Encephalitis lethargica: the mind and brain virus
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New York ; Heidelberg ; Dordrecht ; London
Springer
[2018]
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adam_text | 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 epidemic.............................. 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 Vienna....................................... 15
Wiesner’s streptococcus: the encephalitis pathogen
identified?..................................................... 19
Other reports from Vienna.......................................... 19
Not all quiet on the Western Front: René Cruchet
and ‘epidemic encephalomyelitis .................................. 21
Was encephalitis lethargica really present in France 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 polio......................... 55
Crookshank and the question of ‘influenzas’........................ 59
Encephalitis lethargica in France at the close of 1918........... 63
Situation report: Europe, 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..........................
The 1918/19 influenza pandemic.................................
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
Gottingen 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: Japanese encephalitis.................. 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
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 xiïi
The ‘symptomatic triad’ of classic encephalitis lethargica
(1917-1919)......................................................... 192
Lethargy......................................................... 193
The oculomotor pareses of classic encephalitis lethargica .... 199
Other cranial nerve-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
‘Myoclonus’ prior to 1920 ....................................... 221
Other abnormal movements in hyperkinetic encephalitis
lethargica..................................................... 222
Dubini’s electric chorea: Hyperkinetic encephalitis lethargica
in 19th century Italy?......................................... 223
Epidemic hiccup (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
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
Tremor in post-encephalitic parkinsonism...................... 312
Bradyphrenia.................................................. 314
Catatonia in chronic encephalitis lethargica?................. 320
Pathophysiology of post-encephalitic parkinsonism................ 321
Parkinson disease v. post-encephalitic parkinsonism........... 322
Hyperkinesias and dyskinesias in chronic encephalitis............ 325
Oculogyric crises................................................ 332
Nature of the oculogyric crisis............................... 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
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
Epidemiology 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, schizophrenia, and the brainstem........ 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
psychosis...................................................... 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
neuropsychiatrie disorder...................................... 545 7
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 inflammation 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
Inclusion bodies............................................... 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
Striimpell-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 hypothalamus............................................. 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 infection: 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 grey matter.......................... 657
Three key puzzles.............................................. 659
Appendix: Overview of major encephalitis lethargica
neuropathological reports.................................... 662 8
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 nervous system
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
infections.................................................... 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 viruses 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
JahnePs 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
Borna disease: the equine sleeping sickness..................... 746
Red herrings or clues? New forms of encephalitis from 1924 . . . 747
Post-vaccination 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
xvüi
Contents
Aseptic or serous meningitis................................... 791
Further candidate successor disorders.......................... 792
Conclusion..................................................... 793
The arthropod-borne viral encephalitides......................... 794
Post-1945 ‘encephalitis lethargica’: viral encephalitis
and parkinsonism............................................... 797
Bickerstaff brainstem encephalitis............................. 800
The Howard-Lees criteria for encephalitis lethargica........... 801
Encephalitis lethargica: an auto-immune disorder?................ 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
Paul Bernard Foley
Encephalitis lethargica
The mind and brain virus
Encephalitis lethargica (‘sleeping sickness’) was a mysterious disorder that swept the
world in the decade following the First World War, before disappearing without its cause
having been identified. Around 85% of its victims, predominantly children, adolescents
and younger adults, survived the acute disorder, but most developed severe neurological
syndromes, particularly severe post-encephalitic parkinsonism and other severe motor
abnormalities, that incapacitated them for the remainder of their lives.
Despite its brief history, encephalitis lethargica played a major role in a variety medical
discussions between the two World Wars, as this epitome of neuropsychiatric disease
- attacking both motor and mental functions - appeared just as the separation of neu-
rology and psychiatry had reached a critical point. Encephalitis lethargica sufferers
presented an unprecedented combination of neurologic and psychiatric symptoms
- including previously puzzling phenomena primarily associated with schizophrenia
and hysteria, as well as behavioral changes and attention deficit disorders in children
- that not only underscored the,unity of mind and movement in the CNS, but also il-
luminated the critical role played by subcortical structures in consciousness and other
higher mental functions that had formerly been associated with the soul and more
recently presumed to be localized to the human cerebral cortex. Encephalitis lethargica
exerted a greater influence on clinical and theoretic neuroscientific thought between
the two World Wars than any other single disorder, and had an enduring impact upon
neurology and psychiatry.
This book will be of interest to an educated audience active or interested in clinical
(neurology, psychiatry, psychology) or laboratory neuroscience, particularly those
interested in neuropsychiatry, as well as to those interested in the history of the bio-
medical sciences.
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publisher | Springer |
record_format | marc |
spelling | Foley, Paul Bernard 1961- Verfasser (DE-588)123742013 aut Encephalitis lethargica the mind and brain virus Paul Bernard Foley New York ; Heidelberg ; Dordrecht ; London Springer [2018] xxiii, 1083 Seiten Illustrationen txt rdacontent n rdamedia nc rdacarrier Encephalitis epidemica (DE-588)4152134-1 gnd rswk-swf Encephalitis epidemica (DE-588)4152134-1 s DE-604 Erscheint auch als Online-Ausgabe 978-1-4939-0384-9 Digitalisierung UB Regensburg - ADAM Catalogue Enrichment application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=027596501&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis Digitalisierung UB Regensburg - ADAM Catalogue Enrichment application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=027596501&sequence=000002&line_number=0002&func_code=DB_RECORDS&service_type=MEDIA Klappentext |
spellingShingle | Foley, Paul Bernard 1961- Encephalitis lethargica the mind and brain virus Encephalitis epidemica (DE-588)4152134-1 gnd |
subject_GND | (DE-588)4152134-1 |
title | Encephalitis lethargica the mind and brain virus |
title_auth | Encephalitis lethargica the mind and brain virus |
title_exact_search | Encephalitis lethargica the mind and brain virus |
title_full | Encephalitis lethargica the mind and brain virus Paul Bernard Foley |
title_fullStr | Encephalitis lethargica the mind and brain virus Paul Bernard Foley |
title_full_unstemmed | Encephalitis lethargica the mind and brain virus Paul Bernard Foley |
title_short | Encephalitis lethargica |
title_sort | encephalitis lethargica the mind and brain virus |
title_sub | the mind and brain virus |
topic | Encephalitis epidemica (DE-588)4152134-1 gnd |
topic_facet | Encephalitis epidemica |
url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=027596501&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=027596501&sequence=000002&line_number=0002&func_code=DB_RECORDS&service_type=MEDIA |
work_keys_str_mv | AT foleypaulbernard encephalitislethargicathemindandbrainvirus |