neuro5degen-3

8/12/2019 Neuro5degen-3

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Neuro 5 degenerativeA. Leah Kelly EdD, APRN, BC

DegenerationCan be in the CNS- a in !S, Par"in on# , Al$hei%er& , 'untington&Can be in Cord- a in ALS, !SCan be in PNS- a in (B, Char)ot-!arie toothCan be in yna* e- a in !(, radi)ulo*athyCan be in )ranial nerve - Bell Pal y!ulti*le S)lero iA *rogre ive, )hroni), i%%unogeneti) degenerative di ea e o+ the %yelin

heathProbably )ell %ediated autoi%%uneLot o+ ongoing re ear)h o tay tuned

See% geneti) u )e*tibility /R0S turn on1r2 environ%ental trigger

/%%une )ell are a)tivated by %yelin antigen and adhere toendotheliu% thin"ing the viru i %yelin %a)ro*hage eat the%yelin

!S Patho )ontd!yelin en itive ly%*ho)yte get into CNS

!yelin i de troyed- eaten by %a)ro*hage get *la3ue!ore in+la%%atory re *on e and %ediator relea edSo get le nerve )ondu)tion, lot o+ 4hite %atter le ion

See *at)hy de tru)tion thruout CNS

!ulti*le S)lero i1n et at age 6-76- %ore in 4o%en, )old )li%ate68 %ore )o%%on in relative -

E9a)erbation 4ith tre , in:ury, illne , *regnan)y, lee* *roble%So i+ *t ha *eriodi) neuro di ability 4ith later re)overy need to thin" !S;5< are rela* ing re%itting "ind but in =6 yr hal+ 4ill get e)ondary

*rogre iveCo t o+ !SDuration i about >6 yr and age on on et i >>6< have a evere di ability- ?6< are une%*loyedCare )o t about @>7,666 yr on to* o+ )o t o+ ABC %ed!S D8

ague, odd- "illed no4 4ith !R/Loo" at *la3ue , evo"ed *otential and CSPla3ue- in brain, )ordEvo"ed *otential - loo" at vi ion- o+t ee a an o*ti) neuriti

Che)" out *on and en ory tra)tCS 4ith elevated /g(



Sy%*to% u ually begin in one area but then go every4here!S diagno i'o4 it *re ent de*end on lo)ation o+ *la3ue 4h are +ound on !R/0 ually begin 4ith 9 in one area then they %ove aroundEvo"ed *otential 4ill %ea ure the travel o+ ele)tri)ity in CNS

i ual evo"ed *otential- in o*ti) nerve , )hia %- ee a o*ti) neuritiAuditory evo"ed *otential are in *onSo%ato en ory are in en ory *ath o+ brain )ord

!SNo ingle te t to a))urately deter%ine D8

No4 have revi ed diagno ti) )riteria=. Neuro di turban)e li"e that een in !S

. Sub:e)tive re*ort or ob erved>. Duration o+ in)ident 7 hr

7. >6 day bet4een event = !S 9

D9 i %ade by the y%*to%'o4 they )hange over ti%eLe ion in brain or )ord

y*e o+ !SRela* ing-re%itting RR!S

hi ha e9a)erbation re%i ion 4ith no *rogre ion- tart a;5< then 55<

La t 7hr - or %ore in a %onthPri%ary Progre ive-PP!S

Continual do4nhill 4or en 4ithout re tSlo4 te*4i e de)line over %onth - =6 <

Se)ondary Progre ive - SP!SE9a)erbate re%it 4ith lo4 *rogre ion56< o+ RR!S )ro to thi a+ter =6 yr - >6<

Progre ive Rela* ing- PR!SProgre ive 4ith a +e4 rela* e 5<

Area o+ !S involve%entCorti)o *inal i ue

'it dor al )olu%n in *inal tra)tPt *re ent 4ith ti++ne , lo4ing, 4ea"ne , +atigue'ave bo4el, bladder *roble% , he itan)y, urgePare the ia , abnor%al *ain, ti) doloreu9Proble% 4ith vibration, *o ition

Area o+ !S involve%entCerebellar i ue



S*a ti)ity in li%b 4ith ata9iaDy arthria, intention tre%or, ny tag%u

hi i )alled Char)ot# triadNo )oordination, balan)e i ue , vertigoLehr%itte# ign- ho)" li"e en ation along *ine 4ith +le9ion o+ head,

hy*erre+le9ia and a Babin "iArea o+ !S involve%entCerebral i ue

!ood )hange- eu*horia, de*re ion, labilityAn9iety, de*re ionEarly thin"ing *roble% , )an#t u e *ee)h, ee 4ell, *roble% 4ith

)on tru)tion, )al)ulationSo try !!SE/%*aired re)all but good re)ognition/ntelligen)e, language %e%ory inta)t

hi )reate e%*loy%ent i ueArea o+ !S involve%entBrain te% i ue

i ual i ue - blind *ot , )oto%a1*ti) neuriti , ny tag%uParaly i o+ lateral ga$eBlurred vi ion and i%*aired )olor *er)e*tion

!o t )o%%on 9atigue- ;;<al"ing i ue - ;?<

Eva)2void dy +- <Pain- 6<i ual i ue 5;<

Cognitive i ue - 77<re%or 76<

Cour e o+ !Sill ee o+t e9a)erbation re%i ion

1+t 4ith te%* elevationAl o i ue 4ith )al)iu% %etaboli %!ay get +o)al nerve atta)"

Shooting tingle , udden ata9iaeel li"e are lo ing %ind

!S t9 in a)ute e9a)erbationSo%eti%e are o i)" need to )o%e to ho *ital- o+t be+ore are D9'i do e teroid - Solu%edrol to redu)e a)ute in+la%%ation

-=5 %g2"g2day- go +ro% / Solu%edrol to oral *redni oneat)h +or ide e++e)t o+ %ood )hange, (/ bleed, elevated BS, blurred

vi ion, +re3uent urination



at)h +or i%*aired 4ound healing in+e)tion!S treat%ent

or"ing on tran *lanting oligodendro)yte that )an *rodu)e ne4 %yelin!ono)lonal antibodie to F CD7 )ell and u**re i%%une re *on e/ i%%unoglobin

/%%une *rotein to redu)e the in+la%%ation/%%une !odulator - /nter+eron

he A, B C -- inter+eron that are body *rotein %ade in re *on e to +oreignti%ulu

Avone9Beta eronCo*a9oneRebi+ And no4

Novantrone

Antegren/nter+eron B=b- Beta eron

hi i +or rela* ing re%itting "ind(iven ub G to redu)ed ne4 le ionBlo)" %yelin brea"do4n/n:e)t under the "in every other day/nter+eron B=a- Avone90 e daily to* only +or SERedu)e rela* e in rela* ing +or%(iven on)e a 4ee"

(et generally +lu-li"e ide e++e)t/nter+eron B=a- Rebi+ 'igher do ing than Avone9Auto%ati) in:e)torCo*a9one- (lati%er a)etate

or rela* ing +or% - a non-inter+eron, non teroidalSti%ulate antigen *e)i+i) u**re or )ellSyntheti) %yelin ba i) *rotein- redu)e the de tru)tion o+ %yelin

hi i a daily in:e)tionNovantrone- %ito9antroneSlo4 the *rogre ion o+ the neuro di abilityChe%othera*y 4ith a %a9 li+eti%e do eindi)ated +or redu)ing neurologi) di ability and2or the +re3uen)y o+ )lini)al

rela* e in *atient 4ith e)ondary *rogre ive, *rogre ive rela* ingand 4or ening rela* ing-re%itting !S.

A%*yra- dal+a%*ridineNe4 %ed- to hel* 4al"ing in *eo*le 4ith !S that i u ed in )o%bo 4ith other

%ed



1nly thing i it )an )au e ei$ure - i%*rove *eedy abri

Natal$u%abor rela* ing +or% o+ !S- de)rea e rela* e +lare u*

Be t in tho e 4ho )annot tolerate other %edNot good 4ith 4ea" i%%une y te%- lot o+ allergie

eird in)iden)e o+ %ulti+o)al leu"oen)e*ahalo*athy P!L1ther treat%entAntegren- et)

/n trial H an adhe ion %ole)ule blo)"er that lo4 the *rogre ion o+brain le ion

1ther trial 4ith hor%one - e tradiol and *regnan)y i good +or !SStatin at a higher than )ardia) do eRe%yelination -Iale i tran *lanting S)h4ann )ell in brain or te% )ell/N the *i*eline

)ladribine and +ingoli%od, better than e9i ting treat%ent or *la)ebo, )trial o+ o+ >,;66 *atient .

ingoli%od daily v Cladribine i ta"en in hort )our e .da%*ing the auto-i%%une re *on e through )hroni) in+la%%ation.J ri " o+ ide-e++e)t

1ther t9Sy%%etrel +or +atigue- energi$ing antiviralProvigil +or +atigue and 4a"eningAvitrol +or 4ea"ne and eye

0rinary- detrol, ditro*an- late boto9 to %u )leS*a ti)ity- ba)lo+en, $ana+le9, dantriu%, valiu%re%or- inderal, bu *ar, %y oline

Pain- neurontin, lyri)aCognition- ari)e*t, na%endaA)ute e9a)erbationSolu%edrol- and de)adronCyto9an/nter+eron/ /(-*la %a*here i!S n g d9Kno4ledge de+i)itChroni) *ainAlt *hy i)al %obility, alt a+etyDe*re ion

he abandon%ent i ueinal long ter% )are



1ther !S interventionNo4 are living a near nor%al li+e- *an %any 4or" +ull ti%eDeal 4ith )on ti*ation, a)tivity intoleran)eAlt *hy i)al a)tivityPar"in on# Di ea e/n =< o+ *o*ulation at age 55

hen >< by age ;5'arry ru%an,, anet Reno,PDPD0 ually begin at age 55Iounger vi)ti% have a di++erent +or%Di ability )o%e +ro% tre%or, rigidity, balan)e and )oordinationSeveral ty*e

Po tural in tability gait *roble%

re%or a %ain *roble%1+t begin a y%%etri)ally- gradual *rogre ive1+t do not ee tre%or at +ir t

Di++erent ty*e(eneti) - a *roble% o+ )hro%o o%e ,7,

!ay relate to *e ti)ide e9*o ure1r *o t en)e*haliti - a in a4a"eningSo%e AS'D, to9in, trau%a

o9in are C1 *oi oning, )yanide, %ethanol,Drug indu)ed +ro% de*leting tore o+ do*a%ine a in o%e * y)h

drugAl o o%e treet drug de troy ub tantia nigra *athhe Par"in oni % - drug indu)ed, e ential tre%or, nor%al *re ure

hydro)e*halu , u*ranu)lear *al y, Shy Drager, Le4y BodyPar"in on# Patho*hyNor%al %ov%t need do*a%ine- a )he%i)al that %ooth body %ove%ent

he e9tra*yra%idal y te% i re *on ible +or %ove%ent, *o ture,balan)e, 4al"ing

Degeneration o+ do*a%ine *rodu)ing )ell in ub tantia nigra o no tran *ortto triatu%- o thi i a nigro triatal di order- triatu% i )oord )enter+or ev neuro)he%i)al and no4 have %ore AC' than do*a%ine- o*roble%

PD(et y%*to% 4hen have ;6< )ell deathtre%or al o involve erotonin56< have trouble 4allo4ing!o t have a %a " li"e +a)e 4ith no blin" or e9*re ionBa al ganglia al o lie near the hy*othala%u o have *roble% li"e e9)e



*er *iration, ortho ta i , ga tri) urinary retention)o%*ari on1n PE0*ta"e i de)rea ed in *o terior *uta%en and ee hy*er%etabli) triatu%Lot o+ non %otor y%*to%

See *inal )ord tu++ +ro% *on!edulla- *roble% 4th autoni%i) NS/ ue o+ %ell di++eren)e- %ay lo e %ell +ir t a in al$hei%er

Cla i) PD 9 RAPRe t tre%or

/ i a y%%etri), regular rhyth%i), then get alt +le9ion e9ten ion!ove%ent blo)" the tre%or 4hen thala%u ta"e )ontrol

Rigidity/n)rea ed re i tan)e to *a ive %ovt +ro% invol )ontra)t---re ting

%u )le are )ontra)ting- o+t )ra%*

Cog4heel or lead *i*e )on tant uni+or% re i tan)eMBrady"ine iaPo tural /n tability lo or re+le9e

Cla i) PD 9 RAPRe t tre%orRigidityBrady"ine ia

Can#t initiate %ovt, in)rea ed rea)tion ti%e, lo4 to %oveStriated %u )le +eel 4ooden- it till +or hr - )an#t turn in bed

hen get a"ine ia, dy arthria, drooling ialorrheaMill +ree$e in *la)ePo tural /n tability

(et a +e tinating gait- lo4 hu++ling- *ro*ul ive and retro*ul ive'ead i +le9ed +or4ardPoor balan)e, +all ea ily

!ore PD 9E9)e androgen - grea y, eborrhea

i ual di turban)e ble*haro *a %Can#t o*en eye or "ee* the% o*enLittle blin"ing

Sen ory nu%bne - tingling, a"athi ia, urinary i ue6< be)o%e de%ented- %o t are de*re ed, a*atheti), de*endent

ill have S ! de+i)it , lo4 thought ,PD De*re ionSee in 56< o+ *atient

1+t +ir t are de*re ed and then develo* the tre%or



R2t la)" o+ )ate)hola%ine!ay al o have an agitated de*re ion

!ay al o have lee* di turban)eSlee* rever al- D A, E!A

htt* 22444.youtube.)o%24at)hJvHyl'O 1=? ?6htt* 22444.youtube.)o%24at)hJvHeKh1 aI-INo +eatureH+v4relNot to )on+u eAbout 5< o+ *o* have benign e ential tre%or- that# +a%ilial

A y%%etri)al tre%or that i +a t and ee be t 4ith ar% out tret)hed1ther )au e o+ PD are drug - anti* y)hoti) , Reglan- H Par"in onianA RES RE!1R di a**ear 4ith voluntary %ove%ent- le evere 4hen

rela9edNo tre%or in lee*

A +e4 4ith he%i*ar"in oni %- :u t = ide

Progre ion o+ PDell via PE )an ee *oor u*ta"e in Puta%en

0nilateral tre%or rigidityhen bilateral tre%or rigidityhen un teady +allhen need a )ane, 4al"erhen in 4heel)hairhera*y +or PD

!ain tay i %edi)ationo alleviate ign y%*to%

1+t 4on#t treat til 9 are real bad!o t 4or" on L-Do*a or blo)" )holinergi) +iberry no4 to give non ergot do*a%ine agoni t to )ontrol y%*to% and

allo4 L-Do*a delayL-do*a i till the gold tandard

L-Do*aA ynthe i$ed a%ino a)id that )an be inge ted and tran +or%ed by brain

*rotein to %a"e do*a%ine- but little ta"en in i )onverted o al%o tnone get thi *ure )o$ do*a%ine )annot )ro blood brain barrier

/n early PD brain )an )onvert L-Do*a but not o %u)h later onSo u ually given a Sine%et 4hi)h i )o%bo o+ levodo*a and )arbido*aCo%boLevodo*a i )onverted to do*a%ine by en$y%e D1PA de)arbo9yla e in CNS

PNS to i%*rove PD 9- but a)tivation in *eri*hery )au e N - ogiven 4ith )arbido*a 4h )annot )ro blood brain barrie but *revent)onver ion in *eri*hery o no ide e++e)t

Plu %a"e %ore do*a%ine avail to CNSSine%et



hi i Carbido*a- Levodo*ahi i in - the < o+ ea)h a 52=66

S%aller do e %ore +re3uently are hel*+ul v /D- )an ta"e u* to ;tab 2day

Carbido*a i le+t o+ la h- "ee* +ro% 4a ting the L-Do*a al o*revent N

hi i %ore *otent in %all a%t and hel* )ontrol (/ 9- inhibit gigen$y%e

CR +or%ulation )an be halved, not )ru hed- la t longer in blood oteady a%t to brain

Sine%etSe )on ti*ation, o%nolen)e, vivid drea% , hallu)ination a 4ell a

ortho ta iAl o lot o+ dy "ine ia , dy tonia , +atigue, )on ti*ation, diarrhea, urine

i ue , ortho ta iCR +or%ulation )o t %oreNever to* thi abru*tly

Co t i @;72%onth

anti)holinergi)he e 4or" on the a)etyl)holine to "ee* balan)e

ArtaneCogentinKe%adrinBenadryl

he e are +or the tre%orStart 4ith 1 C benadryl in tre

he e are al o +or anti* y)hoti) tre%orDanger o+ )on+u ion2hallu)in in elderSy%%etrel

hi i a%antadine- an antiviral +or the +lu but hel* 4ith %ove%ent i ue -a"ine ia, rigidity

See% to blo)" reu*ta"e o+ do*a%ine and in)rea e relea eSe- nau ea, hy*oten ion , hallu)ination

or" on N!DA re)e*tor-Al o i anti+lu1+t tarted a %onothera*y- o+t to* tart to o*ti%i$e 9 relie+ =66->66 %g early in PDDo*a%ine Agoni t

he e a)t by ti% o+ do*a%inergi) re)e*tor o need no )onver ion- o+tentart 4ith the e!any neurologi t no4 tart 4ith the e to hold o++ on need +or



Sine%et- the e are adverti ed +or re tle leg , again AN , hallu)Per%a9Parlodel!ira*e9Re3ui*Ne4 *at)h- RotigotineNon ergot do*a%ine agoni t- on)e a day *at)h )over 7 hr - good 4ith tho e

4ho )annot 4allo4- and no)turnal 9Start 4ith CoG tooSlee* in PDA di ea e advan)e - get %ore lee* di turban)e - and %o t do*a%inergi)

drug are edationSo hould not drive1r o%e are given *rovigilRe3ui* !ira*e9

Re3ui*-Ne4er %ed- do*a%ine re)e*tor agoni t +or early t9 a ad:un)t toL-Do*a- al o +or re tle leg

=. 5 %g B/D, ad:u t u* to 6 %g B/DSe are di$$y, lee*y, nau ea, hallu)inateCo t @=>52%onth

Al o in grou* i !ira*e9-*ra%i*e9oleAn agoni t too- u* to =.5 %g /D, a%e SE a above- )o t @=6?2%onth

Parlodel1lder do*a%ine agoni t- =. 5 %g B/D

Ad:u t u* to 6 %g B/D(ive nau ea, heada)he, di$$ySide e++e)t hard here but le li"ely to get dy "ine ia , and dy tonia

So o+t on the e do*a%ine agoni t rather than L-Do*aAP1KINQ in:e)tion

a*o%or*hine hydro)hlorideM.6 %L .6 %L.6 %L during o++T *eriodSE Nau ea, vo%iting, lo4 blood *re ure, lee*ine , dy "ine ia ,

hallu)ination , )he t *ainAd:un)t levodo*a thera*y to treat o++T *eriod - ne4C1! drugCo%ta% the e are )ate)hola%ine -%ethyltran +era e inhibitor - thi i

en$y%e all over body brain'el* 4ith %otor +lu)tuation

he e drug are u ed 4ith Sine%etE9tend the duration o+ L-Do*a but u e it only 4ith Sine%et to hel* u e

a lo4er do ageAl o u e to hel* 4ith 4earing o++ *heno%enon

a %ar- )an enter brain



Co%tan- doe notSine%et enta)a*oneStaveloN!DA re)e*tor antagoni t

hi ha enta)a*onein it i%*rove 9 and 3uality o+ li+e, but till get idee++e)t

Seligiline- Elde*rylhi i a neuro*rote)tor- to lo4 *rogre ion at 5 A! and =P!

/ an !A1 4ith no +ood re tri)tionCannot ta"e 4ith De%erol o d2) be+ore urgeryAl o i a *at)h %ar"eted a E!SA! +or treat%ent re i tant de*re ion

Ne4 !ed- A$ile)t-ra agilineA ne4 o*tion- !A1-B +or initial t9 or +or )o%bo thera*y along 4ith Sine%et

to blo)" brea"do4n o+ do*a%ine- i%g

But i%ilar i ue are hallu)inationCannot ta"e 4ith SSR/Al o avoid 3uinolone , gra*e+ruit :ui)eCo en$y%e G0 e 4ith tatin - u e 4ith *ar"in on# o+ten +ir t

Anti* y)hoti) ne4 %edAnti* y)hoti Do not )au e PD but are o+t a))u ed

hey %ay un%a " or )au e it to a**ear earlier

Ne4 e%erging agent in *at)h and other +or% to treat- rotigotine ne4do*a%ine agoni t- in *at)h- a**ly any4here- on)e daily 4ith )ontd drugdelivery - good in 4allo4 *rob , no)turnal i ue

Par"in on i ue1)) have Par"in onian )ri i

Do e related %otor +lu)tuationre%or, de)rea ed de9terity, vo)al o+tne , lo o+ lee* re+re h%ent,

re tle ne1+t ee delayed ga tri) e%*tying, r2t a *rotein %eal or anti)holinergi). (et e9er)i e

1n-1++ Re *on ehi i a +lu)tuating re *on e- 4ith udden un*redi)table hi+t bet4een

under over treat%entSee abru*t )hange in %u )le tone - +ro% rigidity o++M to nor%al onM then

ba)" to o++ againSee thi a+ter 5 year o+ treat%ent1n- have L do*a re *on e 4ith good %obility and +e4 y%*to%1++ no re *on e 4ith return o+ y%*to% o are di ru*tive, de*re ed



an9iou!ay need Clo$aril

earing 1++ Pheno%enonE++e)tivene o+ e9ternal L do*a regularly and *redi)tably de)line

Al o )alled end o+ do e +ailureNeed to in)rea e do ing interval , get %aller do e , or give ti%e relea e

+or%ulationAl o add C1! %ed!ay get en ory i ue , * y)h, %otor +lu)tuation at ti%e o+ do e +ailure

*are the iaDy "ine iaDy "ine i) %ove%ent - )horei+or% %ove%ent and %yo)lonu

Re*etitive %ove%ent o+ lo4er li%b1+t ee at ti%e o+ *ea" do ing

Lo4er the do e or add do*a%ine agoni t li"e y%%etrelDy tonia by de+inition i a *ain+ul u tained *a % 4ith *o turing 4hen L-

do*a i lo4 a in early A!htt* 22444.youtube.)o%24at)hJvH +=N6O+5/3A

ree$ingStu)" in *la)eDi++i)ulty tarting to 4al"-So +allAnd 4ith it i evere burning *ain, re tle leghtt* 22444.youtube.)o%24at)hJvH3' r0I "Cg NRH=

reating the P y)h *artor the de*re ion 4ill need an antide*re ant- either ti%ulating or edatingde*ending on *re entation

'allu)ination +ro% overdo ingH to* the y%%etrel, agoni t oranti)holinergi)

Redu)e the do e o+ L do*aAdd $y*re9a, )lo$aril or ero3uel

or lee* *roble% give CR +or%, de yrelAdd )a++eine in dayti%e

Surgery in Par"in on&Autologou tran *lant o+ adrenal %edulla

etal tran *lanthala%oto%y +or tre%or

Pallidoto%y +or dy "ine iaDone under %i)ro )o*e 4ith !R/ guidan)e0 u only do = ide o +e4er )o%*li)ation

hala%oto%y +or re%orSa%e ga%%a "ni+e irrad



hen *o t o* )reate a le ion/rradiate the !L thala%i) nu)leu o neuron no longer tran %itPallidoto%y

or dy "ine ia , rigidityor tho e 4ith advan)ed *rob

De troy the nu)leuStereota)ti) urgery under !R/ guidan)e0 u only one ide

etal tran *lantStill not doing a 4ell a ho*edDBS- *a)ing the brainEle)tri)ally ti% target *art o+ brain- o dee* brain ti%ulation to re*la)e

hala%oto%yo ub thala%i) nu)leu , globu *allidu or !/

1r other %odalitie

/n DBS 4h ub titute +or thala%oto%y- ele)trode into thala%u to *a)e thetre%or )au ing )ell

Al o loo"ing to %ole)ular biology +or gene thera*y/dea to *a)"age and deliver do*a%ine )ell traight to brainCollagen into vo)al )ord to i%*rove voi)e!ed via *at)h and *ill

Nur ing intervention in PD!o t 4ill need * y)h u**ort

ill al o need hoo"u* to "no4 4hat i the a)tive area o+ re ear)hShould not go to u**ort grou* initially- 4ait til into the *roble% a 4hile-

and e9er)i e trenuou ly1ngoing evaluation o+ ADL - dre ing, groo%ing, 4al"ing, 4riting, drivingAvoid any ri " +or +all tu++- li"e )atter rug , too %u)h +urniture, hard

+au)et(et a lo4 bed 4ith ar% re t , grab bar , 1 %odNur ing intervention in PDE9er)i e +or %obility +le9ibility

o *revent )ontra)ture , %u )le 4ea"neea)h ro)"ing and rhyth%i) rela9ation o 4ill %ove el+UU..ea)h balan)e and energy )on ervation

Need breathing and gait training ha% tring tret)h'igh te**ed %ar)hing

S*ee)h thera*y +or the tongue *rotru ion, li* %a)"ing, gri%a)ing(et to *ra)ti)e 4allo4ing, eating, )he4ing

N g in PDluid %anage%ent- need at lea t 666 ))

'el* 4ith )on ti*ation- al o +iber, e9er)i e'i )al, lo4 *rotein diet )o$ *rotein blo)" the e++e)t o+ L-Do*a- or *read



out the *roteinAvoid iron large %eal that al o blo)" do*a%ine al o

Allo4 ti%e +or )o%*letiona"e Sine%et >6 %inute be+ore eating

Drin" lot o+ 4aterPD alternative thera*ie!a age a)u*un)ture are goodS4i%%ing )laCoen$y%e G a %ed that hel* J 'o4Re tle LegA )o%%on %ove%ent di order a))o%* by lee* )o%*laint 4ith irre i tible

urge to %ove leg in re t at nite- 4ith un*lea ant en ation - 4or en atre t- al o

a)hy,burning, )ree*ing, )urrent , )ra4ling, re tleRelieved by %ovt, tret)h

Cau ation, *atho'ereditaryiron de+i)ien)y o loo" at eru% +erritinAl o r2t )a++eine, ni)otine, etoh, antide*re antal o di ru*tion o+ do*a%ine )tr in ub)orte9Al o hy*ere9)itable *inal re+le9e

Re tle leg!ore in 4o%en

ir t +i9 iron, %ove%ent, e9er)i e

reat%ent i do*a%ine agoni t - u u not ergot a +ir t line-Ro*inirole- Re3ui*- 6. 5 %g daily DAAl o Sine%et- e * i+ inter%ittent but %ay not 4or" a 4ell 52=66(aba*entin- +or le inten e or i+ neuro*athyDarvo)et, )odeine, or non ben$o lee*er

!ovt videoeb)li*

htt* 22%ed4eb.bha%.a).u"2' P2de*t 2)linVneuro2tea)hing2tutorial 2*ar"inon 2*ar"in on .ht%l

!ya thenia (raviChroni) *rogre ive autoi%%une di ea eProble% at neuro%u )ular :un)tion- 4ith a *roble% in A)h re)e*tor

Lo o+ a)etyl)holine in *o t yna*ti) neuron related to autoantibodie

See )lo e a o)iation 4ith lu*u RAProble% o+ voluntary, triated %u )le



Can be *re)i*itated by %y)in , Beta Blo)"erhe *roble%

!1RE !IAS 'EN/AIoung 4o%en %en a+ter 56See +lu)tuating 4ea"ne +atigue

/nitial *re entation i +atigueor en 4ith e9er)i e, i%*rove 4ith re t

Danger 4hen atta)" )he t dia*hrag%hen lo e )ough, ability to ta"e dee* breath

Al o eye y%*to% --*to i , di*lo*ia= t *re entation in 76<, total o+ ;5< have eye 9

!ya thenia S9atigue

Eye i ue *to i , double vi ion!otor *roble% tone, dy *hagia, +a)ial droo*, 4allo4 *roble%

Develo* a narling +a)e- )an#t %ilePro9i%al %u )le 4ea" Patho o+ !(Nor%al neuro%u )ular :un)tion relea e a)etyl)holine +ro% %otor nerve

ter%inal in *a)"age - li"e boat going a)ro the ga*he e +loat a)ro the yna* e and bind to re)e*tor on %otor end *late

Sti%ulation o+ %otor nerve relea e the e *a)"age or boat +illed 4ithA)h to +loat a)ro and de*olari$e the o** nerve and )au e a)ontra)tion

the yna*ti) %e%brane on hore i +olded o

are redu)ed re)e*tor+e4er re)e*torAl o antibodie on %e%braneAl o en$y%e "ill the hi* in tran *ort

D9 o+ !(/nitial vie4 o+ y%*to%

hen 4ill do en ilon te t / or do /! Neo tig%ine te tith en ilon, *t 4ill have i%%ediate return o+ trength, hi i al4ay

done 4ith *to iAl o no4 loo" at hi eru% level o+ A)hR antibodie thi ho4 the

i%%une *roble%D9 o+ !(Al o 4ill do C o+ thy%u - =5< have thy%u tu%or or ti ue )hange but

till do not "no4 4hy

Di ea e )hara)teri$ed by e9a)erbation re%i ion 4ith +lu)tuating everity



!ed in !((ive Anti)holine tera e %ed 4h retard the brea"do4n o+ a)etyl)holine at

yna* e(ood +or o%e, not other

ill inhibit the en$y%e!e tinon and Pro tig%in

Do age 4ill vary 4ith *atient, level o+ tre , heat!u t give on ti%e every 7- hr to "ee* level evenSo i+ *t i o++ +loor, end 4ith *atient'a %u )arini) ide e++e)t - diarrhea, )ra%*Cannot )ru h

!ed in !(Al o de)rea e the in+la%%ation 4ithPredni one

'i do e til ee i%*rove%ent then ta*er o++

hat are ide e++e)tAl o give / i%%unoglobin - / /(/%%uno u**re ant

Cy)lo *orine, a$athio*rineNur ing Care !(/%*ort o+ )ough and 4allo4- )he)" and en)ourage

i%e a)tivity to *ea" o+ %edear a %edi alert bra)elet

Do *la %a*here i - to re%ove antibodie - *rote)t the a))e iteat)h lyte

hy%e)to%y +or !(Do a %edian ternoto%y thy%e)to%yill be be t about >-5 yr a+ter urgery

!ya theni) )ri iSudden 4or ening u ually *re)i*itated by inter)urrent in+e)tion

Al o a +un)tion o+ under%edi)ationSo %ight in)rea e the drug do eSee ' N, re *iratory di tre ,Pt 4ill have no )ough or 4allo4

ill be in)ontinent!ay need to be intubated

So reat the in+e)tion, give %ore %edCholinergi) Cri i -!('ere the *atient i over%edi)ated!ay loo" li"e %ya theni) )ri i but i o**o ite'ere *t ha abdo%inal )ra%* , diarrhea, e9)e *ul%onary y%*to%An9iety, 4eating, +a )i)ulation , blurred vi ion



Re *iratory )o%*ro%i e i 4or ePt need to be intubated and then %ed 4ear o++ So%eti%e *la %a*here iblood i e*arated into )ell and *la %aW *la %a i re%oved and re*la)ed

4ith +re h +ro$en *la %a, and thi al o re%ove the antibodie that )au ethe 4ea"ne - 5 e9)hange hel* +or about %onth

ALS- a%yotro*i) lateral )lero ihi i al o "no4n a Lou (ehrig# di ea e

!o t )o%%on %otor neuron di ea eA di ea e o+ %iddle aged %en-Al o in Ste*hen 'a4"ingPt 4ill urvive about -=6 yr and die o+ re * in+e)tion5666 diagno ed ea)h year but no )lue a to 4ho%ALS

hi i a di ea e o+ %otor neuron to voluntary %u )le +ro% degeneration o+anterior horn and )orti)o *inal tra)t

0**er %otor neuron begin in brain and goe to )ord- degeneration in u**er%otor neuron lead to *a ti)ity and overa)tive re+le9e

1ther 0!N *rob are *ri%ary lateral )lero i , * eudobulbar *al yALS 9ALS Di ea e o+ %otor neuron to voluntary %u )le +ro% degeneration o+

anterior horn and )orti)o *inal tra)tLo4er %otor neuron begin in )ord and yna* e 4ith 0!N- )o%%uni)ation

bet4een t4o i via gluta%ate- and then to the %u )leDegeneration here evere 4ea"ne , *araly iALS i both 0!N and L!NSo an i ue o+ %etaboli %, tran *ort and gluta%ate torageALS

X6< have no hereditary *attern0 ually begin 4ith )lu% ine , +atigue, *a ti)ity, hy*erre+le9ia, tri**ing,

%u )le t4ingehen get *roble% o+ 4allo4ing, )he4ing, *ea"ing, or %ove%ent

So have dy arthria, dy *hagia, or droolingSo 4ith u**er lo4er %otor neuron i ue get 4ea"ne o+ all voluntary

%u )leEye %u )le , anu , and bladder are u ually *ared

So no in)ontinen)e and till +un)tion e9ually

ALSDiagno e by )lini)al *re entation and EE(



1nly *ain i *ain o+ i%%obility!ental tatu i +ine- but 4ill ulti%ately die o+ re *iratory +ailure!ay have e9aggerated laugh, )ry, ya4n

1+t have labile e%otionhi i relentle ly *rogre ive and 4ill die by *neu%onia )o$ o+ 4ea" re *

%u )leALSNo i ue o+ )ognition or intelligen)eSo are +ully a4are o+ every *roble%, lo

ill u e *o itioning, *lint , breathing e9er)i eAnti)holinergi) and antihi ta%ine +or the e)retion

!ed - ALSRilu$ole-Reluten

Prolong urvival

Anti gluta%ate- 56 %g B/D 4ill )o t @ 662%onthNo )ure but lo4 degeneration and *rote)t 4hat i le+t

NeurontinAnti)onvul ant to redu)e level o+ gluta%ate at yna* e bet4een 0!N

and L!N8 ALS

No4 trialing nerve gro4th +a)torCollateral reinervation to rea)h 4ido4ed %u )le +iber and *re erve

neuron )ell bodie/( , (ND , BDN

0 ing brain derived neurotro*hi) +a)tor!yotro*hin lo4 %u )le degenerationALSLive in ho*e and +o)u on 4hat till )an doSet rea onable, attainable goalSu**ort the +a%ily and *atient(ive +luid , *o ition, %all +re3uent high nutrient +eed

ill give %u )le rela9er , li"e valiu%, Ba)lo+en +or the *a ti)itySo 4ill do R1!, "ee* u*right, e9er)i e, avoid +atigue

ALSill do in)entive *iro%etry

(ive *ul%onary )areor" 4ith *ee)h

Stay on vent to hel* 4ith re * *araly i56< 4ill die in -5 yr

5< 4ill la t 5 yrSo%e 4ill live u* to 6 yr

(uillain Barre Syndro%e



A)ute in+la%%atory de%yelinating *olyneuro*athy- 4here ly%*ho)yte and%a)ro*hage eat the %yelin heath or *eri*heral or )ranial nerve - ohave de%yelination and a9on degeneration

Progre ive *olyneuro*athySee% to be autoi%%une o+ J 1rigin

Degeneration in *arti)ular in *eri*heral nerve!a:or )au e o+ generali$ed *araly i in)e *olio

'ad 0R/ everal 4ee" be+oreSee%ing li"e to E* tein Barr, '/ , )a%*yloba)ter

(uillain BarreCS 4ith in)rea e *roteinNerve )ondu)tion velo)ity lo4ed- 4ith en ory and %otor loPre ent 4ith *araly i

!o t )o%%on ty*e i a )ending *araly i but are other variantDe )ending 4ill have ra*id re *iratory involve%ent

or t at 7 4ee" * in+e)tion- u ually y%%etri)ally 4ea"- )an#t get u*D R gone, ee *are the ia , %u )le *ain, 4ea"ne and neuro*athie(B(BBigge t danger i re * %u )le *araly i

5< 4ill have total *araly iRe%yelini$ation %ay ta"e year to be )o%*leteSo a e %ent i

ea"ne , nu%bnePain in 56<- o+t evere 4ith %ovt- houlder, thigh

Loo" +or the autono%i) nervou involve%ent 4ith )ardia) i ue ,dia*hore i , lo o+ *hin)ter , ileuCan be li+e threatening

(uillain Barre!a:or i ue i dy autono%ia

Arryth%ia , %alignant hy*erten ion, hy*oten ion and te%*eratureirregularitie

!ay need re *iratory and )ardiova )ular u**ortill %o tly re)over at %o yr

56< 4ill +ully re)over>5< 4ill have re idual de+i)it=5< 4ill have handi)a*

(uillain BarreDiagno ti) te t to rule out other di orderLP CS 4ill ho4 elevated eru% *roteinE!( 4ith lo4ed nerve )ondu)tion(B treat%ent



Re *iratory u**ort- even ventilator/ i%%unoglobin - / g 8 5 dayStool o+tener0 e neurontin, tegretol +or *ain*la %a*here i ,Care i u**ortive-

ill need rehab P , 1 e9er)i e, loveno9 e%otional deva tation- ;5< re)overN g D8 (B/ne++e)tive breathing *attern r2t neuro%u ) 4ea"neA)ute *ainAlt ti ue *er+u ion r2t the dy autono%iaKno4ledge de+i)it

rige%inal NeuralgiaAl o )alled ti) doloreu9

Pain along the 5th )ranial nerve- the trige%inal that ha > bran)hein any o+ the > bran)he=2 5,666 - %ore o+ten in 4o%enand u ually in %iddle age

/ntrin i) le ion 4ithin nerve- *re ent li"e !SAl o e9trin i) le ion +ro% )o%*re ion tret)h, dental ab )e e

i) Doloreu9

YSevere *ain i%ilar to an ele)tri) ho)"M in the +a)e, %outh, or teeth o+ thea++e)ted ide.

Y 'eightened en itivity around the %outh and no e, 4hi)h, 4hen tou)hed,%ay trigger an atta)". 1ther thing u)h a e9*o ure to )old, eating ordrin"ing, *arti)ularly )old li3uid or +ood, bru hing the hair or teeth, or4a hing the +a)e %ay al o trigger atta)" .

Y /nvoluntary )ontortion al o "no4n a ti) douloureu9M o+ the +a)e inre *on e to the *ain.

Y Bout o+ *ain +or everal 4ee" or %onth in a ro4, +ollo4ed by a*ontaneou )e ation o+ y%*to% . Period o+ re%i ion %ay la t +or

day or year , but they tend to be)o%e horter a the *atient age .

rige%inal NeuralgiaDaily or inter%ittent *aro9y % o+ e9)ru)iating *ain-u u in 4o%enLearn 4hat )au e - )he4, tal" et)- avoid1+t re)ur at interval - +eel +ear or return de*re ion1+t +ro% )hroni) )o%*re ion- *ul ating ve el, 4ear a4ay nerve

*rote)tion- or !S(et !R/ to loo" +or )au e



/ntervention i) Doloreu9ant to 3uiet do4n the nerveegretol- 4at)h CBC, liver

DilantinLiore altri)y)li)Surgerie li"e nerve blo)" , rhi$oto%ieNur ing intervention are to4ard

PainStarvationtear

i) Doloreu9Surgery

!i)rova )ular de)o%*re ion to "ee* ve el o++ o+ nerveRadioablation- *robe thru )hee" and ablate nerve

Balloon de tru)tion o+ nerve- balloon %i)ro)o%*re ion(ly)erol in:e)tion into nerve to "illStereota)ti) radio urgery 4ith ga%%a "ni+e to ablate- but ide e++e)t %ay be

nu%bne/ntervent i)/n (a%%a Kni+e Radio urgery, a ingle highly )on)entrated do e o+ ioni$ing

radiation i delivered to a %all, *re)i e target at the trige%inal nerveroot. (a%%a Kni+e Radio urgery i non-inva ive and avoid %any o+ theri " and )o%*li)ation o+ o*en urgery and other treat%ent . reat%entre ult in long-ter% *ain relie+ in nearly X6< o+ the *atient . So%e lo

o+ en ation nu%bne M o))ur in =6< to =5<Bell Pal yhi i at ?th )ranial or +a)ial nerve

!otor *araly i0nilateral *re entationCould be +ro% her*e

hi i generally *ain +ree o thin" are having a tro"e

9 i analge ia, teroidorry about )orneal *rote)tion

BellBell Pal ySee *re entationRadi)ulo*athie

he e are nerve *roble% o+ the a9on that travel u* and do4n the te% tothe )ord

Are ea ily in:ured- *arti)ularly 4here *inal nerve )o%e o++ )ordSee )o%*re ion, in+la%%ation trau%a



Radi)ulo*athy di order o+ *inal nerve rootRadi)uliti

rau%a )an tear, avul e di ru*tSee )hange in trength, tone and %u )le bul" Pain, *are the ia al o )alled )au algia

Radi)ulo*athiehe e are nerve *roble% o+ the a9on that travel u* and do4n the te% to

the )ordhi lead to need +or *ain %edi)ine

/n)rea ing do e o+ o*iateAddi)tionLo o+ 4or" and +un)tion!ay u e ten unit , neurontin, elavil but *ain i o+t in)a*a)itating

S*inal urgery again!a:or )hange no4- %ini%ally inva ive te)hni3ue - %all in)i ion

Lu%bar %i)rodi )eto%y i done out*atient!etalli) in tru%ent to u**ort un table ti ue a bone gro4 over

yr0 e di++erent angle and lot o+ )re4 , *late , rod/ntrao* +luoro hel* to *in*oint areaS*inal teno i no4 )o%%on in elderly o o+t u e arti+i)ial 4edge

i%*lant t4een *inou *ro)e e to e9*and the )anal- avoidde)o%*, la%i

1 teobiologyNo4 ne4 *rodu)t to enhan)e bony +u ion t4een vertebral bodie

0 ing )adaver bone, de%inerali$ed bone %atri9 and bone %arro4a *irate to +ill in bony de+e)t and %a"e olid bony +u ionS)a++old the de+e)t 4hile bone gro4 over year1 teo*oro i )an )au e evere ba)" *ain and )an u e )e%ent to hel*

and regain heightArea o+ radi) *ain

ary 4ith *re ure and area

neuro5degen-3

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