History 193
May 5, 2014
Encephalitis Lethargica
The Spanish l! pan"e#ic in 191$ %as a great trage"y killing ro!ghly 50 #illion people&
'!t "!ring this ti#e %as another "isease #aking its %ay thro!gh the cro%"s leaving s!((erers in
a co#a like trance& )nlike the (l!, %hich is a kno%n vir!s, the ca!se o( the #ysterio!s *sleepy
sickness+ is still "e,ate"& -as it a vir!s relate" to the Spanish (l! or so#ething entirely
"i((erent. Encephalitis lethargica %as a ca!se" ,y a rare (or# o( streptococc!s an" an atypical
i##!ne response&
Encephalitis Lethargica is a #ysterio!s ,!t o(ten (orgotten plag!e& The "isease (irst
starte" in 191/ an" the epi"e#ic laste" !ntil a,o!t 192/ ,!t the e((ects laste" long a(ter& The (irst
cases %ere sol"iers (ighting in -orl" -ar 0 in rance& They le(t the ,attle(ront %ith %o!n"s or
ac!te illnesses ,!t arrive" to ne!ropsychiatry clinics in 1aris an" 2ienna %ith strange sy#pto#s&
The sol"iers %ere 3!st so sleepy& rench physician 4ean 5ene 6r!chet re#arks, *0t %o!l" have
see#e" al#ost serene at (irst7,lank, e8pressionless (aces, (ree o( terror an" pain, cal#ly asleep
in ro% a(ter ro% a(ter ro%& -hat %as #ost (rightening a,o!t these #en %as that they %o!l" not
%ake& 0t #!st have (elt like ,eing in a roo# (!ll o( the ,reathing "ea"&+ 96ros,y 11: 6r!chet
co!l" not (ig!re o!t i( these sy#pto#s %ere ca!se" ,y a ne% che#ical %ar(are ,!t they %ere not
like other %ar in3!ries& '!t not too long later in 2ienna, a civilian %as seen presenting si#ilar
sy#pto#s to those o( the sol"iers& *This sleepy, lethargic patient ha" nothing to "o %ith the %ar&
The physicians co!l" not i"enti(y %hat %as ,roken7there %as no physical tra!#a, no history o(
insanity, no single in(ection that ca!se" sy#pto#s like this&+ 96ros,y 13: ;n" a(ter this civilian
ca#e #ore an" #ore& The "isease starte" sho%ing !p in Englan" an" event!ally ;#erica& ;ll
ha" sol"iers co#ing ho#e (ro# the %ar ,!t it %as not only sol"iers ,eco#ing ill& This co!l" not
have ,een a %arti#e "isease& 0n ;pril o( 191<, 6onstantin von Econo#o o( 2ienna na#e" the
"isease encephalitis lethargica, si#ply #eaning *a s%elling o( the ,rain that #akes one sleepy&+
96ros,y 1$:
The Spanish l! pan"e#ic %as a tr!e killer& ro# 191$ to 1919, 50 #illion people
thro!gho!t the %orl" "ie"& =!ring this ti#e encephalitis lethargica %as also #aking a ,igger
sprea" thro!gho!t Englan" an" no% ;#erica& 0t see#e" pla!si,le that encephalitis lethargica
co!l" ,e so#e sort o( a(ter a((ect o( the (l!& Many cases starte" %ith (l!>like sy#pto#s& ever,
sore throat, hea"ache& This %as the case %ith ;"a#, a preparatory school ,oy %ho (ell ill %ith a
sore throat on his %ay ho#e (or Easter ,reak& His #other p!t hi# to ,e" an" *the (a#ily "octor
"iagnose" a light case o( the (l!&+ 96ros,y //: 0t %asn?t !ntil the ne8t "ay that he s!""enly
starte" acting o!t t%itching& The ne8t year he starte" having respiratory tics& He looke" like a
#an %ith 1arkinsonis#& He %o!l" conv!lse, "rool, an" %as constantly in pain& His (l! t!rne" o!t
to ,e encephalitis lethargica, %hich he s!((ere" sy#pto#s o( (or several years& ;t the ti#e
tho!gh, *the (a#ily ha" no reason to ass!#e it %as anything %orse than a case o( the (l!&+
96ros,y //: 5osie %as a yo!ng girl %ho once s!((ere" a nor#al strain o( the (l! ,!t %as le(t %ith
poor eyesight an" a li#p& The ne8t year she %as "iagnose" %ith 1arkinsonis# tho!gh her #other
note" she %as constantly tire"& 'y 192$ she ha" gro%n a tre#or in her ar# an" ha" a #ask>like
e8pression& Her personality ,eca#e violent& ;(ter%ar"s she %o!l" ,eco#e e8tre#ely lethargic&
She ha" a ten"ency (or sel(>har# even a"#itte" to tearing her o%n eyes o!t& ;ccor"ing to
ne!rologist =r& @liver Sacks, *these (or#s o( ,ehavior %ere #ost clearly an" !n"isg!ise"ly
#ani(est in chil"ren, %ho so#eti#es sho% a,r!pt changes o( character, an" s!""enly ,eco#e
i#p!lsive, provocative, "estr!ctive, a!"acio!s, salacio!s, an" le%", so#eti#es to a A!ite
!ncontrolla,le "egreeB s!ch chil"ren %ere o(ten la,ele" C3!venile psychopaths? or C#oral
a#ents&?+9Sacks 1<: )n(ort!nately it see#s that psychosis is !n!s!al (or in(l!enDa victi#s& 0t is
possi,le ,!t not %ell e8plaine"& *;ltho!gh it is kno%n that ; strains o( the vir!s can trigger 6ES
co#plications, the pathogenic #echanis#s ,ehin" s!ch co#plications are poorly !n"erstoo"&
Moreover, (e% #o"ern psychiatrists regar" in(l!enDa as a trigger (or psychosis& Fet (or so#e 30
years, (ro# the (irst epi"e#ics o( 5!ssian in(l!enDa in the 1$90s thro!gh to the *Spanish+
in(l!enDa o( 191$719, the *psychoses o( in(l!enDa+ %ere a %i"ely recognise" psychiatric
pheno#enon&+ 9Honigs,a!#: ;s it also t!rns o!t, in 2002, =r& 4ohn @8(or", a virologist, teste"
,rain tiss!e (ro# $ patients %ho ha" s!((ere" an" "ie" (ro# the "isease in 191$ as %ell as tiss!e
(ro# the ,rain o( a #an %ho ,eca#e ill in 1931 an" "ie" in 2002 (or evi"ence o( in(l!enDa& ;ll
ca#e ,ack negative&
-ar(are an" in(l!enDa "o not co#pletely e8plain %hat co!l" have ca!se" this epi"e#ic&
The Spanish (l! pan"e#ic "i" not ,reak !ntil 191$ ,!t cases o( encephalitis lethargica %ere seen
(irst in 191/, *the onset o( the epi"e#ic o( encephalitis lethargica see#s to have prece"e" the
in(l!enDa pan"e#ic, an" it contin!e" to rage (or at least ten years a(ter the in(l!enDa pan"e#ic
ha" rece"e"&+ 9=o,son $3: 0t %o!l" not #ake sense (or encephalitis lethargica to ,e a post>viral
"isease o( the (l! i( it starte" earlier& 0t %as (inally in 2004 that there %as a ,reakthro!gh to the
#ystery o( this strange "isease& =r& =ale 5!ssel sa% a patient %ho ,eca#e ill in 2000& She %as
an active %o#an ,e(ore ,eco#ing ill ,!t *she "i" not #ake a (!ll recovery an" no% nee"s (!ll
ti#e care (ro# her partner&+ 9''6 Ee%s: Seeing this strange illness #ake a co#e,ack, =r&
5!ssel ,egan research on the ca!se& -ith the help o( colleag!es he (o!n" 20 other recent cases
o( encephalitis lethargica& ;long %ith the help o( =r& ;n"re% 6h!rch, he ,egan st!"ying the
cases& @ne thing all the patients ha" in co##on %as a sore throat& -hen testing (or in(ection
they (o!n" all res!lts ca#e ,ack positive (or a rare (or# o( streptococc!s ,acteria, %hich ca!ses
strep throat& -hen they looke" over #e"ical recor"s (or patients o( encephalitis lethargica they
(o!n" that #any starte" %ith sore throats an" teste" positive (or "iplococc!s, a (or# o( the
streptococc!s ,acteria& *0t see#s that in so#e people the ,o"y has a #assive i##!ne reaction to
the streptococc!s ,acteria an" then t!rns on the ,o"y itsel(G attacking the ,rain an" "estroying
it&+ 9''6 Ee%s: 0t see#s to ,e the #ost pla!si,le that this ,acteria ,e the ca!se i( it is
#entione" in recor"s (ro# the original epi"e#ic an" is seen in #ore recent patients& -hat starts
o!t as a si#ple sore throat t!rns into a "evastating li(e sentence an" there is not #!ch that can ,e
"one to stop it&
The #ystery ,ehin" this "isease #ay never "isappear& The "isease itsel( has not as a(ter
the epi"e#ic o( 191/ to the 1920s there have contin!e" to ,e isolate" cases as recently as 200/&
The goo" ne%s is that tho!gh this "isease #ay contin!e to "evastate so#e, o!r #e"ical
technology has contin!e" to a"vance an" the recent st!"ies sho% pro#ise o( (ig!ring o!t once
an" (or all the tr!e ca!se o( encephalitis lethargica& @nce the ca!se is kno%n, the #ost i#portant
part can ,egin, (ig!ring o!t a c!re&
-orks 6ite"
6ros,y, Molly 6al"%ell& Asleep: The Forgotten Epidemic That Remains One of Medicine's
Greatest Mysteries& Ee% ForkB 'erkley, 2010& 1rint&
=o,son, Mary& HEncephalitis Lethargica&H Disease: The Extraordinary Stories ehind !istory's
Deadliest "illers& Lon"onB I!erc!s, 200<& $0>$3& 1rint&
Honigs,a!#, Mark& H*;n 0ne8pressi,le =rea"+B 1sychoses o( 0n(l!enDa at in>"e>SiJcle&H The
Lancet& E&p&, 23 Mar& 2013& -e,& 2$ ;pr& 2014&
KhttpBLL%%%&thelancet&co#L3o!rnalsLlancetLarticleL100S0140>/<3/M252$13M2529/0<01>
1L(!llte8tN&
HMystery o( the orgotten 1lag!e&H ''6 Ee%s& ''6, 2< 4!ly 2004& -e,& 2$ ;pr& 2014&
KhttpBLLne%s&,,c&co&!kL2LhiLhealthL3930<2<&st#N&
Sacks, @liver -& A#a$enings& Ee% ForkB Harper 1erennial, 1990& 1rint&