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    PART VIIITHE DISEASES OF THE SKELETAL MUSCLES

    GENERAL DISCUSSIONTh e ske le ta l mu scles are protected by th eir ana tom ica l rela-ti on sh ips from many ol the diseases affect ing the viscera l mu scles.

    Ve ry few of th e pr imary mu scul ar di sea ses a re found in o rdinarypracti ce, es pec ially in thi s country. Anything which int erferesmarkedl y w ith th e inn ervat ion or th e circulation of st r ia ted mu s-cles may produ ce atrophy or weakness.T oxin s or in fection s of th e mu scl es cause th e mu scle fiber s tolose th eir st ri rc : th e mu scul ar nu clei may increase in number :and th er e is usu ally a multiplicati on o f th e in ter stitia l co nnec ti veti sues . F at is fr equ ently depo itc d bet ween th e mu scle fibersund er such conditions. Th e ab iotrophic mu scul ar diseases, likesim ila r diseases in th e nervous sys tem, ap pear to be du e to acongenita l or hereditary def ect of the germ pla sm.Mu scles kept contrac te d by a cons ta ntly ac t ing st imulus acquirea pecul iar sta te which seems int ermedi at e be tween normal mu s-cular cont rac tio n and cont rac ture . Doub tless th e co nd itio n finall vterminates in fibrosis. Thi s co nstant con t rac t ion is noti ced mostfrequently in th e mu scles of th e deeper layer s o f th e ha ck , wh enth ese a re . t imul at cd hy nerve im pul ses reflexly prod uce d by bonylesion s or by visce ra l di sease. Th e mu scle becomes stiff on palp a-ti on , often with hard er kn ot s or co rds whi ch are usu all y ve ryhyp er sen sitive ; sometimes the entire muscle becom es hyp csth ct icor ane sth eti c afte r lon g co nt ract ion. R igidi t y o f th e spinal a reas

    affec ted is promot ed by th ese co nt rac t ions, es pec ially whe n seve ralspinal segments a re in volved, .Th e affe cted muscles a re weaker tha n normal; they tend torecur to th eir contracted sta te af te r relaxati on, and it appea rs ve ryprobable th at th ey are rcsponsible for th e perpe tuation of bonylesions, and for t hci r recurrence aft er co rrec tion. It is evid entthat normal senso ry stimula t ion docs not ari se from muscles keptunduly ten se; thu s. th ey are at lea . t partly responsib le for th edimini sh ed ac t ivity o f th e ner ve cente rs o f th e corresp ondingspinal s e ~ m ~ Such mu scul ar tension is properly call ed a "mu s-cular lesion.Bony lesion s are Freq uently fou nd respon sibl e for weaknessof individual mu : cles o r for mu scl e g roups, hut not, so far a ourpresent kn owledge goes, for tru e mu scular di case.447

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    CHAPTER XLIDI SEASES OF lIIUSCLESACUTE POLYMYOSITIS

    (I nfectious myositis)Th is is a rare disease, probab ly due to the presence of someunknown in fect iou s organis m. I t is a true inflammat ory pr ocess ,

    cha racter ized by hyperemia, swelling. pain, and ede ma o f th emuscles. Leu cocytic in filtration is presen t in th e mu clc .Th e mu scl es of th c arms and leg s firs t become hard , swollen,pain ful and st iff; later, t he mu scles of the face and trunk bec omeinvol ved ; swallowing and respirati on become difficult ; a nd deat husually occ urs in a few weeks in th e acute form . Rar ely th e ac uteform of the disease may become ch ro nic and deat h he delayed fortwo or three yea rs . A low fevcr is usu ally present dur ing th eea rlier stages.T he treatment is un sa ti sfact or y, on accoun t of our ign oran ceconcerning th e tru e cause of th e cond ition . Such treatm ent assee ms to he ind icated by thc condition of thc pa tient a t t he timeof th e exa m ina tio n may be given as a palli ati ve mea sure. Th e

    tr eatm ent which gcnc ra lly increases thc re sista nce of the body toinfect ion is indica ted on gc ncra l pr inciples. Rest in bed, a nonpurin diet , wit h very free wa te r d rinking , a re pe rhaps th e mostuseful fa ct or s in th e treatm ent.

    MYOSITIS OSSIFICANST wo form s of th is disease arc k nown. T he local form is due toirritat ion of sing le mu scles, or mu scle groups. I t is present inhorsemen as th e result of th e pressure of thc sa ddle upon th cleg s ; it occasiona lly affe ct s th e mu scl es of th e shoulders in menwho ca rry heavy burdens upon th e sho ulders . I n this for m themu scl es affected undergo first th e cha nges cha rac te ris tic of ac utemyositi s, wi th marked ove rg rowth of the int er titi al connectiveti ssue. Th e sca rl ike ti ssue thus formcd und ergoes slow ly progressive calc ification.General , or syst emic, myositi oss ificans is a rar e disease , cha rac terized by the sp onta neo us occurrence of Lon clike g rowths,involving mostl y th e mu scu lo-t endinou s areas. The cause isun known . No history of her edity or syphilis is pre sent. It appea rs usu ally befor e th e tenth yea r of life.

    448

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    ACUTE l'OLV.lflOSITISThe m uscl es aff ected, usually fir st o f thc s houlder or pelvicg ird le, becom e slightly swo lle n, s tiff a nd painful; a sma ll ha rd

    lum p app ea rs, which may rea ch th e size of a n o range; t hc calc ification ex te nds along th e ten don an d th c m uscle, follow ing th e connect ive t issue trab ecu l:c. 'I'h c m us cle does no t become paralyzed,hut movem ent of th e ncighboring joint becomes impossibl e onaccount o f th e 0 sifica t ion of th e tend on s, In th i s ty pe the tumorsresemb le ncwl v forme d bone.It i rare {or lifc to he prolonged beyond th c t wentieth Year.Dea t h occ urs either fro m so me iu terc urrc nt d i ease , or as theresult of suffoca t ion du e to the in volvem ent of the respiratorymu scles.

    SECONDARY MUSCULAR DISEASESRheumatic Myositis . Rh eumati c myositi s is an inAammationof th e mu scles due to th e bact eria wh ich ca us e othe r rh eumaticdi sease s. (q . v. )Suppurative Myositis . Suppurat ive myosi ti s , or mu scularab scess, mav be du e ei the r to th c inf cct ion of a wo und o r tose pticemia. Th e d iagn o is o f thi s co ndi t io n rests upon th e pain ,leucocy tosis, a nd other sy mptoms o f pu s fc rma tion. Th e treat-ment is su rg ica l.Gouty myositis i a pa inful chronic inAamm at ion of th e mu cles

    assoc iated wi t h sy tcmi c go ut. (q. v. )Trichiniasi s . Trichiniasis (CJ. v.) o fte n involves th e mu scles.Th e d iagnos i rest s up on th c hi ' tory u f pork ea t iug, th c cha rac te ro f th e pain , marked eos inophilia. and so metimes a microscopi cal

    examina tion o f a n exc ised bit of mu scle. T hc treatm cnt is sy mtorn at ic , and the prognosis may be ver y se rious,

    Paralysis of the Striated Muscles without Atrophy is due tosome lesion of thc uppcr ncu ron sys tem.Paralysi s, or Weakness, of the Str ia ted Muscles w ith Atrophymay be du e to in jury to th e mu scle it self as in th e primary mu scular di seases, to di eases a ffccting th e motor end-p la tes, to di sea eof thc nerve trunk , o r to di sea se of th e an te r ior horn s o f the spinalco rd. Th ese co ndi tions a re di scu sed in co nnection with th e di sea ses of th e nervou s syste m.

    Arthritic Muscular Atrophy . After th e occ ur rence of a r thr i t isany where in t he body, th e m uscles which move th e afTcctcd jo int sund ergo a va riable amount of a trophy . T hi s typ e o f atrop hy is notassocia ted wit h th e pr esen ce o f any hyp ert rophi c fibers and w it honly slig h t increa se in th e interstitial t issu e.It see ms to be du e to so me reAex trop h ic eff ec t, resulti ng fro mthe irrita t ion o f th c sc rtso ry nerves di stribut ed to th e a r t icu lar

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    450 THE SKELE TA L M USCLESsurfaces, w ith the subs idence of the inflammation in the joint.The mu scles may regain their normal size and strength. Occa-sio na lly th e at rophy per si st s and seco nda ry contractions occ urwh ich may resemble th ose found in anterior poliomye liti s .

    THE MUSCULAR DYSTROPHIESThi s group of disea ses aff ecting th e skeleta l mu scles includesseve ra l subd ivisions, a ll of which are character ized by the importance of heredity in their et io logy; by th e appearance of the disease befor e pub erty; as a rule, by the fact th at a certain amoun tof hyp ertrophy is associated with the atrop hy; and by the lack ofrecognizabl e nerve les ions.No tr eatment appears to be of any value in preventing theult imate fat al outcome, th ough in some very mild ca ses the af ter -hist ory of th e pati ent may not he se rious ly modified by the occur-r en ce of th is disease in early life.Myoton ia Con genita (Thom sen's di sea se) . T his is a famili aldi sease of the mu scles, characteri zed by the following symptoms:When th e pati ent attempts any movement, afte r a period of rest ,th e mu scl es affecte d contract st rongly and do not relax for a con-side rab le inter val : the next contraction i follow ed by a somewhat

    diminished contracture period; the third. by a till less prolongedtonic contracti on; until fina lly th e pati ent becomes able to per-form the moti on whi ch he had first decided up on . T his series ofevents is repeated wheneve r th e patient endeavors to begin anycom plex action , as in walking. Sometimes the attack is so severea s to th row him to the g ro und , more frequent ly there is merelydifficul ty in get t ing sta rted .Th e visce ra l mu scles are never involv ed . Mental disturbancesmay he p resent . Reflexes are slig htly modifi ed or unchanged.]\[uscul ar weakness is noticeab le, th ough th e mu scles m ay benorm al or considc rablv inc rea sed in size. E lec trica l react ions a rechan ged (myo ton ic reacti on).A similar at ypical di sea I.' ma y occ ur without her editary ba sis;it s cause is unknown.Th e attack s are made worse by expo ure to cold and by emo-ti onal excit em ent. Treatment is practi cally useless. I t does notgreatly shorten life, but recovery is not to be expected.

    P seudohypertrophic Muscular Atrophy, or pseudomusc ularhyp ertrophy (D uc he nne) is a form of the disease which is char-acteri zed by it s first affecting the muscles of the ca lve s of the leg s.Thi s comes on rath er slowly as a hyp ertrophy and may at firstbe considered ev idence of the child's excell ent health . There isusually a lordosis, whi ch exaggerates the deformity. The mu scles are very weak, even when the size is much greate r than nor-

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    M USCULAR D f STROPHIES 451mal. Other muscles of the bod y, including the t runk and respi ratory mu scles, become affected and death occurs from cachexia.Occa ion ally th is form of mu scul ar atrophy is associated withepileptic attacks and with mental defect.

    Leyden-Moebius type. In thi s form of mu scu lar atrophy th ehypertrophy is not apparent , and th e hereditary influ ence is evenmore marked. Otherwise , th e disease is like that ju st de scr ibed.The Scapulohumeral type has been described by Erb . I t comeson later in life, even up to th e age uf tw enty, and aff ects first themu scles of the shoulder g irdle.A peculiar wing-like positi on o f th e scapulre re ult s from theatrophy of the se mu scles. Th e di sea . e ex te nds to the leg andtrunk mu cle s, and death occurs as already mentioned.Facioscapulohumeral type (Dcjcr inc-Laudouzy ) . Thi s d is-ea se is especially cha racterized by it s onset in ab out the t hi rd orfour th year , affe cting' first t he muscle s of the face. T he di sea seaffects then th e shoulder, leg and trunk muscles. Th is form ofthe di sease is very slow in it s progress and pat ients may live tobe thirty or forty years old.Atrophic Myotonia. Th is is a rare disease characterized by

    abn ormally slow relaxation of ce r ta in mu scle g roups after contraction, and by the occur rence of atrophy in th e mu scles affected.Oppenheim 's Myoton ia. Tn thi s di sease th e mu scles undergoflaccid paralysis with loss u f th e reflexes. It does not shor ten lifeand is incurable. The mu scles atrophy and th e pati ent becom eshelple ss. l\Ia ssag e o f the affected mu scles and electrical s timulation see m to dcla v so mewhat the course of th e disea se. So merelation bet ween th e th ymu s g land and thi s di sease has heensugge ted .Myasthen ia Gravis. Thi is a rar e disease of unknown et iology . Th e mu scl es of masti cati on. speec h an d deglutition are involved, and also th e ex trin sic eve mu scles. Th e di sease is cha racterized by very rapid fati gu e: whi ch is inh er ent in the muscleit self, rath er than in th e nervou s cont ro l. Dyspn ea , dy sphagia,and pt osis of the eyelid are noti ceabl e sy mpto ms . Death occur sfrom exhau sti on, or the patient may be s trangled while t rying toswa llow .Dysbasia Lordoca Progressiva (Tortipc lvis) . T his is a diseasepecu liar to J ews, whi ch appears in children and yo ung ad ult s.Muscu lar spasms of the lumbar and pelvic region ca use a def or mityof th is pa rt of the body. Th ere is marked lordosis of th e dorsolu mbar spinal co lumn. There arc no sig ns of organic d isease ofth e ner vou s or osseo us sy stems . 'l'hc terms " monkey ga it" or"d romeda ry gai t" have bee n app lied to t his co ndition.

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    452 TIlE SKELETAL JJUSCLESFUNCTIONAL MOTOR DISTURBANCES

    T he di ffer ent va rieties o f cho rea, spasm, ti c and tremor arcgcnera lly considered funct ional or idiopathic. I t is needless tosay t hat t he use of t hese term s is merely a confes sion of ou rignorance of th e struct ural or bioc hemical cha nges which m ustneces ari ly he p resen t in every disease. T he tcrm spasm isapp lied to th ose mu scul ar cont rac tions whic h result fr om so meirrit ati on in th e lower Tc flex arc . Tru e spasm is in voluntary andis not to be contr ollcd by an y process of educa tion. Th e choreiform movem ent s resemble, t o so g rca t an ex te n t, th e movem ent sre ult ing from pa thological changcs in the basal gang lia, especia llyin th e lenticul ar nu cleus, th at it may be granted th at th esemovements are du e to the irr itation somewhere in t he h ighcrreflex arc , including perhaps t he pont ine centers a s we ll as theba al ganglia. Tic, or, as it is sometimes ca llcd, habit spasm,rc u lt s from the repet ition of complicated movem ent s. These areinvoluntary in th c hcg inning and are o ften initiat ed tinder so meemo tiona l s train. Reeducati on is o ftcn efficient in dea ling withth ese cases , Th c sca t of th e d is tur bed fu nction in th e ti c ispr obab ly in th e deeper lay ers of th e cer ebral cortex wh ere othe rhabitu al uncon sciou s actions are contro lled.All of th e functiona l motor ne uroses having a ccrta in degreeof heredit ar y tain t res t upon t he presence of a neuropathic cons tit ution. I n all of thcm, the treatment m ust include th e m easu resnecessary to sec ure good nutr ition , good elimina t ion, rest andwholeso me, sane, hygienic lif e for t he pa tie nt.

    Primary Athetosis . Thi s is a rare fu nctional di sea se, cha rac teri ze-d by the occ urrence of s low at he to id movements of t hehands. It occ urs in la te m iddle life or old age and is not associatcd with mental dete riorat ion. O nly aftc r o rgan ic lesion s ofthe brain and especia lly of the corpora striata and optic t halamu shave bee n eliminated ca n a diagnosis of prima ry athetosis be made.o t rea tment affects t he course of th e disease. It persists through-out life , which it does no t see m to shorten,Senile Tremor. T his occ urs in old people freq ue ntly at th eage of 70 or th ereabout s. Th e fingc rs and thumb arc usually heldstraight, a t right angles t o th e hand s , and th e t remor in volvesboth fingcr s and hand and occas iona lly th e neck mu selos a reaffec ted , so th at th e pat ient constantly nods hi s head. Th e condit ion is no t associa ted with any men t al pec uliari tv and th e onl vpat hological con ditions are th ose charactcrist ic of old age. jharm resul t s fro m the condi tion and no t reatment is of th e leastavail in contro lling it .Toxic Tremor. Alcoho l or tobacco. lead, mer cury and ce rtainot he r metall ic poi son s or th e o rga nic poisons or auiointox ication

    may all ac t up on th e nerve ccnters a to pr odu ce a rath er irreg ular

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    PERIODIC P.IR.ILI"SIS 453tremor. Th e co nd it ion di sappears w ith th e elimination of thepoison.

    PERIODIC PARALYSIS(Family peri od ic paralysis)

    T hi s is a family di sease of rare occ ur rence in th i s co untry,cha rac terized by the occ ur rence of a lmos t o r quite tot al paralysis,from which th e patient rather spee dily appa re nt ly recovers.Et iology. Th e d isease is a lways hereditary. I t ap pears tofollow Mendel 's la w, th ough th c numbe r of cases on record is notsufficie nt to prove t he law by exact numbers.'I'he individua l attack may occu r wi thout recognizablc ca use ,

    but t hcy arc frequent ly p recipitated by m u scular exe rt ion or byoverea ting.P athology. A lmost no structu ra l changes arc constant ly present. Anexa mination of th e muscles some times shows sligh t vacuolization with ocr a-sionally a hypertrophied cell. Jn some cases no changes in eit her th e mnscleso r the centra l nervous sys tem a rc to IJe foun d upon th e most ca ref ul examination.The dimin ished exc retion of th e calc ium and magn esium sa lts in the urineseems to IJe fa irly constant. I t has been sugges ted that th e paralysis is du e toth e excess of these sa lts thu s retained within the blood whi ch inhi bits th e actiono f th e ner ve and muscle cells.D iagnosis. Th e symp to ms and hi tory a rc fairly typ ical. Th efam ily h ist ory shows th e hereditary ta int , w hile th c histor y of

    prev ious a ttack wi th recovery sho uld make th e diagnosis certai n.T here a rc prodrome usu ally of a vague discomfort , Thc patien tgoe to lcep an d awakens comp letely para lyzed . Occasiona llyth e pa ra lysis is not quite comp lete, but it usua lly invo lves all fourlimb s. Speech, th e sp h incte rs, t he respiratory mu scles and dcg lu-t it ion a rc not affec ted. T hc reflex es a rc clearl y d iminished ormay be ab ent. Th e mu scles do not usu ally co nt rac t in answe r toth e elcctric current . Th e heart is Ircqu cut ly found dilat ed onexa mination during an a tt ack. Thi s di sappears with recovery.Examinati on of th c urine sho ws so me a lbumin , so me blood andoc casiona lly so me hcmoglohin.Th e a ttack last s a fcw hours to a few days. 'I'h cn a ve ry p ro-found per spirat ion occ urs, followed by weakness, so metimes sleepand a gradua l recovery. Du ring conva lescence th c mu scles a rcve ry weak and st rcng th is rega ined only af te r so me days or weeksof g radua l im provement.

    T reatment . Th e treatment is devot ed to prevcn ting a t tacks.Ove reat ing, co ns tipa t ion, un du e mu scul ar exe rtion, are to beavo ide d. Th e co rrection of such bon y lc ion s as may be foundon examina tio n sho uld be of valu e in sec uring good nutrition andgood elimination.Prognosis. The prognosis is bar! for complete recovery ; isvcry good for re covery fr om any attack, and is bad for the descend-ents of the individual. Marriage sho uld be prohibited.