Aprils 1956. P H Y S I O T H E R A P Y Page Seven Observations on Neuromuscular Dysfunction in the Addington Outbreak By J. STRUAN ALEXANDER, M.B., B.S. (Lond.), M.R.C.S., L.R.C.P., D. Phys. Med. Durban. (R e p rin te d fro m th e S o u th A fric a n M e d ic a l J o u r n a l, V ol. 30, N o . 4.) T H IS m e m o ra n d u m will discuss th e n e u ro m u s c u la r dysfunction w hich o c c u rre d in th e m a jo rity o f cases a f f e c t e d by th e so-called A d d in g to n disease. A s an a p p ro a c h to so lim ited a field it is d e sirab le to c o n sid e r briefly c ertain relevant clinical fe atu res. In tro d u c tio n . In th e e arly m o n th s o f 1955, a n d m erging into th e ta il e nd o f a n e p idem ic o f p o lio m y elitis, a b o u t 90 nurses a t th e A d d in g to n H o s p ita l, D u r b a n , a n d 50 civilian cases w ere a d m itte d to h o s p ita l suffering fro m a polio-like disease. A s th e n a tu r e o f th e disease u n fo ld e d it was realized th a t cases o f a sim ila r c h a ra c te r h a d o c cu rre d sporadically fo r som e m o n th s b e fo re th e m a jo r o u tb re a k . N ature o f the Disease. A te n ta tiv e d e sig n a tio n o f e n ­ cephalom yelitis is c o n v en ien t. A s in p o lio m y elitis, o n se t was a b ru p t, w ith c o n s titu tio n a l sy m p to m s a n d m o to r paresis. B u t a t th e sam e tim e , p a in w as a p ro m in e n t sym ptom , u sually p e rip h e ra l a n d in m an y cases p ro tra c te d and severe. M o re o v e r, th e p a resis d id n o t c o n fo rm to a lo w e r-m o to r-n eu ro n lesion. Clinical Features. C o n s titu tio n a l sy m p to m s included headache, p ro s tra tio n , m ala ise a n d m ild p yrexia. M ild em otional d iso rd e r w as u su a lly expressed as e u p h o ria , but m ental d e p ressio n w as a lso n o te d , p a rtic u la rly in th e later c h ro n ic stages, in w hich p e rio d re a d y fa tig u e a n d inability t o c o n c e n tra te w ere a lso evident. M o to r involve­ ment re su lte d in p a resis o f o n e o r m o re lim bs. P a in was liable to have a d is trib u tio n sim ila r to th e c o ex isten t paresis. A c ard in al a n d c o n fu sin g fa c to r h a s b een th e v a ria b le n a tu re o f th e disease, b o th in te rm s o f severity a n d a lso in term s o f th e d is trib u tio n o f lesio n s in th e selective focal points o f th e c e n tra l n e rv o u s system . T h u s, e ith e r c o n sti­ tu tio n a l sy m p to m s, o r m o to r sy m p to m s, o r sensory sym ptom s m ig h t p re d o m in a te , a n d conversely o n e o r m o re o f these c o m p o n e n ts m ig h t be m in im a l o r a b se n t. I t is c lear th e re fo re t h a t th e m a jo r p re se n tin g signs a n d sym ptom s a re su b je ct to c o n sid e ra b le v a ria tio n . \ Course o f the D isease. O n set h a s fo r th e m o s t p a r t been a b ru p t. O n th e c linical evidence it w o uld a p p e a r th a t p atien ts p ro g ress th ro u g h 3 fa irly c learly defined stages, viz. th e a c u te o r activ e stage, th e c o n v alesce n t stage, a n d the c h ro n ic stage. I t m u s t be em p h a sise d th a t th e d u ra tio n o f these p h a se s is v a ria b le , a n d h a s b e en closely re la te d to the severity o f th e sy m p to m s. I n m ild cases sy m p to m s have com pletely c le are d u p a fte r a w eek o r tw o ; b u t in m any p a tie n ts sy m p to m s h ave sm o u ld ere d fo r a s lo n g as 3 m o n th s , a n d tr u e relapses h a v e o c c u rre d a s la te as 10 weeks a fte r o n se t. A fte r su b sid e n c e o f th e a ctiv e p h a se , there has been r a p id a n d a t tim e s d ra m a tic im p ro v e m en t. T oday, som e 4 m o n th s since th e first cases w ere notified, a residual c o re o f p a tie n ts re m a in s h o sp ita liz e d , o r physically ha n d ic ap p e d , a n d th e lo n g -te rm p ro g n o sis c a n n o t a t th is stage be firm ly e stablished. Relapses. I t is suggested t h a t re la p se s have been o f tw o different types. F irstly , a m in o r set-back o f a tra n s ie n t na tu re , in w hich th e p a tie n t ra p id ly re tu rn s to th e p re ­ relapse level. A sim ilar p h e n o m e n o n is k n o w n t o o c cu r d u rin g th e c o n v alesce n t sta g e o f p o liom yelitis a n d is no m ore th a n fa tig u e re a c tio n . In th e second ty p e o f relapse there is evidence o f renew ed a ctiv ity o f th e c a u sa tiv e p a th o ­ logical process. W e h a v e seen, fo r e xam ple, severe p a in , c o m p le te p a raly sis a n d c o ld sw eating skin in lim bs w hich h a d a lm o st re tu rn e d to n o rm a l. H ith e rto u n affected lim bs h a v e also b ecom e affected. I n th is ty p e o f re la p se re co v e ry h a s follow ed th e ta r d y a n d e rra tic c o u rse o f a n in itia l a tta c k . I t is n a tu r a l th a t m a n y fa c to rs h a v e been in c rim in a te d as c a u s a tiv e o f th e s e severe relapses, b u t in so m e instances th e y o c c u rre d in p a tie n ts u n d e r c o n d itio n s o f s tric t b ed-rest. H y ste ria . A s th e re h a v e b e en o v e rt a n d c o v e rt references to hy ste ria , th is su b je ct m e rits b rief discussion. M ass h y ste ria is surely a n u n te n a b le h y p o th esis in view o f th e c o n siste n t p a tte rn o f signs a n d sy m p to m s o c cu rin g in cases c o m in g fro m w idely sc a tte re d p o in ts in th e D u rb a n a re a . E n ce p h alitic sy m p to m s such as e u p h o ria , m e n ta l d e p ressio n , fa tig u ab ility , etc. have b e en a p ro m in e n t fe a tu re , th e lo c o m o to r d isability is o f a co m p le x n a tu re a n d p aresis is re la te d t o v o litio n a l in itia tio n o f m o v em e n t. T hese c o n sid e ra tio n s c o m p lic a te a sa tis fa c to ry assessm ent o f h ysteria. I n m an y cases a p sy chogenic o v e rla y c o u ld n o t b e ru led o u t. N eurom uscular D ysfunction. S om e degree o f m uscle w eakness h a s b een p re se n t in th e m a jo rity o f cases, o n e o r m o re lim bs b e ing involved, a n d th e legs being affected m o re o fte n th a n th e a rm s. I n a m in o rity o f cases, p a re sis has o c c u rre d in m uscles o f th e b a c k , a b d o m e n a n d neck. D is trib u tio n h a s been a sy m ­ m etrical. A t o n se t p a resis w as severe a n d w id e sp rea d , ten d in g to involve a ll th e jo in ts in a lim b ra th e r th a n selected m uscle-groups. In a few p a tie n ts sp a sticity w as p re se n t. W ith th e recessio n o f th e a c u te a ctiv e sta g e re co v e ry o f m o v em e n t h a s been ra p id f o r a few days. I n m ild p a resis th e im p ro v e m en t m ig h t b e d ra m a tic a n d c o m p le te , b u t in th e m o re severely p a re tic th is recessio n o f sy m p to m s has c o m e to a h a lt a t a p o in t s h o r t o f full recovery, a n d b e y o n d th is level p ro g ress h a s been ta rd y a n d su bject to relapses. W hen th e in itia l diffuse w eakness h a s receded in th e low er lim b, p aresis h a s re m a in e d m a in ly in th e h ip flexors, knee ex te n so rs, a n d a n k le dorsiflexors. I n th e w a rd s the u n s u p p o rte d f o o t in a p o s itio n o f e q u in o v a ru s w as fam ilia r, a n d fo o t-d ro p h a s been a p ra c tic a l difficulty in m a n y a m b u ­ la n t cases. T h e p a rtic u la r p a tte rn o f m o v em e n t h a s b een sufficiently c o n s ta n t to fo rm a useful g uide to diagnosis. F o r e xam ple, w ith th e p a tie n t lying su p in e a n d try in g to raise th e stra ig h t leg, th e re is a n e gative p h a se in w hich n o m o v em e n t ta k e s p lace. A s h ip flexion g ets g o in g it does so in je rk y fa sh io n , w ith a c o u rse tre m o r o f th e w hole lim b. W h en th e m a x im u m ra n g e o f h ip flexion h a s been estab lish e d , it is fo u n d th a t th e p o w e r o f th e m uscle is g o o d a n d is a b le to resist c o u n te r-p re s su re . H o w ev e r, if resis­ ta n c e is a p p lie d a t th e o u ts e t, a light finger p re ssu re is suffi­ c ie n t to p re v e n t a ctive m ovem ent. R eflexes h a v e fo r th e m o s t p a r t show n little c h a n g e ; possibly th e y h a v e been slightly d epressed, a n d in p a tie n ts show ing sp asticity c learly in creased k n e e-jerk s h a v e been o b se rv ed a n d h a v e pe rsisted . W astin g h a s b e en c o n sp ic ­ u o u sly m in im al even a fte r p ro lo n g e d b ed rest. E lectro-D iagnosis. F u ll in v estig a tio n s b y c u rre n t m e th o d s o f s tre n g th d u ra tio n curves a n d a c c o m m o d a tio n r a tio s w ere d o n e o n o v e r 20 o f th e nurses. A sm a lle r g ro u p R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) Page Eight P H Y S I O T H E R A P Y April, 1956. o f civilian cases w ere also in v estig a te d d u rin g th e c h ro n ic e stab lish e d p h a se . T h ese in v estig a tio n s fa iled to show a n y significant d e v ia tio n fro m n o rm a l a n d m ay be ta k e n to e x clude d e stru ctiv e d e n e rv a tio n o f th e low er m o to r n e u ro n . Illu stra tiv e C ases. E x c e p t fo r case N o . 7 a n d 8 a ll th e p a tie n ts cited a re y o u n g nurses. Case 1. (G ). F ir s t seen 2 m o n th s a fte r o n se t, a t w hich tim e w as m a k in g g o o d recovery fro m o rig in a l p a resis o f left a r m a n d left leg. R e la p se o c c u rre d w ith a b r u p t onset, w eakness o f left a rm o nly, w ith c o ld sw eating o f skin o f h a n d , severe p a in in s h o u ld e r a n d in ab ility to m ove m o re th a n a flicker o f fingers. R e co v e ry o f left a rm h a s been slow a n d m a rk e d p a resis re m a in e d 4 w eeks-later. Case 2. (F ). T e n weeks a fte r o n se t slight p a resis o f left a rm , g ro ss p a resis o f left leg, n o a ctiv e m o v em e n t a g a in st g ra v ity , c o ldness o f left fo o t. T h re e m o n th s a fte r o n se t fitted w ith lo n g c alip er a n d g e ttin g a b o u t w ith a id o f stick. Case 3. (B). A t 10 w eeks a fte r o n se t, w h en slowly re ­ co v erin g fro m p aresis o f left a rm a n d b o th legs a n d i n tra c t­ a b le h e ad a ch e s, suffered relapse. R ig h t a r m affected fo r first tim e. M o d e ra te paresis b u t n o skin coldness. A t 12 weeks still a b ed p a tie n t b ecause o f c o n s titu tio n a l sym ptom s. Case 4. (W ). F ir s t seen 9 w eeks a fte r o n se t, m ild p aresis o f left a rm , b o th legs sp lin te d , c o m p la in in g o f c ra m p s in legs, o nly a flicker o f a ctive m o v em e n t in to es. M ild c o n ­ s titu tio n a l sy m p to m s. R e sp o n d e d well t o activ e assisted m o v em e n t, a n d w ith in 3 w eeks w as a m b u la n t a n d a b le to leave h o sp ita l. Case 5. (M ). Seen 6 w eeks a fte r o n se t, o rig in a l p aresis o f r ig h t a rm a n d rig h t leg, la te r o n se t o f p a resis in b o th legs. A t th is sta g e m a rk e d p a resis o f left leg w ith c o ld fo o t a n d f o o t - d r o p ; w as fitted w ith s h o rt iro n a n d d ischarged a t a b o u t 11th week. C ase 6. (H ). E leven weeks a fte r o n se t show ed c o n s ti­ tu tio n a l sy m p to m s, in ab ility to c o n c e n tra te , m ild p aresis in b o th legs, da y -b y -d a y flu c tu a tio n . W h e n a sk e d to raise rig h t leg a g a in st re sistan c e de v elo p ed gross tre m o r w hich s p re a d to left leg. M o to r sy m p to m s w ere su b o rd in a te d to e m o tio n a l sta te . Case 7. (F ). O n s e t w ith m ild m ala ise (d id n o t th e n o r a fte rw a rd s s to p w o rk ), p a in a n d paresis o f left leg, p a in a t first sim u la te d sciatica. Slow im p ro v e m e n t o f p a resis o v e r pe rio d o f 3 m o n th s . E ig h t w eeks a fte r o n se t developed p a in in left a rm . ' Case 8. (M rs. B .). Severe c o n s titu tio n a l a n d m o to r in volvem ent. N o w , 10 w eeks since o n se t, p aresis o f b o th legs, m o re m a rk e d in th e left. E x te rn a l r o ta tio n o f left h ip a n d c o rre c tio n is p a in fu l. Is n o w a m b u la n t. W ith th e su d d e n im p a c t o f larg e n u m b e rs o f cases o f a n unid en tifie d d isease, pe rp le x in g p ro b lem s o f diag n o sis a n d m a n a g e m e n t a ro se . E arly a n tic ip a tio n o f a c lear-cut se quence o f a c u te , c o n v alesce n t a n d c h ro n ic stages h a v e n o t b een fulfilled. In d e e d , th e on ly c o n s ta n t fe a tu re h as been th e e rra tic c o u rse. W ith re g a rd to m o rb id processes in th e c e n tra l n e rv o u s system , a c le are r c o n c e p t o f th e ir d is trib u tio n a n d d u r a tio n is fu n d a m e n ta l to d iag n o sis a n d r a tio n a l tre a tm e n t. In th e early stages c o m p le te m e n ta l a n d physical re st a re o f first im p o rta n c e a n d e x perience h a s fully e n d o rsed th is p rinciple. W ith th e recession o f sy m p to m s w e h a v e been fa ce d w ith th e p ro b le m o f h o w b e st t o achieve th e m ax im u m recovery in d isa b le d lim bs in th e s h o rte st p ossible tim e. E ssentially th is a m o u n ts t o th e b a la n c e d a p p lic a tio n o f rest a n d exercise, w ith d u e re g a rd t o th e ge n era l c o n d itio n o f th e p a tie n t. A p p ra is a l o f th e c o u rse o f p a re sis c a n n o t b e d iv o rc e d fro m th e c o n c o m ita n t e m o tio n a l c o n stitu tio n a l b a c k g ro u n d . I t is n o w fa irly well e stab lish e d t h a t th e a n sw e r to th e th e ra p y p ro b le m lies so m ew here betw een th e tw o extrem es o f o v e r-z e alo u s p re m a tu re activ ity a n d u n d u ly prolonged im m o b iliz atio n . T h e re a re g ro u n d s fo r diversity o f o p in io n o n th e degree a n d d u ra tio n o f sp lin tin g o f lim bs, ju s t a s is th e case in th e m a n a g e m e n t o f p o liom yelitis. A n ob jec tiv e analysis o f cases tre a te d w ith c arefully su pervised re -education o f affected lim bs d oes n o t in d ic a te t h a t th is pro c ed u re h a s b een o th e r th a n h elpful. In siste n ce o n th e dangers o f exercise h a s c re a te d in th e p a tie n t a re s is ta n t and a p p re h e n siv e a ttitu d e th a t h a s h a m p e re d supervised re­ e d u c a tio n o f m o v em e n t a t a la te r stage. T h e re a re ra tio n a l g ro u n d s fo r th e in tro d u c tio n o f c are fu lly -g ra d u a te d rem edial exercises a t a n a p p ro p ria te sta g e d u rin g recovery, w ith the o b je c t o f fa cilitatin g v o litio n a l m ovem ent. S U M M A R Y . N e u ro m u s c u la r d y sfu n c tio n a s it o c c u rre d in th e A d d in g ­ to n o u tb r e a k is described. M e a su res fo r physical re h a b ilita tio n a re c o n sid ere d a g a in st th e c om plex clinical p a tte rn into w hich th e y h a d to b e in te g ra te d . July 1955. N O T E .— A lth o u g h th e D e p a rtm e n t o f P h y s io th e ra p y a t th e J o h a n n e s b u rg G e n e ra l H o s p ita l h a s h a d th e o p p o rtu n ity o f o b se rv in g o nly o n e c ase o f th e D u r b a n M ystery Disease, a n d th e re fo re n o co n clu sio n s c a n b e d ra w n , it w as in teresting to re a d t h a t D r. A le x a n d e r o b ta in e d n o a lte re d electrical re ac tio n s. I n th is o n e c ase th e p a tie n t h a d a N e u ro to n test o n th ree oc ca sio n s, th e first te s t tw o m o n th s a fte r th e in itia l onset o f th e disease, a n d th e second o n e a m o n th la te r, th e th ird test b e ing a fte r a fu rth e r m o n th ’s tre a tm e n t. T h e follow ing re su lts w ere n o t e d :— A C C O M M O D A T IO N T E S T . (N o rm a l is 3— 6): D e c e m b e r 10th, 1955 = 2 -5 J a n u a ry 21st, 1956 = 1 -75 M a rc h 24th, 1956 = 1 • 75 T h ese resu lts a r e significant o f a P .R .D . S T R E N G T H D U R A T I O N C U R V E T E S T . T h e re w ere c h anges fro m th e n o rm a l o n e ach o c casion in t h a t th e c u rv e w as slightly h ig h e r th a n n o rm a l, and irre g u la r, w hich a g a in c o u ld be in te rp re te d as a P .R .D . C H R O N A X IE . T h e m uscles te s te d in d ic a te d a lo n g C h ro n a x ie . G A L V A N IC F A R A D I C T E S T N o rm a l i.e. th e m uscles show ed a b risk re sp o n se to b o th th e F a ra d ic a n d g alvanic c u rre n t, w ith K .C .C . g re a te r th a n A .C .C . A lth o u g h th e p a tie n t h a d v o lu n ta ry m o v em e n t in m uscles te ste d , th e y w ere in clined t o fa tig u e ra p id ly , a n d th e tests show ed a lte re d re sp o n se s to electrical stim uli. T h is I re p e a t c a n n o t be co n clu siv e as it w as a n iso la te d case, b u t I felt it w as w o rth fu rth e r in v estig a tio n sh o u ld th e o p p o rtu n ity arise. J ea n B la ir, Senior L e c tu rer in P hysiotherapy U niversity o f the W itwatersrand. FOR SALE O N E U L T R A -S O U N D T H E R A P Y M A C H IN E — “ U ltra - S o n a r” , a s g o o d a s new , co m p le te w ith ru b b e r-w h ee led ta b le a n d accessories, c o stin g n ew £395. F irs t c h eq u e fo r £295 will get th is m achine. Special a tte n tio n is d ra w n t o th e fa c t t h a t th is “ U ltra - S o n a r” m ac h in e h a s two tre a tm e n t h e ad s su itab le fo r su p e r­ ficial a n d deep th e ra p y . R e p ly :— P .O . 26, P h y s io th e ra p y D e p t., G e n e ra l H o s p ita l, Jo h a n n e sb u rg . R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. )