepsy since this work was first published, and the purpose ofthis edition is to demonstrate the relevance ofthese discoveries to the practical management ofepilepsy in this age group.This book is written therefore for those who are involved in the management of children with epilepsy.
Seizures are a symptom and many diseases manifest with this problem.In addition,the management of patients with epilepsy often involves a wide variety of fields including pediatrics,neurology,neurophysiology, nursing,pharmacology,neuroradiology,genetics,neurosurgery,neuropathology,psychiatry,psychology,social work and education.Consequently,the contributors come from a wide variety ofdifferent disciplines.On behalfofSheila,I must thank all ofthese authorities for helping to make this edition relevant to those who care for children with epilepsy.
During the writing ofthis book,Sheila developed a brain tumor that made it increasingly difficult for her to edit.Her approach to this illness and her ability to retain her spirit despite her problems reflected the outstanding nature ofSheila’s character and contributed greatly to the completion ofthis edition.I had the good fortune to work with Sheila on her last major work and was supported by her warmth,experience and strength ofcharacter.We were helped considerably by Joanna Koster,Sarah Burrows, Zelah Pengilley ofArnold Publishing and the proofreader, Rose James.I need also to thank Leila Laakso,Julie Baron, Pam Sekhon and Francesca Zanotto at British Columbia’s Children’s Hospital,who kept the ship afloat;Drs Mary Connolly,Olivia O’Mahony,Peter Wong,Kati Wambera and Alan Hill,who helped me complete the work;and Pnina Granirer for her artistic work.Finally,my greatest thanks must be to my wife,Kate,and my children, Seonaid,Alastair and Nic,who make it all worthwhile.
Kevin Farrell
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Sheila Wallace – an appreciation
This is not an obituary for Sheila but rather an appreciation,a dedication,indeed an attempt at a celebration ofthe remarkable individual who began the venture that is this book,not knowing that it was to become her memorial.
Sheila’s early life and education were in England.She went to the sort ofschool that instils a certain language and accent ofauthority,her voice precise,concise,wellmodulated and even pukka.I first met her when we were both junior doctors doing paediatrics in the fertile intellectual breeding ground ofEast Anglia,a flat part of England very different from her later haunts in hilly Wales and mountainous Scotland.Ever since she was amused to boast that she had been senior to me in rank although younger than I by several months.Later she said that being tough-minded and competitive was necessary for a woman in medicine in those days.Humour was certainly an integral part ofher make-up throughout her life,and many readers will remember her hearty laugh and almost conspiratorial guffaw.
Then she took offnorthwards to Edinburgh where in paediatric neurology Tom Ingram was king.He it was who suggested that she immerse herselfin the study offebrile seizures.This led to her book The Child with Febrile Seizures,published in 1988,and to a lifetime studying the
phenomenology and treatment ofepileptic seizures that culminated in the present volume.
It is important to recognize that Sheila was not merely an epileptologist in an ivory tower but was a fully qualified wide-ranging child neurologist.She was fortunate in having been able to join the Department ofNeurology at Great Ormond Street Hospital for Sick Children under Drs John Wilson and Edward Brett and to become the Consultant Paediatric Neurologist at the University Hospital ofWales in Cardiff.In a busy career in the British National Health Service – predominantly single-handed –she found time to contribute to the literature on the varied problems ofpaediatric neurology that came her clinical way:genetic,metabolic,infectious or whatever.
I have mentioned and illustrated Sheila’s lifelong and often irrepressible sense ofhumour,but three other facets ofher character are worthy ofnote.These were common sense,the ability to communicate,and innate skills in the organization ofspace and time.Kevin Farrell tells me that the attribute ofcommon sense was very apparent to him as a co-editor.She was down to earth,with a no-nonsense style and the ability to give political correctness the lack of respect it deserves.There is a web site devoted to common sense that includes a discussion ofits localization within
the brain.No-one will be surprised to hear that the answer is not clear:I can only imagine Sheila’s reaction to the question!
Sheila was a master – or mistress – ofcommunication. To many worldwide she was known not only for her spoken presentations and lectures,given with great clarity and effect,particularly on the epilepsies ofchildhood and their medical management,but also for her quiet but clear and penetrating questions given at the microphone in a serious rather deep voice with inflections ofhumour and accompanied by a quizzically-raised eyebrow.To leaders in the field ofchild neurology and epilepsy she was known for her work in committees and boards.She was elected more than once to the boards ofboth the International Child Neurology Association and the European Paediatric Neurology Association.She was chairman ofthe Professional Group ofthe Epilepsy Task Force (UK), member ofthe Scientific Advisory Committee ofthe Epilepsy Research Foundation,and member ofthe teaching faculty for the Master ofScience degree in Epileptology at King’s College,London.She served on the editorial boards of Developmental Medicine and Child Neurology and the European Journal ofPaediatric Neurology.That she was able to persuade so many to collaborate with her (and the number ofcontributors to this book is now over 60 from 14 countries) is a tribute to her pre-eminent communication skills.
Turning now from her left to her right hemisphere, Sheila was also gifted with immense skills in space and in time.At international meetings it was she who arrived by the most adventurous route,and afterwards backpacked into byways offthe path ofthe official post-conference tours.She was indeed an inveterate traveller.She managed 49 countries,and only regretted not making the halfcentury.My most relaxed memory ofher is ofa long lunch in the open air under a plane tree – in truth,the identity ofthe tree was in dispute,despite Sheila’s botanical expertise (she had made a beautiful garden in the grounds ofher personally restored 500 year old home in Wales where she came first with her late husband) – by some water in Strasbourg,en route to a board meeting of the European Paediatric Society in Baden-Baden.We ate a fish called sander and drank wine from Alsace.When Sheila got home she sent me a post card to confirm that sander pike-perch.
It was fun to write chapters for Sheila’s most important book.When I queried the title ofour chapter in the second edition she replied:
Good! I told Kevin you would want to write about anoxicepileptic seizures, and I give you permission to do so. As
for the title: I do not like ‘nonepilepsy’ it sounds too gimmicky and I should remind you, this is a serious book! We can sort out something which we agree on later, but call it differential diagnosis for now.
But when we were late another email arrived:
I hope it really is nearly done, and that you are able to send it to Kevin before I die of the glioblastoma which has recently raised its ugly head in the mid/posterior part of my corpus callosum! A little bit of neuroanatomical knowledge is undoubtedly helpful in directing one to the relatively intact verbal skills that I still retain! Please finish it soon. I hope you are reasonably well at the moment, and I salute our years of comradeship. I have always enjoyed your company.
Sheila.
What do you do then? I telephoned and spoke with her for at least an hour or maybe two.She had lost her sense ofspace and time because the tumour had invaded her right parietal lobe.She wanted to stay in her old house and beautiful garden,but could not and soon had to be moved to hospital.I told her nurse that she was world class,and wrote to Sheila that her voice was in the heads ofthousands throughout the world.To be destroyed by a glioblastoma in one’s own brain must have been an extraordinary hardship for her,but her reaction was typical ofthe robust bravery that had characterized her life for decades before.When I asked her ifshe was still going to the International Child Neurology Association meeting in Beijing in October 2002,Sheila replied that no,she would have no more deadlines,no more deadlines – just one.Her sense ofhumour,now black humour,had not deserted her.
Kevin wrote to me that in her final illness: she demonstrated a wonderful ability to enjoy the moment and to live life to the fullest. In many ways, I think that she had many of the qualities of a good highland lady, which may stem from the fact that she had the good luck to train in Scotland.
Be that as it may,she is not easily pigeonholed:she was truly her own person.I fancy she might smile to think that through this new edition ofher book she will now easily attain and exceed the 50 countries into which she strove to communicate about epilepsy and about children.
Xe-SPECTxenon-133 single photon emission computed tomography
ZCAzone ofcortical abnormality
ZSZellweger syndrome
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Definitions and classification of epileptic seizures and epilepsies
OLAF HENRIKSEN AND SHEILA J WALLACE
Key points
Variability in the clinical expressions ofepileptic seizures was recognized by the Ancient Greeks,who described both generalized and focal seizures.However,more formal definitions and classifications were not published until the twentieth century,when it was recognized that these would advance the practice ofepilepsy.Identification of seizure type is essential for correct management.Similarly, classification is necessary for communication and for comparingdata.As such,it is important that the terminology is based on simple,well-defined facts that are retrievable
and broadly acceptable.These aims led the International League Against Epilepsy (ILAE) to form Commissions onClassification.The need to include the EEG characteristics in addition to the clinical findings was recognized from the outset.Early discussions centered on defining the criteria for particular seizure types and a proposal for a clinical and electroencephalographic classification ofepileptic seizures was published in 1981.1 Consideration was then given to a classification based on age at onset, etiology,natural history and localization ofthe region of
Table 1.1 Proposed diagnostic scheme for people with epileptic seizures and with epilepsy (Reproduced with the permission of Blackwell Publishing Ltd from Engel J Jr. Epilepsia 2001; 42: 796–803)
Epileptic seizures and epilepsy syndromes are to be described and categorized according to a system that uses standardized terminology, and that is sufficiently flexible to take into account the following practical and dynamic aspects of epilepsy diagnosis:
1 Some patients cannot be given a recognized syndromic diagnosis.
2 Seizure types and syndromes change as new information is obtained.
3 Complete and detailed descriptions of ictal phenomenology are not always necessary.
4 Multiple classification schemes can, and should, be designed for specific purposes (e.g. communication and teaching, therapeutic trials, epidemiologic investigations, selection of surgical candidates, basic research, genetic characterizations).
This diagnostic scheme is divided into five parts, or axes, organized to facilitate a logical clinical approach to the development of hypotheses necessary to determine the diagnostic studies and therapeutic strategies to be undertaken in individual patients:
Axis 1 Ictal phenomenology, from the Glossary of Descriptive Ictal Terminology, can be used to describe ictal events with any degree of detail needed.
Axis 2 Seizure type, from the List of Epileptic Seizures. Localization within the brain and precipitating stimuli for reflex seizures should be specified when appropriate.
Axis 3 Syndrome, from the List of Epilepsy Syndromes, with the understanding that a syndromic diagnosis may not always be possible.
Axis 4 Etiology, from a Classification of Diseases Frequently Associated with Epileptic Seizures or Epilepsy Syndromes when possible, genetic defects, or specific pathologic substrates for symptomatic focal epilepsies.
Axis 5 Impairment: this optional, but often useful, additional diagnostic parameter can be derived from an impairment classification adapted from the WHO International Classification of Functioning and Disability (ICIDH-2).
2Definitions and classification of epileptic seizures and epilepsies
Table 1.2 Definition of key terms (Reproduced with the permission of Blackwell Publishing Ltd from Engel J Jr. Epilepsia 2001; 42: 796–803)
Epileptic seizure type
An ictal event believed to represent a unique pathophysiologic mechanism and anatomic substrate. This is a diagnostic entity with etiologic, therapeutic and prognostic implications. [new concept]
Epilepsy syndrome
A complex of signs and symptoms that define a unique epilepsy condition. This must involve more than just the seizure type: thus frontal lobe seizures per se, for instance, do not constitute a syndrome. [changed concept]
Epileptic disease
A pathologic condition with a single specific, well-defined etiology. Thus progressive myoclonus epilepsy is a syndrome, but Unverricht-Lundborg is a disease. [new concept]
Epileptic encephalopathy
A condition in which the epileptiform abnormalities themselves are believed to contribute to the progressive disturbance in cerebral function. [new concept]
Benign epilepsy syndrome
A syndrome characterized by epileptic seizures that are easily treated, or require no treatment, and remit without sequelae. [clarified concept]
Reflex epilepsy syndrome
A syndrome in which all epileptic seizures are precipitated by sensory stimuli. Reflex seizures that occur in focal and generalized epilepsy syndromes that also are associated with spontaneous seizures are listed as seizure types. Isolated reflex seizures also can occur in situations that do not necessarily require a diagnosis of epilepsy. Seizures precipitated by other special circumstances, such as fever or alcohol withdrawal, are not reflex seizures. [changed concept]
Focal seizures and syndromes
Replaces the terms partial seizures and localization-related syndromes. [changed terms]
Simple and complex partial epileptic seizures
These terms are no longer recommended, nor will they be replaced. Ictal impairment of consciousness will be described when appropriate for individual seizures, but will not be used to classify specific seizure types. [new concept]
Idiopathic epilepsy syndrome
A syndrome that is only epilepsy, with no underlying structural brain lesion or other neurologic signs or symptoms. These are presumed to be genetic and are usually age dependent. [unchanged term]
Symptomatic epilepsy syndrome
A syndrome in which the epileptic seizures are the result of one or more identifiable structural lesions of the brain. [unchanged term]
Probably symptomatic epilepsy syndrome
Synonymous with, but preferred to, the term cryptogenic, used to define syndromes that are believed to be symptomatic, but no etiology has been identified. [new term]
onset and this resulted in a proposal for a classification of epilepsies and epileptic syndromes in 1989.2 More recently, attempts to correlate specific epilepsy syndromes with demonstrable changes in genotype have provided some unexpected results.For example,the clinical phenotype ofgeneralized epilepsy with febrile seizures plus (GEFS ) (see Chapter 4A) embraces many different seizure types and a very variable natural history,and maps to more than one chromosomal site.
In 1997,the ILAE constituted a new Task Force on Classification,which formed four working groups concerned with (a) descriptive terminology for ictal events;(b) seizures;(c) syndromes and diseases;and (d) impairment.3 The Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy is reproduced (with permission) as Table 1.13 and the definitions ofkey terms as Table 1.2.4
The proposed diagnostic scheme classifies patients using four main axes:ictal phenomenology,seizure type,syndrome and etiology.A fifth axis,impairment,is considered to be often useful but has been proposed at this stage as an option.
KEY POINTS
• Definitions ofseizure type are based on a purely descriptive phenomenological approach
• An epilepsy syndrome comprises a complex of signs and symptoms that define a unique epilepsy condition that may have different etiologies
• An epileptic disease is a pathologic condition with a single,well-defined etiology,e.g.
Unverricht-Lundborg disease
• An epileptic encephalopathy is a condition in which theepileptiform abnormalities themselves are believed to contribute to the progressive disturbance in cerebral function
• It is proposed that the term focal replace partial
• It is proposed that impairment ofconsciousness not be used to classify a seizure and that the terms simple and complex be no longer used
• It is proposed that probably symptomatic epilepsy syndrome refer to those epilepsies previously described as cryptogenic
• The concept of benign epilepsy syndrome has been clarified and that of reflex epilepsy syndrome changed
REFERENCES
1.Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsia 1981; 22: 489–501.
2.Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989; 30: 389–399.
3.Engel J Jr. ILAE Commission Report. Proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE task force on classification and terminology. Epilepsia 2001; 42: 796–803.
4.Blume WT, Luders HO, Mizrahi E, Tassinari C, van Emde Boas, Engel J Jr. ILAE Commission Report. Glossary of descriptive terminology for ictal seminology: report of the ILAE task force on classification and terminology. Epilepsia 2001; 42: 1212–1218.
Paroxysmal nonepileptic disorders: differential diagnosis of epilepsy
JOHN STEPHENSON, WILLIAM P WHITEHOUSE AND SAMEER ZUBERI
A significant proportion ofchildren suspected ofhaving epilepsy,and even those with a definite diagnosis ofepilepsy or even refractory epilepsy,have never had an epileptic seizure.Misdiagnosis rates are high throughout the world,irrespective ofthe wealth and resources available to the health-care system.The principal reason for this is that the diagnosis ofepilepsy will always be dependent on the difficult,lonely,art ofhistory taking.The physician taking the history must have an expertise in recognizing the clinical features ofall the myriad forms of epileptic seizures,but this must be combined with an equally expert knowledge ofthose paroxysmal disorders that may mimic epilepsy.Most ofthe patients presenting to a first seizure or epilepsy clinic will not have epilepsy, and this alone justifies the place ofthis chapter in a book on epilepsy.
The diversity ofnonepileptic events is considerable and all ofthe variations have certainly not yet been described.The most common type ofnonepileptic seizure leading to diagnostic confusion in clinical practice is the anoxic seizure,or syncopal convulsion.1,2 The term seizure is used in this chapter to include nonepileptic events,such as fainting fits,in the same way that we use it when speaking ofepisodes with apparent psychological mechanism,the so-called pseudo-epileptic seizures.
It is our clinical impression that misdiagnoses of epilepsy are more likely to arise when a child has more than one paroxysmal nonepileptic disorder,for example
two or more ofthe following:psychological events, syncopes,migraine and sleep disorders.That said,syncope was a surprisingly uncommon diagnosis in recent video-EEG studies ofparoxysmal nonepileptic events in children and adolescents from tertiary centres.3,4 In the study ofBye et al., 3 which included children with developmental delay and mental retardation,psychological and sleep phenomena predominated and the EEG frequently showed misleading ‘epileptiform’discharges. Kotagal et al.4 reported on 134 children and adolescents referred to a pediatric epilepsy monitoring unit at the Cleveland Clinic over a 6-year period.The most common diagnoses in children under 5 years ofage were stereotypies,sleep jerks,parasomnias and Sandifer syndrome.The most common diagnoses in the 5–12-year age group were conversion disorder (psychogenic pseudoepileptic seizures),inattention or day dreaming,stereotypies,sleep jerks and paroxysmal movement disorders. Finally,over 80 per cent in the 12–18-year age group had a diagnosis ofconversion disorder (hysteria,or psychogenic pseudo-epileptic seizures).Why syncopes,the most common type ofnonepileptic disorder misdiagnosed as epilepsy,2,5 did not feature as highly in these studies is not clear,but might reflect an ascertainment bias in that these children had sufficiently frequent episodes to be admitted for video-EEG investigation and a significant proportion ofthe children studied had concomitant epilepsy.
We have somewhat arbitrarily divided nonepileptic events into the following categories:
• syncopes and anoxic seizures
• psychological disorders
• derangements ofthe sleep process
• paroxysmal movement disorders
• migraine and possibly related disorders
• miscellaneous neurological events
• anoxic-epileptic seizures.This is an important and under-recognized phenomenon in which provoked syncopes themselves trigger epileptic seizures in individuals who rarely have unprovoked epileptic seizures.6
SYNCOPES AND ANOXIC SEIZURES
An anoxic seizure is the consequence ofa syncope,which occurs usually as a result ofa sudden decrease in cerebral perfusion ofoxygenated blood,either from a reduction in cerebral blood flow itselfor from a drop in the oxygen content,or a combination ofthese.1,6 Although we have categorized the syncopes,it must be recognized that overlaps occur.Indeed,it seems likely that what we call reflex anoxic seizures or reflex asystolic syncope (RAS), breath-holding spells or prolonged expiratory apnea, vasovagal syncope and neurocardiogenic syncope are all varieties ofthe same disorder,called by the adult cardiologists neurally mediated syncope.When parents or children are bewildered by these diagnoses,or annoyed when told that ‘it’s only breath holding’or ‘only a simple faint’,contact with a family support organization (such as http://www.stars.org.uk) may be very helpful.
Reflex anoxic seizures or reflex asystolic syncope (RAS)
Gastaut used the term reflex anoxic cerebral seizure to describe all the various syncopes,sobbing spasms and breath-holding spells which followed noxious stimuli in young children.7 Since 1978,the term reflex anoxic seizure has been used more specifically to describe a particular type ofnonepileptic convulsive event,most commonly induced in young children by an unexpected bump to the head.8 Although other terminology,such as pallid breath-holding and pallid infantile syncope have been applied to such episodes,9 the term reflex anoxic seizure is now widely recognized.10,11
The clinical picture has been extensively documented.6 Previously,almost all the available information came from descriptions by parents who had witnessed their child’s attack or from observations ofepisodes reproduced by ocular compression,and it is only in
recent times that home video-recordings ofreflex anoxic seizures have become available.The first video-recording ofa ‘natural’attack obtained by one ofthe authors occurred when a 4-year-old girl was having capillary blood taken.The accompanying convulsive syncope was more severe than that previously recorded in the same child after ocular compression.Although the duration ofpostictal stupor was also longer,other mothers who watched the videotape were surprised at how quickly the child recovered.Until the advent ofcardiac loop recorders,there was very little direct evidence ofthe pathophysiology ofnatural attacks.In a child who had two episodes while connected to EEG and ECG recorders,cardiac asystole alone appeared to account for the resultant anoxic seizure.6 At the time ofthe first edition ofthis book,only three further recordings had been obtained.12 Observations ofthe anoxic seizure induced by ocular compression in one of these children help to explain the confusion between reflex anoxic seizures and breath-holding spells.When this particular girl had ocular compression,asystole was apparent from the moment the thumbs began the ocular pressure, which is the usual finding in such cases.In the first few seconds ofthe asystolic period,however,there were repeated expiratory grunts at about three grunts per second.The parents recognized these grunts as similar to those noticed in the natural events.Thus,the anoxic seizure in this case was clearly related to the cardiac asystole and the expiratory grunting was an epiphenomenon.In cyanotic breathholding attacks resulting in syncope or even a convulsive syncope,the amount and duration ofexpiratory grunting is greater and longer and the latency between the stimulus and the nonepileptic motor seizure is undoubtedly greater. Since prolonged cardiac recording by loop recorders has become feasible in young children,many episodes of prolonged reflex asystole have been recorded and several examples published.13–16
Extracts from a letter from a consultant neurologist may give the reader some idea ofthe clinical diagnostic difficulties that were experienced before the phenomenon ofreflex asystole was well known:
Thank you for asking me to see this seven-year-old young man. As a toddler he began to have attacks of loss of awareness, rigidity and eye rolling which would be induced by minor knocks. This has continued and recently an episode occurred in which he had an undoubted tonic/ clonic seizure with incontinence of urine. Curiously, as far as I can tell from mother’s account, every attack has been triggered by a minor bump on the head and he has never had an attack out of the blue. He had difficulties at birth. The family history is clear except for a convulsion in the mother when she was tiny, about which there is no further information. It seems to me that this boy is having a form of reflex epileptic seizure and my inclination would have been to start treatment with sodium valproate.
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The Sheriff of Cornwall accordingly was directed to eject him by force. Rogers, however, barricaded the house, and prepared to defend it. He supplied himself with gunpowder and slugs, and cut loopholes in his doors and shutters from which to fire at the assailants.
On June 18th, 1734, the Under-Sheriff and a posse went to Skewis and demanded the surrender of the house. From two to three hundred people attended, for the most part sympathisers with Rogers, but not willing to render him effectual assistance.
As the Under-Sheriff, Stephen Tillie, persisted in his demands, and threatened to break into the house, Rogers fired. The bullet passed through Tillie’s wig, singed it, and greatly frightened him, especially as with the next discharge one of his officers fell at his side, shot through the head.
Several guns were fired, and then the Under-Sheriff deemed it advisable to withdraw and send for soldiers.
On the arrival of a captain with some regulars, Tillie again approached, when Rogers continued firing, and killed a bailiff and shot a soldier in the groin. Two more men were wounded, and then the military fired at the windows, but did no harm. Mrs. Rogers stood by her husband, loading and handing him his gun.
The whole attacking party now considering that discretion constituted the best part of valour, withdrew, and Rogers was allowed to remain in possession till March in the following year, that is to say, for nine months. Then he was again blockaded by soldiers, and the siege continued for several days, with the loss of two more men, when at last cannon were brought from Pendennis Castle.
Many years after, one of Rogers’ sons gave the following account of his reminiscences of the siege: “He recollected that his father was fired at, and had a snuff-box and powder-horn broken in his pocket by a ball. He recollected that whilst he himself (then a child) was in the bed several balls came in through the window of the room, and after striking against the wall rolled about on the floor.
One brother and sister who were in the house went out to inquire what was wanted of their father, and they were not permitted to return. On the last night no one remained in the house but his father, himself, and the servant-maid. In the middle of the night they all went out, and got some distance from the house. In crossing a field, however, they were met by two soldiers, who asked them their business. The maid answered that they were looking for a cow, when they were permitted to proceed. The soldiers had their arms, and his father had his gun. The maid and himself were left at a farmhouse in the neighbourhood.”
Henry Rogers, whom the soldiers had not recognised in the darkness, managed to escape, and pushed on in the direction of London, resolving to lay his grievances before the king. He was dressed in a whitish fustian frock, with imitation pearl buttons, and a blue riding-coat over it.
As soon as it was discovered that he had decamped, a reward of £350 was offered for his apprehension. He had already shot and killed five men, and had wounded seven. He was not, however, taken till he reached Salisbury Plain, where he hailed a postboy, who was returning with an empty chaise, and asked for a lift. He was still carrying his gun. The boy drove him to the inn, where he procured a bed; but the circumstances, and the description, had excited suspicion; he was secured in his sleep, and was removed to Cornwall, to be tried for murder at Launceston along with his serving-man, John Street.
His trial took place on August 1st, 1735, before Lord Chief Justice Hardwicke. Rogers was arraigned upon five indictments, and Street upon two. Both received sentence of death, and were executed on August 6th.
The house at Skewis has been recently in part rebuilt, when a bag of the slugs used by poor Rogers was found.
It is in Crowan parish.
The church of S. Crewenna stands on a hill, and has a good tower. It contains numerous monuments of the S. Aubyn family, and some brasses only recently restored to the church, after having lain for many years lost or forgotten in a cupboard at Clowance.
It is hard to say whether the fulsome memorial of Sir John S. Aubyn, who died in 1839, is more painful or amusing reading to such as know his story.
The church has been “restored” in a cold and unsympathetic fashion.
Clowance, the seat of the Molesworth S. Aubyn family, has noble trees, and is an oasis in the midst of the refuse-heaps of mines. There are some early crosses in the grounds.
But to return to the Irish invasion.
A second party of the colonists was under Fingar or Gwinear, son of Olilt, or Ailill, probably one of the Hy Bairrche family, which was expelled their country about 480. He brought over with him his sister Kiara, whose name has become Piala of Phillack in Cornish, according to a phonetic and constant rule. According to the legend he had over seven hundred emigrants with him. He and his party made their way from Hayle to Connerton, where they spent the night, and then pushed south to where now stands Gwinear. Here Fingar left his party to go ahead and explore. He reached Tregotha, where is a fine spring of water, and there paused to refresh himself, when, hearing cries from behind, he hurried back, and found that Tewdrig, the Cornish king or prince, who lived at Riviere, on a creek of the Hayle river, had hastened after the party of colonists, and had fallen on them and massacred them. When Fingar came up Tewdrig killed him also. Piala, the sister, does not seem to have been harmed; and as in the long-run the Irish succeeded in establishing themselves firmly in the district, she settled near Riviere and founded the church of Phillack.
Ludgvan has a fine tower and some old crosses, the font also is early, of polyphant stone; but the church has been badly
churchwardenised and meanly restored. It was founded by Lithgean, or Lidgean, an Irish saint, son of Bronfinn or Gwendron. There is a representation of the mother in the rectory garden wall, where she is figured holding what is apparently a tree in one hand and in the other a fleur-de-lis.
Hereabouts the whole country is devoted to early potatoes and spring flowers. In March the fields are white with narcissus or golden with daffodil, or rich brown with the Harbinger wallflower. It is a curious fact that yellow wallflowers meet with no sale; consequently one kind only, and that dark, is grown.
The kinds of narcissus mostly grown are the Scilly White; of daffodils the Soleil d’Or, Grand Monarch, Emperor and Empress, Sir Watkin, and Princeps. These flowers are packed in baskets or boxes in bunches, a dozen blossoms in each bunch, and four dozen bunches in each basket. Women are employed to pick in the morning and to tie in bunches in the afternoon.
A special train takes up the flowers daily to London. The rate charged is £4 10s. per ton, but for fish £2 10s., as they take less room. The flower harvest lasts from February to June, and is followed by one of tomatoes.
Between Ludgvan and Perran-uthnoe intervenes the parish of S. Hilary. The church is devoid of interest, but there are inscribed stones in the churchyard. On the village inn may be read the invitation:—
“THE
JOLLY TINNERS.
“Come, all true Cornish boys, walk in, Here’s Brandy, Beer, Rum, Shrub, and Gin. You cannot do less than drink success
To Copper, Fish, and Tin.”
A local riddle asked is:—
“As I went down by Hilary’s steeple I met three people.
They were not men, nor women, nor children. Who were they then?”
The answer, of course, is one man, one woman, and a single child.
S. Michael’s Mount is a grand upshoot of granite from the sea. As a rock it is far finer than its corelative in Brittany, but the buildings crowning the Cornish mount are vastly inferior to the magnificent pile on Mont Saint Michel. Nevertheless, those that now form the residence of Lord S. Levan are by no means insignificant or unworthy of their position. The masses of granite crag, especially to the west, are singularly bold, and if some of the modern work be poor in design, it might have been much worse.
Within there is not much to be seen—a chapel of no great interest, and a dining-hall with good plaster-work representation of a hare hunt running round it. The drawing-room is new and spacious, and contains some really noble portraits.
At the foot of the rock is a draw-well, and a little way up is a tank called the Giant’s Well. S. Michael’s Mount was the habitation of the famous giant with whom Tom Thumb tried conclusions.
In or about 710, according to William of Worcester, an apparition of S. Michael the archangel was seen on the Tumbain Cornwall. This Tumba was also called Hore-rock in the Wood, “and there was formerly grove and field and tilled land between the Mount and the Scilly Isles, and there were a hundred and forty churches of parishes between the said Mount and Scilly that were submerged.... The district was enclosed by a most vast forest stretching for six miles in from the sea, affording a most suitable refuge for wild beasts, and in this were formerly found monks serving the Lord.”
It is quite true that there is a submerged forest in Mount’s Bay, and that the marshy snipe-ground near Marazion Road Station covers large timber, a portion of this great forest, but the submergence cannot have taken place in historic times. That there was, however, an encroachment of the sea in the middle of the sixth
century, we learn from the Life ofS. Paul, Bishop of Leon. He came to the bay to visit his sister, Wulvella, of Gulval, when she complained to him that she was losing much of her best land by the advance of the sea; and he, who had been brought up in the Wentloog levels, and taught by his master, S. Iltyd, how to keep up the dykes against the tides in the Severn, banked out the sea for her.
This was precisely the time when the district of Gwaelod was submerged in the Bay of Cardigan. The king of the district was Gwyddno Longshanks. It was the duty of the warden of the dykes to ride along the embankments, that had probably been thrown up by the Roman legionaries, and see that they were in order. Seithenyn was the Dyke-grave at the time.
One night Gwyddno and his court were keeping high revel, and the dyke-master was very drunk. There was a concurrence of a spring-tide and a strong westerly wind, and the waves overwhelmed the banks. The king escaped with difficulty before the inrolling stormy sea. A poem by the king, who thus lost his kingdom, has been preserved.
In Brittany about the same time there was a similar catastrophe.
In Mount’s Bay, however, an extraordinary tide may have done damage, but certainly did not cause such a submergence as was supposed by William of Worcester.
It has been supposed that the Mount is the Ictis of the ancients, which was the site of the great mart for tin, but this is more than unlikely. What would have been the advantage of making a market on this conical rock? It is much more likely that the great tin mart was in one of the low-lying islands of Kent.
Castel-an-Dinas commands an extensive view; it stands 763 feet above the sea, and is within sight and signalling distance of the two other similar castles on Trencrom and Tregonning. It is more perfect than either, and is very interesting, as it has got its wall with the face showing through the greater portion of the circuit. There were at
least two concentric rings of fortifications and numerous hut circles within the area, but these have been much pulled about when an absurd imitation ruinous tower was erected on the summit. Within the camp is a well, and outside it on the west side is one cut in the rock, to which a descent is made by about twenty steps.
On the side of the hill is the very interesting and indeed wonderful group of clustered huts called Chysauster. Of these there remain four distinct groups, two of which have been dug out. They consist of an open space in the midst, with numerous beehive huts and galleries running out of it.
The period to which they pertain is very uncertain. They ought to be investigated by such as are experienced and trained in excavation of such objects, and not be meddled with by amateurs. The tenant has begun (1899) to destroy one of the groups. In the centre of one of the huts may be seen neatly cut the socket-hole of the pole which sustained the roof, and in another the lower stone of the quern in which grain was pounded. There are other collections of a similar character, but none so perfect.
In the neighbourhood of Penzance are some of the “Rounds,” formerly employed for the representation of sacred dramas. They are, in fact, open-air amphitheatres. The well-known Gwennap Pit, in which John Wesley preached, has been mistaken for one of these, but was actually a disused mine-hole.
In these pits the miracle-plays in the old Cornish tongue were performed. Of these plays we have a few preserved, that have been printed by Professor Whitley Stokes. But the Cornish language ceased to be spoken, and after the Reformation religious plays ceased to be required. The people were learning the art of reading, and the press gave them the Bible, then these miracle-plays were replaced by low comedies, often very coarse in their humour, and spiced with many local allusions and personal jokes. This continued till Wesleyanism denounced stage-plays, and then these pits were devoted to revival-meetings and displays of hysterical religion. There
were two Rounds near Penzance, Tolcarre, and one at Castle Horneck.
Adjoining Penzance to the south is Newlyn, a fishing village formerly, now both a fishing village and a settlement of artists; for the advantage of the latter a good place of exhibition for their pictures has been provided by that generous-hearted son of Cornwall, who has done so much for his native county, Mr. Passmore Edwards.
Newlyn takes its name probably from S. Newlyna, whose church, founded on her own land, is near Crantock and Newquay. The name means the White Cloud. She migrated to Brittany, embarking, it may be supposed, at this port in Guavas Bay. She is a Breton replica of S. Winefred, for she had her head cut off by an admiring chieftain, whose affection was changed into anger at her resistance. In Brittany she has a fine church at Pontivy Noyala.
A cantique is sung there by the children, the first verse of which runs thus:—
“Deit, Créchénion, de gleuet Buhé caër Santes Noaluen, Ha disguet guet-he miret Hag hou fé hag non lézen,”
which means, “Come, ye Christians, hearken all, and hear the tale of S. Noewlyn. From her example learn to keep your faith and your innocence.”
S. Paul’s takes its name from a founder who was born in Glamorganshire, and was educated by S. Iltyd. He was schoolfellow with S. David, S. Samson, and Gildas. He is said to have gone to a King Mark, but whether this were the Mark, King of Cornwall of the romancers, the husband of the fair and frail Ysseult, we cannot be sure. He quarrelled with the king, and left him, because he was refused a bell in Mark’s possession, which he admired and asked for. He settled in Brittany, in Leon.
LAND’S END
THE LAND’S END
The Irish settlers in Penwith—Difference between Irish and Cornish languages—The Irish saints of Penwith—Other saints—Penzance— S. Ives—Restored brass—Wreck of Algerine pirates in 1760— Description of Penwith—The pilchard fishery—Song—Churches of the Land’s End—S. Burian—S. Paul’s and Dolly Pentreath The
Cornish language—Cornish dialect—Old churches and chapels— Madron—Prehistoric antiquities.
THE Land’s End is properly Penwith, either Pen-gwaed, the Bloody Headland, or Pen-gwaedd, the Headland of Shouting. Probably it is the former, for it was the last place of refuge of the Ibernian population, and in the first years of the sixth century, even perhaps earlier, it was occupied by Irish settlers, and that there was fighting is clearly shown us in the legend of SS. Fingar and Piala. It must have been to the original people of the peninsula what Mona was to the Welsh.
All we know about this invasion is what is told us in the legend just mentioned, and that states that Fingar, son of an Irish king, came to Hayle, landed there with his party, and was fallen upon by Tewdrig, the Cornish duke or king, who massacred some of the party. But the names of the parishes tell us more than that. They show us that the Irish were not defeated, that they made good their landing, and that they spread and occupied the whole of Penwith and Carnmarth, that is to say, the entire district of West Cornwall up to Camborne and the Lizard district.
The colonists cannot have been few, and they must have purposed settling, for they brought women along with them; and that they were successful is assured by the fact that those killed by Tewdrig are recognised as martyrs. Had the Irish been driven away they would have been regarded as pirates who had met their deserts.
Now this inroad of saints was but one out of a succession of incursions, and the resistance of Tewdrig marks the revolt against Irish domination which took place after the death of Dathi in 428, the last Irish monarch who was able to exact tribute from Britain; though Oiliol Molt may have attempted it, he was too much hampered by internal wars to make Irish authority felt in Britain. Oiliol fell in 483.
