PART V III
THE DISEASES OF THE SKELETAL MUSCLES
GENERAL DISCUSSION
Th e ske le ta l mu scles are protected by th eir ana to m ica l r elati on sh ips from man y o l t he diseases affect ing the visce ra l mu scles.
Ve ry few o f th e pr im ary mu scul ar di sea ses a re found in o rd ina ry
practi ce, es pec ially in thi s co u nt ry. A nyt h ing w hich int erfer es
m ark edl y w it h th e inn er vat ion o r th e circ ula t ion of st ria te d mu scles may produce at ro phy o r weak ness.
T oxin s o r in fecti on s o f th e mu scles ca use th e mu scle fiber s t o
lose th eir st ri rc : th e mu scul ar nu cl ei m ay in cr ea se in number :
and th er e is usu a lly a multipli cati on o f th e inter stitial co nnec ti ve
ti sues. F at is fr equ ently depo itc d bet ween th e mu scle fiber s
und er suc h co nd it ions. Th e ab iotrop hic mu scul ar d isea ses, like
sim ila r di sea ses in th e ner vou s syste m , ap pear to b e du e to a
congenital or heredit ar y def ect o f the g erm pla sm.
Mu scl es k ept co n t rac te d by a co ns ta nt ly ac t ing st im ulus acquire
a pecul iar sta te wh ich seems int erm edi at e be twee n normal mu scula r co nt rac tio n and co nt rac t u re . Doub tl ess th e co nd itio n fi nall v
ter mi nat es in fibro sis. Thi s co ns t an t co nt rac t ion is noti ced most
fr equently in th e mu scles o f th e deep er lay er s o f th e ha ck , wh en
th ese a re . t imul at cd hy nerve im pul ses r eflexl y prod uce d by bon y
lesion s or by visce ra l di sea se. Th e mu scl e becom es st iff o n palp ati on , o fte n with hard er kn ot s o r co rds whi ch a re u su all y ve ry
hyp er sen sitive ; so me t imes the entire muscl e becom es hyp csth ct ic
or anesth eti c a fte r lon g co nt ract ion. R igidi ty o f th e s pina l a reas
a ffec te d is prom ot ed by th ese co nt rac t ions, es pec ially w he n several
s p inal seg me nts a re in vol ved,
.
Th e affe cte d m uscles a re w eaker t ha n norm al; t h ey t end to
recur to th eir co n t rac te d sta te af te r relaxati on, and it appears ve ry
prob able th at th ey are r cs po ns ible fo r th e perpet ua t ion o f bon y
lesion s, and for t hcir recurren ce aft er co rrec t ion. It is evid ent
that norm al se nso ry st im ula t ion docs not ari se from m u scles kept
undul y ten se; thu s. th ey are at lea . t partly r esp onsib le for th e
dimini sh ed ac t iv ity o f th e ner ve ce nte rs o f th e corresp onding
s pinal se~ m~ : l ts. S uc h mu scul ar t en sion is p ro pe rly call ed a "mu scular lesion.
Bon y lesion s are Freq ue ntly fou nd r es pon sibl e for weak ness
of individual mu : cles o r for mu scl e g ro ups , hut not, so far a our
present kn owled g e g oes, for tru e mu scular di case.
447
CH APTER XLI
DI SE ASE S OF lIIUSCLES
ACUTE POLYMYOSITIS
(I n fectious myositis)
Th is is a rare d isease, probably d ue to the prese nce o f so me
unkn o wn in fect iou s orga nis m. I t is a tru e inflamm at or y pr ocess ,
cha racterized by hy per em ia , swelling. pain, and ede ma o f th e
m uscl es. Leu cocy ti c in filtrat ion is p re sen t in th e m u clc .
Th e mu scl es o f th c a r ms a nd leg s firs t becom e hard , sw o lle n,
pain ful a nd st iff; lat er , t he mu scles of t he face and trunk bec ome
in vol ved ; swa llo w ing a nd respi rati on become d ifficult ; a nd deat h
usu all y occ urs in a few weeks in th e ac u te form . Rar ely th e ac u te
form o f the di sea se m ay becom e ch ro n ic a nd deat h he d elay ed for
t wo or three yea rs . A lo w fevcr is usu ally p resent during th e
ea rlier stages.
T he treatment is un sati sfact or y , o n accoun t of our ign oran ce
co nce r ning th e tru e ca use o f th e cond it ion . Suc h trea tm ent as
see ms to he ind ica te d by t hc co nditi on o f thc pa tient a t t he tim e
of th e exa m ina tio n may be give n a s a palli ati ve measure. Th e
tr eatm ent w h ich g cnc ra lly in cr eases thc re si sta nce o f the bod y to
inf ect ion is in dicated o n gc nc ra l pr inciples. Rest in be d, a non purin di et , wit h ve ry free wa te r d rinkin g , a re pe rhaps th e most
useful fa ct or s in th e treatm ent.
MY OSITIS OSSIFICANS
T wo form s o f th is di sea se a rc k now n. T he local fo rm is due to
irrit at ion of sing le mu scles, o r mu scle group s. It is present in
horsem en as th e result o f th e p ressure o f t hc sa dd le upon th c
leg s ; it occasiona lly affe ct s th e mu scles o f th e s ho ulde rs in m en
who ca r ry hea vy burd en s u pon th e sho ulde rs . I n thi s for m t he
mu scl es a ffec te d und er g o first th e cha nges cha rac te ris t ic o f ac ute
myo siti s, wi th m arked ove rg rowt h o f th e int er titi al connect ive
ti ssu e. Th e sca rlike ti ssu e thu s formcd und er g oes slow ly progressive calc ifica tion.
General, or syst em ic, myositi ossificans is a r ar e di sease, cha rac te rized by the sp onta neo us occu r rence o f Lon clik e g ro wt hs ,
in volving mostl y th e mu scu lo-t endinou s a re as . The ca use is
un k nown . No h istory o f her edity o r sy ph ilis is p re se nt. It a ppears usu ally befor e th e t enth yea r of life.
448
ACUTE l'OLV.lfl·OSITIS
4~9
The m uscl es aff ect ed , u su all y fir st o f thc s ho u lde r o r pel vic
g ird le, bec om e slig h t ly swolle n, s t iff a nd p ainful; a s ma ll ha rd
lum p a pp ea rs, which ma y reach th e size of a n o ra nge; t hc calc ificati on ex te n ds alo ng th e t en don an d th c m uscle, following th e co nnect ive t issue trab ecu l:c. 'I'h c m us cle does no t b ecome p araly zed,
hut m o vem ent o f th e ncighboring joint b ecom es impossibl e o n
acco unt o f th e 0 sifica t io n o f th e t end on s, In thi s ty pe the tum ors
resemble n cwl v fo rme d bon e.
It i rare {or lifc to he p rol on g ed b eyond th c t wenti eth Year.
Dea t h occ urs eit he r fro m so me iu t erc urrc nt d i ease , o r as the
result o f s u ffoca t io n du e to the in vol vem ent o f the r espiratory
mu scl es.
SECONDARY MUSCULAR DISEASES
Rheumatic Myositis. Rh eum ati c myositi s is an inAam matio n
of th e mu scles d ue to th e bact eria wh ich ca us e ot he r rh eu matic
di sease s. (q . v.)
Suppurative Myositis.
Suppurat ive m yosi ti s, or mu scul a r
ab scess, ma v be du e ei t he r to th c inf cction o f a wo u nd o r to
se ptice m ia . • Th e d iagn o is o f thi s co ndi t io n rest s u pon th e pain ,
leucocy tosis, a nd ot he r sy m pto ms o f pu s fc rma t io n. Th e treatm ent is su rgica l.
Gouty myositis i a pa in ful chron ic inAamm at ion o f th e mu cles
assoc ia te d wi t h sy tcmi c go ut. (q. v. )
Trichiniasis. Tri chini a sis (CJ. v.) o fte n in vol ves th e mu scles.
Th e d iagnos i rest s up on th c hi ' to ry u f pork ea t iug, th c c ha rac te r
o f th e pain , m ark ed eosino phi lia . a nd so me t imes a mi croscopi cal
exa m ina t io n o f a n exc ise d bit o f mu scle. T hc tr ea tm cnt is sy mt orn at ic, a nd t he progn o sis m ay be ver y se r io us,
Paralysis of the Striated Muscles without Atrophy is d ue to
so me lesio n o f t hc u ppcr ncu ron syst em.
Paralysis, or Weakness, of the Striated Muscles w it h Atrophy
may be du e to in jury to th e mu scle it self as in th e prim ary mu scu lar di seases, to di eases a ffcctin g th e m otor end-pla tes, t o di sea e
o f t hc ner ve trunk , o r to di sea se of th e a n te r ior horn s o f the sp inal
co rd. Th ese co ndi tio ns a re di scu se d in co nnec t io n with th e di sea ses o f th e nervou s syste m.
Arthritic Muscular Atrophy. Aft er th e occ ur rence o f a r th r it is
a ny w here in t he bod y, th e m uscl es whi ch mo ve th e afTcctcd jo int s
und ergo a va r ia ble a mo unt o f a t ro phy . T hi s typ e o f atrop hy is not
associa ted w it h th e pr esen ce o f a ny hyp ert rophi c fiber s a nd w it h
o nly slig h t increa se in th e int erstiti al t issu e.
It see ms to b e du e to so me reAex trop h ic eff ect, r es ulti ng fro m
th e ir ri ta t ion o f th c sc rtso ry nerves di stri but ed to th e a r t icu la r
450
THE SKELE TA L M USCLES
su rfaces, w ith the subs idence of t he infl ammation in t he joint.
The mu scl es m ay regain their normal size and st reng t h. Occasio na lly th e a t ro p hy per si st s a nd seco nda ry contractions occ u r
wh ich m ay resembl e th ose found in anteri or poliomye liti s.
THE MUSCULAR DYSTROPHIES
Thi s group of di sea ses aff ecting th e skeleta l mu scles includes
seve ra l subd iv isions, a ll o f w h ich are cha rac terized by the im porta nce o f heredity in their et iology; by th e a ppe a rance o f the di sease befor e puberty; a s a r ule, by the fact th at a certai n a moun t
o f hyp ertroph y is a ssociated wit h the atrop hy; a nd by the lack of
recogni zabl e nerve les ion s.
No tr eatm ent app ears to be o f any value in preventing the
ult imat e fat al outco me, th ou gh in so me very m ild ca ses the afterhist or y of th e pati ent m ay not he se rious ly modified by the occurr en ce o f this di sea se in early life.
M yoton ia Con genita (Thom sen's di sea se) . T his is a famili al
di sea se of the mu scl es, characteri zed by the followi ng sy mptoms:
Wh en th e pati ent att empts any m ovement, afte r a period o f rest,
th e mu scl es affec te d contract st ro ngly and do not relax fo r a conside rab le int er val : the next contra cti on i foll ow ed by a somewhat
dimini sh ed contracture peri od; the third. by a till less prolonged
tonic contracti on; until fina lly th e pati ent becomes able to perform the moti on whi ch he had first decid ed up on . T his series of
events is rep eated wheneve r th e patient endeavors to begin a ny
com pl ex a cti on , a s in walking. Sometimes the attack is so severe
a s to th ro w him to the g ro u nd , more fr equent ly there is merely
d ifficul ty in get t ing sta r te d .
Th e v isce ra l mu scl es are nev er involv ed . Mental di sturbances
m ay he p resent . R eflex es are s lig h tly modifi ed or unchanged.
]\[uscul ar weakness is noticeab le, th ough th e mu scles m ay be
norm al or co ns idc rablv inc rea sed in size. E lec trica l react ions a re
ch an g ed ( myoto n ic re acti on).
A simila r at y pi cal di sea I.' ma y occ u r with out her editary ba sis;
it s ca use is unkn own.
Th e atta ck s are mad e wor se by expo ure to cold and by emoti on al ex cit em ent. Treatment is practi cally useless. I t does not
greatly sho r te n life, but rec overy is not to be expected.
P seudohypertrophic Muscular Atrophy, or pseudo m usc ula r
hyp ertrophy ( D uc he n ne) is a form of the di sease which is chara ct eri zed by it s first a ffec t ing the muscles of the ca lve s of t he leg s.
Thi s co mes on rath er slow ly a s a hyp ertrophy a nd may at first
be con sid er ed ev ide nce o f the child's excell ent health. There is
usu all y a lordosis , whi ch ex ag ge rat es the deformity. The mu scles are ver y w eak , ev en when the size is much greate r than no r-
M USCULAR D f STR OPHIES
451
m al. Other m uscles of the bod y, including the t ru nk and respi rato ry mu scles, become affected and death occurs fr om cachexia.
O cca ion ally this form o f mu scul ar atrophy is ass ociated with
epileptic attacks and with mental defect.
Leyden-Moebius type. In thi s form of mu scu lar atrophy th e
hy per t rophy is not apparent , and th e her editary influ en ce is even
more mark ed. Otherwi se , th e disease is like that ju st de scribed.
The Scapulohumeral type h as been d escribed by Erb. I t comes
o n later in life, even up t o th e ag e uf tw enty, and aff ect s first the
mu scles of the sho ulder g irdle.
A peculiar wing-like positi on o f th e sc apulre re ult s fr om the
atrophy of the se mu scles. Th e di sea. e ex te nds t o the leg and
trunk mu cle s, and death occu rs as already mentioned.
Facioscapulohumeral t ype (Dcj cr inc-L audou zy ) . Thi s d isea se is especially cha racteri zed by it s ons et in ab out the t hi rd or
four th y ea r, affe cting' first t he m uscle s of t he fa ce. T he di sea se
affects t hen th e shoulder , leg and trunk muscles. Th is form of
t he di sea se is very slo w in it s pr ogress and patients may live to
be t hirty or forty years old.
Atrophic Myotonia. Th is is a rare d isea se characterized by
abn orm ally slo w rela xation o f ce r ta in mu scle g ro ups after contraction, and by the oc cur rence o f atrophy in th e mu scl es affected.
Oppenheim's M yotonia. Tn thi s di sea se th e mu scl es undergo
flaccid paraly sis with loss u f th e reflex es. It does not sho r te n life
and is in curabl e. The mu scl es atrophy and th e pati ent becom es
helple ss. l\Ia ssag e o f the a ffec te d mu scles a nd elec t r ica l s t im ulation see m to dcla v so me what the co u rs e o f th e disea se. So me
relati on bet ween th e th ymu s g la nd and thi s di sea se has heen
sugge ted .
Myasthenia Gravis. Thi is a r ar e di sease o f unknown et iolog y . Th e mu scl es o f ma sti cati on. speec h an d deglutiti on are involved, and al so th e ex t rin sic eve mu scl es. Th e di sea se is characteri zed by very rapid fati gu e: whi ch is inh er ent in the m uscle
it self, rath er than in th e nervou s co nt ro l. Dy spn ea , dy sphagia,
and pt o sis o f the eyelid are noti ceabl e sy m pto ms . D eath occurs
from exhau sti on, or the pati ent may be s t ra ng led while t rying to
swallow .
Dysbasia Lordoca Progressiva (Tortipclvis) . T h is is a disea se
pecu liar to J ews, whi ch app ears in childre n a nd yo ung ad ult s.
M uscu lar spasms of the lum ba r and pelvic regi on ca use a def or m ity
of th is pa rt of t he body. Th ere is marked lor dosis of th e dor so lu m ba r spinal co lu m n. There arc no s ig ns of organ ic d isea se of
th e ner vou s or osseo us sy ste ms . 'l' hc ter ms " mo nkey ga it" o r
" d ro meda ry gai t" have bee n app lied to t h is co nd iti on.
452
TIlE SKELETAL JJUSCLES
FUNCTIONAL MOTOR DISTURBANCES
T he di ffer ent va rieties o f cho rea, spas m , ti c and tremo r a rc
gcnerally considered funct ional o r idi opa thic. It is needless to
say t hat t he use of t hese t erm s is merely a confession o f ou r
ignorance of th e struct ural or bioc hemical cha nges which m ust
neces ari ly he p resen t in every disease. T he tcrm spasm is
app lied to th o se mu scul ar co n trac tions w hic h r esult fr om so me
ir rit ati on in th e lo wer Tc flex arc. Tru e spas m is in volunt ary and
is not to be co n tr oll cd by an y process o f ed uca t ion. Th e cho re iform movem ent s resembl e, t o so g rca t a n ex te nt, th e movem ent s
re ult ing fro m pa tho logical changcs in t he ba sal ganglia , especially
in th e lenticul ar nu cleu s, th at it m ay be gra nted th at th ese
movem ents are du e to th e irritation somewhere in t he h ig hcr
reflex a rc , inclu d ing perhaps t he pontine centers a s we ll a s t he
ba a l ga ng lia. Tic, o r, as it is sometimes ca llcd, habit spas m,
rc ults from t he repet iti on of complicated movem ent s. These are
in voluntary in th c hcg in ning a nd a re o fte n initiat ed tind er so me
emo tiona l s t ra in. R eeducati on is o ftc n e fficient in d ealing with
th ese cases, Th c sca t o f th e d ist ur be d fu ncti on in th e ti c is
pr ob ab ly in th e d eeper lay ers o f th e cer ebral cortex wh ere o the r
habi tu al u ncon sciou s actions a re controlled.
A ll o f th e functio na l moto r ne uroses hav ing a ccrta in d eg ree
of heredit ar y tai nt res t u pon t he presence o f a neuropathi c cons tit uti on . I n a ll of thcm, the treatment m ust incl ude th e m ea su res
necessar y to sec ure good n utr iti on , good eli mina t ion, rest a nd
w ho leso me, sane, hygi eni c lif e for t he pa tie nt.
Primary Athetosis . Thi s is a rare fu ncti on al di sea se, cha rac teri ze-d by t he occ u r re nce o f s lo w a t he to id movements of t he
hands. It occ urs in la te m iddl e life o r o ld age a nd is not a s sociatcd w ith men tal dete riorat ion. O nly aftc r o rgan ic lesion s o f
t he b ra in and especially o f the corpora striata and optic t halamu s
have bee n eli minated ca n a di ag no sis o f prima ry athetosis be m ade.
o t rea t ment affects t he co u rse of th e disease. It per sists throu ghou t life, which it does not see m to shorten,
Senile Tremor. T h is occ urs in old peopl e freq ue ntly at th e
age o f 70 o r th er ea bout s. Th e fingc rs and thumb a rc usuall y held
stra igh t, a t right a ng les t o th e hand s , and th e t rem or in volves
both fing cr s and hand and occas iona lly th e neck mu selos a re
affec ted , so th at th e pat ient co nstantly nods hi s head . Th e co ndit ion is no t associa ted wit h a ny m en t al pec uliari tv a nd th e o nl v
pat ho log ica l con ditio ns are th ose charactc rist ic of old age. j T~
ha rm resul t s fro m t he condi tio n and no t reat ment is of th e least
avai l in co ntro lling it .
Toxi c Tremor. Alcoho l or tobacco. lead , m er cury and ce rtai n
ot he r met all ic poi son s or th e o rga nic poisons o r a uiointox icat ion
m ay all ac t up on th e ner ve cc nte rs a to pr odu ce a rath er irreg ular
PERIODIC P.·IR.ILI"SIS
tremor.
poi son.
453
Th e co nd it ion di sapp ears w ith th e elim ina t ion o f the
PERIODIC PARALYSIS
(Fam ily peri od ic par alysis )
T hi s is a famil y di sease o f rare occ ur re nce in thi s co u nt ry ,
cha rac t er ize d by the occ ur re nce o f a lmos t o r quite t ot al pa raly si s,
fr om which th e p atient rather spee d ily appa re nt ly r eco ver s.
Etiol ogy . Th e d isease is a lways h er ed ita ry. It ap pears t o
foll o w M endel's la w , th ou gh th c n umbe r of cases o n recor d is not
sufficie nt to prove t he la w by exact n umbers.
'I'he individ ua l attack may occu r wi thout r ecog niza bl c ca use,
b ut t hcy arc freque nt ly p recipitated by m u scu la r exert ion o r by
overeat ing.
P a th o logy. A lmos t no structu ra l changes arc constant ly presen t. A n
exa mination of th e mu scles some times shows sligh t vac uo liza tio n with ocr asiona lly a hypertroph ied cell. J n so me cases no cha nges in eit her th e mn scles
o r th e centra l nervous sys te m a rc to IJe foun d upon th e most ca ref ul exa mina tion.
Th e dimin ish ed exc re tion of th e calc iu m and magn esium sa lts in th e urine
seems to IJ e fa irly co ns ta nt. It has been suggeste d th at th e par alysis is du e to
th e excess of these sa lts thu s r eta ined wi thin t he blood whi ch inhi bits th e action
o f th e n er ve and mu scle cells.
D ia gnosis. Th e sy mp to ms a nd hi tory a rc fairly typ ica l. Th e
fam ily h ist ory s hows th e her ed it ar y t a int , w hile th c h istor y of
prev ious a ttack wi t h recovery s ho u ld m ake th e diag no sis certai n.
T he re a rc prodrome usu a lly of a vag ue discomfor t, Thc patien t
goe t o lcep an d awakens completely paralyzed. Occasionally
th e pa ra lysis is not quite co mplete, but it usua lly in volves all fo u r
limb s. Speech, th e sp h incte rs, t he respiratory mu scles and dcglu t it ion a rc not affec te d.
T hc reflex es a rc cl earl y d iminis he d o r
m ay be a b ent. Th e mu scl es do not usu all y co nt rac t in a nswe r t o
th e elcc t r ic cu r rent . Th e heart is Ir cqu cut ly found d ilat ed o n
exa m ina t ion durin g an a tt ac k. Thi s di sappears w ith recovery .
E xamin ati on o f th c urin e sho ws so me a lb umin , so me b lood a n d
oc casio na lly so me hcm ogl ohin.
Th e a ttac k last s a fcw hours to a few day s. 'I'h cn a ve ry p rofound per spirat ion occ u rs, foll o wed by weak ness, so me times slee p
and a grad ua l reco ver y. Du rin g co nva lesce nce th c mu scles a rc
ve ry w eak a nd st rc ngt h is r eg a in ed on ly af te r so me days o r w eek s
o f g ra d ua l im provem ent .
T r ea t me nt . Th e treatm ent is d evot ed to prevcn tin g a t tacks.
Overe a t ing, co ns tipa t io n, un du e mu scul ar exe r t ion, are to be
avo ide d. Th e co r rec t ion o f s uc h bon y lc ion s as may be found
on exa m ina tio n sho u ld be o f v alu e in sec u r ing good nutrition a n d
good elimination.
Prognosis. The prognosi s is bar! for complete r ecovery ; is
vcry g ood for re covery fr om a ny attack, and is bad for the descendent s of the ind iv idual. Marriage sho u ld be prohibited.
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