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Home , Bradyphrenia, Encephalitis lethargica, Polio, Viral encephalitis

Encephalitis lethargica Felix Stern (1884–1941). Source: Universitätsarchiv, Göttingen. Paul Bernard Foley

Encephalitis lethargica The mind and brain

123 Paul Bernard Foley Neuroscience Research Australia Sydney, NSW Australia and

Unit for History and Philosophy of Science University of Sydney Sydney, NSW Australia

ISBN 978-1-4939-0383-2 ISBN 978-1-4939-0384-9 (eBook) https://doi.org/10.1007/978-1-4939-0384-9

Library of Congress Control Number: 2016944489

Springer New York Heidelberg Dordrecht © Springer Science+Business Media New York 2018 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made.

Printed on acid-free paper

This Springer imprint is published by SpringerNature Dedicated to the memory of Felix Stern, and for Kay and Max Acknowledgements

As with my previous book, I commenced the work for this volume in ignorance of the dimensions of the story I wished to tell. I had indeed discovered encephalitis lethargica during my exploration of the history of for , and was tempted by the thought that a closer examination of this peculiar disorder might provide insights into the etiology of idiopathic Parkinson’s disease, and thus into potential new avenues for its management. I am no longer convinced that this is necessarily the case, but, on the other hand, a much broader, interesting picture developed in the course of my research, the picture of a multihued infectious neuropsychiatric disorder that presented an unprecedented coalescence of both neurologic and psychiatric symptoms that both underscored the unity of mind and movement in the CNS, and made clear the critical role played by subcortical structures in the elaboration of consciousness and other higher mental functions formerly attributed to the soul and more recently presumed to be localized to the human . Encephalitis lethargica exerted a greater influence on clinical and theoretic neuroscientific thought between the two World Wars than any other single disorder, and its impact upon contemporary and is still evident to those with the eyes to recognize it. In order to even approach doing justice to the many sig- nificant facets of the phenomenon encephalitis lethargica, however, required more time than initially envisaged: “one book always leads to another and another, and they all have something to say, there is something unhealthy about curiosity, not for the reasons usually given, but because it leads inex- orably to exhaustion” (Javier Marías, Your face tomorrow. I. and spear [Vintage Books, London, 2006], p. 119). Or, to paraphrase Bob Dylan, I needed to know my song well before I started singing. My research into the history of encephalitis lethargica and related dis- orders was funded successively by the Prince of Wales Institute (POWMRI; now Neuroscience Research Australia, NeuRA), a Post-Doctoral Research Fellowship from the Alexander von Humboldt Foundation (during which I was a guest researcher at the Institut für die Geschichte der Medizin in Würzburg, ), the Ramaciotti Foundation (Australia), the Australian Research Council (Postdoctoral Research Fel- lowship and Discovery Project (DP0451188), and the University of New

vii viii Acknowledgements

South Wales (Vice-Chancellor’s Postdoctoral Research Fellow). The prepa- ration of the book itself was facilitated by a two-year grant from the National Library of Medicine (NLM Grants for Scholarly Works in Biomedicine and Health; 1G13LM009863-01). Aspects of this work have been individually presented at scientific con- ferences throughout the course of my research, particularly at the annual meetings of the International Society for the History of the Neurosciences (ISHN). I am grateful for the discussions with friends and colleagues at these meetings that played an important role in clarifying details and concepts discussed in this book (as well as for providing encouragement that my efforts were at least intellectually worthwhile), and I am particularly grateful for the many fruitful exchanges with Joel Vilensky (Indiana University, Fort Wayne, USA), with whom I co-organized a symposium on encephalitis lethargica at the 2007 ISHN meeting in Los Angeles, and who invited me to contribute the chapter on historical encephalitis lethargica-like disorders to his volume on the disorder. I will not attempt a comprehensive listing of all those who have helped along the way (for of overlooking anyone at this late stage), but a few deserve special thanks. A number of libraries and archives provided invaluable assistance in locating and supplying material throughout the course of my research. I am particularly grateful to the Staatsbibliothek zu Berlin, both for its prompt and helpful replies to my many e-mailed enquiries, as well as for the friendly assistance I always received during my annual visits to the Unter den Linden library. I enjoyed visiting the new reading rooms in 2014, but somehow missed the labyrinthine corridors and musty smells that had become familiar on the way from the old entrance from Unter den Linden! The assistance provided by Ulrich Hunger (Universitätsarchiv Göttingen) and the Council for Assisting Refugee Academics (UK) in my search for information concerning Felix Stern is very much appreciated; a brief Stern biography is in preparation. The welcome afforded me by the Staats- archiv Chemnitz during my search for information on the former pediatric encephalitis lethargica made from a very pleasant and fruitful stay in their city, whereby the information provided by the SFZ Förderzentrum (Chem- nitz) maximized the value of this visit. I am also grateful to Hermine Hof- mann and Carola Lorenz (Klinika Bavaria, Kreischa) for taking the time to show me around what was formerly the Kreischa Sanatorium and discussing with me the fate of Heinrich von Witzleben, pioneer of the Bulgarian treatment for encephalitis lethargica in Germany. I also thank Professor Glenda Halliday (POWMRI/NeuRA), who provided intellectual and material help throughout the project; Russell Dale (Children’s Hospital, Westmead, Australia), who shared with his experience with contemporary cases of encephalitis lethargica-syndrome; the Sydney Theatre Company; and Maaike Mintjes (POWMRI), who was of great assistance during the early stage of material analysis and organization. I also thank my Berlin colleague Acknowledgements ix

Reinhard Horowski, who for many years has shared and supported my interest in the history of the neurosciences, for providing me with reprinted copies of many of the important EL monographs of the 1920s. Feci quod potui, faciant meliora potentes.

Paul Bernard Foley Sydney, January 2013 Contents

1 Introduction ...... 1 The fascination of encephalitis lethargica...... 2 The ‘sleeping disorder’ ...... 2 The protean disorder ...... 4 The world into which encephalitis lethargica was born . . . . . 5 The significance of encephalitis lethargica for the biomedical sciences of the 1920s...... 7 Learning from the previous ...... 9 The historical analysis of encephalitis lethargica ...... 9 Conclusion ...... 13

2 1915–1918: The origins of encephalitis lethargica ...... 15 The winter of 1916/17 in ...... 15 Wiesner’s streptococcus: the encephalitis identified? ...... 19 Other reports from Vienna...... 19 Not all quiet on the Western Front: René Cruchet and ‘epidemic ’...... 21 Was encephalitis lethargica really present in before 1918?...... 25 Other neurologic disorders on the Western Front ...... 28 The priority debate: Cruchet v. Economo ...... 30 Was encephalitis lethargica seen elsewhere before 1918? ...... 34 Did encephalitis lethargica come from the Far East? ...... 36 The first red herring: Australian X disease ...... 37 1918: Breakout in France ...... 40 Encephalitis lethargica crosses the Channel ...... 43 ‘Epidemic stupor’ ...... 45 ‘Epidemic encephalitis’ ...... 46 The first English overview: Report of an enquiry into an obscure disease, encephalitis lethargica...... 50 Encephalitis lethargica and epidemic ...... 55 Crookshank and the question of ‘’ ...... 59 Encephalitis lethargica in France at the close of 1918...... 63 Situation report: , Christmas 1918 ...... 65 Appendix: Early cases of encephalitis lethargica and purported encephalitis lethargica-like disease, 1890–1918 ...... 67

xi xii Contents

3 1919–1924: The international epidemic...... 83 The 1918/19 ...... 83 The medical conception of ‘influenza’ in 1918...... 84 Influenza encephalitis and the nona ...... 85 Neurologic phenomena of the 1918/19 influenza pandemic ...... 87 Conclusion ...... 88 1920: Encephalitis lethargica sweeps over Europe...... 89 Return to Vienna ...... 90 1919: The first German epidemic ...... 91 The 1920 epidemic: encephalitis lethargica out of Africa?. . . . 95 Göttingen and Felix Stern ...... 99 Encephalitis lethargica: the official response in Germany . . . . 101 France ...... 105 Gabrielle Lévy and post-encephalitic hyperkinetic symptoms ...... 107 Britain: the never ending story ...... 109 The 1924 epidemic: saving the worst for last...... 115 Poland: the land in the middle ...... 119 Elsewhere in Europe...... 120 Encephalitis lethargica in the USSR...... 123 Beginnings in the Ukraine ...... 124 Encephalitis lethargica as an all-Union problem ...... 127 Encephalitis lethargica in the Russian Far East ...... 131 Later cases in the European USSR ...... 134 Conclusion ...... 135 Encephalitis lethargica in North America ...... 136 Neglected Canada?...... 141 South America...... 142 Encephalitis lethargica in Asia ...... 143 The second red herring: ...... 144 Encephalitis lethargica in Africa ...... 146 Encephalitis lethargica in Australia and New Zealand ...... 146 Response at the international level ...... 150 Local precautions ...... 153 The name of the beast ...... 154 Official terminology ...... 158 Conclusion ...... 160 1925 ...... 160 Appendix: Summary of data regarding reported cases of encephalitis lethargica, as recorded in the annual epidemiological reports of the Health Section of the Secretariat of the League of Nations* ...... 161

4 The nature of acute encephalitis lethargica ...... 185 The prodrome and onset of encephalitis lethargica ...... 187 The mucous membranes during the prodromal period . . . . . 187 Onset of the disease proper ...... 189 Contents xiii

The ‘symptomatic triad’ of classic encephalitis lethargica (1917–1919)...... 192 Lethargy ...... 193 The oculomotor pareses of classic encephalitis lethargica . . . . 199 Other cranial -related symptoms...... 204 Fever...... 205 Other symptoms of classic acute encephalitis lethargica . . . . . 205 Parkinsonism in acute encephalitis lethargica ...... 209 The puzzles posed by parkinsonism during acute encephalitis lethargica...... 211 The ‘amyostatic symptom complex’ and encephalitis lethargica ...... 212 The 1920 epidemic: For we are many ...... 214 Hyperkinetic encephalitis lethargica ...... 217 ‘’ prior to 1920 ...... 221 Other abnormal movements in hyperkinetic encephalitis lethargica ...... 222 Dubini’s electric : Hyperkinetic encephalitis lethargica in 19th century Italy? ...... 223 Epidemic (singultus): red herring or clue? ...... 225 Epidemic hiccup and encephalitis lethargica ...... 229 The end of epidemic hiccup ...... 232 Abortive, rudimentary and larvate cases: the dark number of encephalitis lethargica cases ...... 233 Pathologic findings in encephalitis lethargica...... 238 Classification of the forms of acute encephalitis lethargica . . . . . 240 The splitters: the disintegration of encephalitis lethargica . . . . 242 Seeing the wood: parsimony in the classification of encephalitis lethargica...... 243 Neuropathological classifications of encephalitis lethargica . . . 249 Encephalitis lethargica: one disorder or a mélange of symptoms?...... 252 Diagnosis of encephalitis lethargica ...... 253 Prognosis in 1922 ...... 256

5 When night comes falling: chronic encephalitis lethargica ...... 275 Prognosis for those who survived acute encephalitis lethargica ...... 276 Assessing an uncertain future ...... 277 The interval between the acute and chronic phases of encephalitis lethargica...... 282 The nature of the interval: pseudo-neurasthenia ...... 286 Cryptogenic encephalitis lethargica ...... 288 Legal ramifications of the interval...... 290 Chronification of the interval? ...... 292 The symptoms of chronic encephalitis lethargica: an overview. . . 293 xiv Contents

Pathological characteristics of chronic encephalitis lethargica ...... 294 Post-encephalitic parkinsonism (the amyostatic syndrome) . . . . 295 The nature of post-encephalitic parkinsonism ...... 300 The two major motor symptoms of post-encephalitic parkinsonism: hypertonia and bradykinesia–akinesia . . . . . 305 in post-encephalitic parkinsonism...... 312 ...... 314 in chronic encephalitis lethargica? ...... 320 Pathophysiology of post-encephalitic parkinsonism ...... 321 Parkinson disease v. post-encephalitic parkinsonism ...... 322 Hyperkinesias and in chronic encephalitis ...... 325 Oculogyric crises ...... 332 Nature of the ...... 335 The pathogenesis of oculogyric crises ...... 344 ‘Subcortical attacks’ ...... 350 Therapy of oculogyric crises ...... 354 Oculogyric crises after 1930 ...... 355 Respiratory symptoms in chronic encephalitis lethargica ...... 356 Other motor symptoms in chronic encephalitis lethargica . . . . . 359 Oculomotor symptoms (other than oculogyric crises) ...... 361 Unusual forms of chronic encephalitis lethargica ...... 363 The non-motor symptoms of chronic encephalitis lethargica . . . 367 Sleep disturbances in chronic encephalitis lethargica ...... 367 The vegetative symptoms of chronic encephalitis lethargica. . . 369 Vegetative symptoms without parkinsonism ...... 373 Endocrine symptoms in chronic encephalitis lethargica . . . . . 373 Sensory disturbances in chronic encephalitis lethargica . . . . . 374 Outlook: social aspects of chronic encephalitis lethargica ...... 377 The treatment of encephalitis lethargica ...... 378 The longer term fate of encephalitis lethargica patients . . . . . 380

6 The psychiatry of encephalitis lethargica ...... 407 Psychiatric aspects of acute encephalitis lethargica ...... 408 The pseudo-neurasthenic phase in adults ...... 411 Children and encephalitis lethargica ...... 413 Agrypnia: nocturnal excitation crises...... 413 Persistent ‘change of character’ in children with encephalitis lethargica ...... 415 and general features of the behavioral syndrome...... 417 The specific features of the behavioral syndrome...... 422 What was wrong with encephalitis lethargica children? . . . . . 431 Was intelligence damaged by encephalitis lethargica?...... 443 Prognosis for children with behavioral problems ...... 446 Post-encephalitic children as social problems: England and Wales ...... 449 Institutions for encephalitis lethargica children in other countries...... 456 Contents xv

Encephalitis lethargica behavioral syndrome and ‘moral insanity’ ...... 461 Encephalitis lethargica children and the law ...... 464 Postscript: Attention deficit disorders ...... 478 Psychiatric symptoms during the chronic phase in adults...... 482 Disturbances of volition: loss of drive ...... 482 The internal experience of encephalitis lethargica ...... 486 Other psychiatric features of chronic encephalitis lethargica in adults ...... 493 Predisposition to psychiatric symptoms in encephalitis lethargica ...... 498 Encephalitis lethargica and hysteria...... 499 Encephalitis lethargica, , and the ...... 507 Background: Psychoses as brain disorders before encephalitis lethargica ...... 509 What is schizophrenia? ...... 511 The somatic symptoms of schizophrenia ...... 512 Catatonia and chronic encephalitis lethargica ...... 513 Comparing schizophrenia and encephalitis lethargica ...... 522 Localizing schizophrenia...... 532 Why did the debate generate so much interest?...... 534 Symptomatic schizophrenia after the Second World War. . . . 535 Encephalitis lethargica and the localization of the psyche...... 538 Conclusion: Encephalitis lethargica as a chronic neuropsychiatric disorder ...... 545

7 The neuropathology of encephalitis lethargica...... 573 ‘Encephalitis’ before encephalitis lethargica ...... 575 What is ‘encephalitis’?...... 577 1917/18: Economo defines encephalitis lethargica ...... 578 The neuropathology of encephalitis lethargica proves its most consistent feature...... 580 Meningeal involvement in acute encephalitis lethargica . . . . . 581 Macroscopic brain changes in acute encephalitis lethargica . . . 581 Toxic and degenerative changes in acute encephalitis lethargica ...... 582 The reaction in acute encephalitis lethargica. . . 584 The significance of glial proliferation: background ...... 587 Glial proliferation in encephalitis lethargica...... 589 Localization of the acute encephalitis lethargica process . . . . . 596 The destruction of the substantia nigra: the hallmark of encephalitis lethargica neuropathology...... 599 Spatz, encephalitis lethargica and the extrapyramidal motor system ...... 604 Dissemination of the pathologic process in encephalitis lethargica ...... 608 xvi Contents

Was the cerebral cortex always spared by acute encephalitis lethargica? ...... 612 ...... 613 Neuropathology and specific symptoms of encephalitis lethargica ...... 616 Encephalitis lethargica and the ‘sleep problem’ ...... 618 Relationship of acute encephalitis lethargica with known forms of encephalitis ...... 626 Strümpell–Leichtenstern encephalitis and encephalitis lethargica ...... 626 Wernicke encephalitis and encephalitis lethargica ...... 629 Polio and encephalitis lethargica ...... 629 Neuropathology of the interval ...... 629 Consequences: neuropathology of chronic phase encephalitis lethargica...... 630 The cerebral cortex in chronic encephalitis lethargica ...... 632 Neuropathology and parkinsonian symptoms in chronic encephalitis lethargica...... 635 Neuropathology of oculogyric crises and respiratory symptoms ...... 638 Vegetative symptoms: brainstem centres, pituitary and ...... 639 Neuropathology of chronic encephalitis lethargica in children ...... 640 Chronic encephalitis lethargica or post-encephalitis lethargica? ...... 641 Progressive parenchymal degeneration: Jakob and Pette . . . . . 642 Chronic : Spatz ...... 643 Brain imaging of encephalitis lethargica ...... 652 The electroencephalogram (EEG) in encephalitis lethargica . . . . 653 Chronic encephalitis lethargica and the liver ...... 654 Other internal organs...... 656 Coda: the Viennese analysis of the parkinsonian brain (1960–1973) ...... 656 Bringing it all together: Encephalitis lethargica as the prototype of neuroinfections of the ...... 657 Three key puzzles ...... 659 Appendix: Overview of major encephalitis lethargica neuropathological reports ...... 662

8 What caused encephalitis lethargica?...... 683 Toxin theories of encephalitis lethargica ...... 683 Buscaino and the biochemistry of encephalitis lethargica . . . . 684 Encephalitis lethargica, the infectious disease...... 686 Encephalitis lethargica and microbiology in the 1920s ...... 687 Contents xvii

Who contracted encephalitis lethargica? ...... 689 Predisposing factors ...... 692 The increasing susceptibility of the central to attacks of epidemic disease ...... 695 Terrain or constitution in encephalitis lethargica ...... 696 How contagious was encephalitis lethargica? ...... 701 Mass outbreaks and the role of abortive and silent ...... 703 Objections to the primacy of the virus as determinant of epidemiology ...... 708 How did one contract encephalitis lethargica? ...... 709 How ‘neurotropic’ was the encephalitis lethargica virus? . . . . 713 Was encephalitis lethargica an aberrant form of a known human disease? ...... 714 The first specific candidate: Wiesner’s diplostreptococcus...... 716 Rosenow’s streptococcus...... 718 Trypanosoma in the Saarland? ...... 720 Filter-passing in encephalitis lethargica: the herpes connection ...... 721 The Institut Pasteur group: Levaditi, Harvier and the herpes virus...... 722 The Swedish ‘EL virus’ and rabbit encephalitis ...... 730 The Australian virus...... 733 The encephalitogenic herpes virus: the dominant model, but mounting doubts ...... 733 The neuropathological case against the herpes virus ...... 734 Why was the encephalitis lethargica virus so difficult to find? ...... 736 Jahnel’s criticism of the herpes–encephalitis virus identity hypothesis (1925) ...... 738 The end for the identity hypothesis ...... 740 Human herpes encephalitis today ...... 742 The complex virus hypothesis...... 743 Was encephalitis lethargica contracted from animals? ...... 745 : the equine sleeping sickness ...... 746 Red herrings or clues? New forms of encephalitis from 1924 . . . 747 Post- encephalitis/encephalomyelitis (PVE)...... 748 Post-infection encephalitis/encephalomyelitis (PIE) ...... 754 Conclusion ...... 761 Last thoughts before encephalitis lethargica disappears...... 761

9 After the storm...... 781 Encephalitis lethargica at the end of the 1920s ...... 782 Polio and encephalitis lethargica: one last time ...... 785 Diagnostic guidelines after the epidemic ...... 785 What wasn’t encephalitis lethargica? ...... 787 Successor disorders? ...... 788 Neurasthenic states ...... 788 Disseminated encephalomyelitis ...... 789 xviii Contents

Aseptic or serous ...... 791 Further candidate successor disorders ...... 792 Conclusion ...... 793 The -borne viral encephalitides ...... 794 Post-1945 ‘encephalitis lethargica’: and parkinsonism...... 797 Bickerstaff brainstem encephalitis ...... 800 The Howard–Lees criteria for encephalitis lethargica ...... 801 Encephalitis lethargica: an auto-?...... 805 Chronic encephalitis lethargica in the medical literature after 1945 ...... 808 The dopamine connection ...... 812 Relevance to idiopathic Parkinson disease ...... 815 Conclusion ...... 816 Appendix 1: Cases reported as encephalitis lethargica, 1945–1985 ...... 819 Appendix 2: Cases reported as encephalitis lethargica, 1985–2015 ...... 824

10 The fading trail of the sleepy wraith ...... 839 Encephalitis lethargica on film ...... 839 Encephalitis lethargica in non-medical literature and film . . . . . 841 Awakenings ...... 844 Encephalitis lethargica in more recent literature ...... 847 The end...... 850

The most important encephalitis lethargica monographs published during or immediately after the epidemic period . . . . . 857 References ...... 859 Index ...... 1059. Illustrations

Figure 2.1 (1876–1931), 1924 ...... 16 Figure 2.2 René Cruchet (1875–1959) ...... 22 Figure 2.3 Report of an enquiry into an obscure disease, encephalitis lethargica ...... 50 Figure 2.4 Arthur Salusbury MacNalty (1880–1969) ...... 52 Figure 3.1 Arnold Netter (1855–1936) ...... 98 Figure 3.2 Encephalitis in Göttingen, 1917–26 ...... 102 Figure 3.3 Alexandre-Achille Souques (1860–1944)...... 106 Figure 3.4 Encephalitis lethargica in England and Wales, 1919–27 ...... 116 Figure 3.5 Encephalitis lethargica in Europe ...... 121 Figure 3.6 Mikhail Semenovich Margulis (1879–1951) ...... 129 Figure 4.1 Comparative frequency of initial symptoms according to Parsons et al. 1922 ...... 188 Figure 4.2 Encephalitis lethargica, somnolent form ...... 193 Figure 4.3 Encephalitis lethargica, hyperkinetic form ...... 216 Figure 4.4 Acute encephalitis lethargic cases received by the Göttingen Encephalitis Ward, 1917–1927, according to clinical type ...... 246 Figure 5.1 Post-encephalitis lethargica amyostatic syndrome in adults ...... 296 Figure 5.2 Post-encephalitis lethargica amyostatic syndrome in a child ...... 301 Figure 5.3 Severe contractures and cachexia in chronic encephalitis lethargica ...... 307 Figure 5.4 Oculogyric crises in encephalitis lethargica ...... 333 Figure 6.1 Kleist’s model of the role of the in psychomotor function ...... 540 Figure 7.1 Envelopment of a pigmented nigral cell by ...... 586 Figure 7.2 Typical glial rosette in encephalitis lethargica in acute cross-section and overview ...... 590 Figure 7.3 Normal substantia nigra ...... 603 Figure 7.4 Substantia nigra in chronic encephalitis lethargica, showing reduction and depigmentation of the ‘black zone’ ...... 603 Figure 7.5 Hugo Spatz (1888–1969), 1928 ...... 605

xix xx Illustrations

Figure 7.6 Distribution of inflammation in the mesencephalon in encephalitis lethargica ...... 610 Figure 7.7 Economo’s localization of the sleep centre ...... 621 Figure 7.8 Neurofibrillary tangles in the substantia nigra of chronic encephalitis lethargica ...... 647 Figure 8.1 (1874–1953) ...... 722 Figure 8.2 Levaditi’s concept of the ‘ectodermoses neurotropes’. . . . 724 Figure 8.3 Robert Doerr (1871–1952)...... 725 Tables

Table 3.1 Encephalitis cases registered in the USSR and the Baltic states, 1921–1938 ...... 137 Table 4.1 Selected data regarding mortality of encephalitis lethargica during the acute phase...... 191 Table 4.2 The genius epidemicus of encephalitis lethargica ...... 241 Table 5.1 Length of the interval between acute and chronic encephalitis lethargica ...... 285 Table 5.2 Comparison of symptoms (as percentages) in post-encephalitic parkinsonism and sporadic Parkinson disease in 1965 ...... 318 Table 8.1 Age distribution of registered EL cases in four England–Wales , and in four reports including large case numbers, expressed as percentages...... 690 Table 8.2 “Recent illness or condition which may have lowered the patient’s resisting power, or otherwise predisposed him to the present illness”: summary of responses on enquiry schedules in England and Wales collated by Parsons et al., 1922 ...... 693 Table 8.3 Encephalitis lethargica virus strains isolated 1920–1925. . . 734 Table 8.4 Cases of post-vaccination encephalitis, according to the report of the Office International d’Hygiène Publique ...... 755

xxi Abbreviations

AD Alzheimer disease ADEM acute disseminated encephalomyelitis AIDS acquired immunodeficiency virus ALS amyotrophic lateral sclerosis CNS CFS chronic syndrome CMV CSF cerebrospinal fluid EBV Epstein-Barr virus EL encephalitis lethargica GStA Geheimes Staatsarchiv (Preußischer Kulturbesitz), Berlin HHV human herpes virus HIV human immunodeficiency virus HSV JEV Japanese encephalitis virus LCMV lymphocytic choriomeningitis virus LGB Local Government Board MAO monoamine oxidase NMDA N-methyl-D-aspartic acid OC oculogyric crisis PANDAS pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections PD Parkinson disease PEP post-encephalitic parkinsonism PVFS post-viral fatigue syndrome SARS severe acute respiratory syndrome TBE tick-borne encephalitis USPHS United States Service VZV

xxiii Nature is nowhere accustomed more openly to display her secret mysteries than in cases where she shows traces of her workings apart from the beaten path; nor is there any better way to advance the proper practice of medicine than to give our minds to the discovery of the usual law of nature, by the careful investigation of cases of rarer form of disease. For it has been found in almost all things, that what they contain of useful or of applicable, is hardly perceived unless we are deprived of them, or they become deranged.

William Harvey, letter dated 24 April 1657 (Works [London: The Sydenham Society, 1847], pp. 616f.)

The desire for a separation of neurology from psychiatry strikes me as being the same as requiring that a violinist play only the G and D strings because the A and E strings are reserved for other musicians … The unity of the nervous system must be reflected by the unity of the science concerned with it.

Adolf von Strümpell (1924) Gedanken über die Entwicklung und die ferneren Aufgaben der Neurologie. Deutsche Zeitschrift für Nervenheilkunde 81: 62–68.