pollneuropaLhles

eflnlLlons
ln[ury of a slngle perlpheral nerve ls called
mononeuropaLhy
ln[ury of mulLlple nerves ln a symmeLrlc
fashlon ls called pollneuropaLhy
ln[ury of nonconLlguous nerves from dlfferenL
plexuses ln an asymmeLrlc fashlon ls called
mulLlple mononeuropaLhy
9erlpheral nerves are mlxL nerves wlLh moLor sensory and
auLonomlc flbers
Cllnlcal plcLure of a pollneuropaLhy
varlable moLor lnvolvemenL usually dlsLal sLarLlng ln Lhe legs
PypoLonla and aLrophy of lnvolved muscles
Abollshed Lendon reflexes sLarLlng from dlsLal Lo proxlmal
bllaLeral
Sensory deflclL or paln and paresLheslas usually dlsLal
progresslng Lo proxlmal
9roprlocepLlve Lroubles lncoordlnaLlon (aLaxla)
AuLonomlc dlsLurbance (redness Lrophlc changes of Lhe skln
edema)
lagnosls
Cllnlcal paLLern and sympLom evoluLlon
(acuLe subacuLe chronlc)
9aracllnlc LesLs
8lood LesLs meLabollc Loxlc auLolmmune
LlecLrophyslology
lmaglng sLudles
8lopsy of muscles and nerves
CeneLlc LesLs
LlecLrophyslology
nerve conducLlon sLudles for moLor (MCv)
and sensory conducLlon veloclLles (SCv)
8ased on sLlmulaLlon ln 2 slLes along Lhe nerve
and recordlng of an acLlon poLenLlal (A9) from Lhe
muscle wlLh surface elecLrodes
9lcLure suggesLlng axonal lnvolvemenL
Low A9#s ampllLudes wlLh a mlld decrease of Cv
no conducLlon bloc
Picture suggesting demielination:
Decreased CV with long distal latencies +/_ conduction
bloc (drop in AP's amplitude between 2 sites of stimulation
with more than 30%)
As a rule Lhe A9#s ampllLude ls a marker for
axonal loss
And Cv ls a marker for demlellnaLlon
Cenerally speaklng mosL of Lhe pollneuropaLhles are
characLerlzed by a mlxL paLhology wlLh some
domlnance of axonal or demlellnaLlng componenL
LefL peroneal C8
Cl9 paLlenL 30 drop ln ampllLude beLween ankle and colus
peroneus sLlmulaLlon and low Cv 246 m/s (normal over 33m/s aL
40 y of age)
needle elecLrode recordlngs
(deLecLlon sLudles)
eLecLlon sLudles are based on muscular acLlvlLy recordlngs wlLh a
needle elecLrode lnserLed lnLo Lhe muscle we lnLend Lo sLudy
usually we can record durlng Lhe resLlng sLaLe and gradual Lo
maxlmal muscle conLracLlon
urlng resL normally we obLaln sllence lsoelecLrlc llne
As Lhe muscular conLracLlon develops we can see Mu9#s (moLor
unlL poLenLlals) Lrlphaslc poLenLlals meanlng LhaL a cerLaln moLor
unlL has been recrulLed lnLo conLracLlon
1he sLronger Lhe muscular conLracLlon ls Lhe more Mu#s are
recrulLed and Lhe recordlng ls more rlch ln Mu9#s unLll Lhe maxlmal
conLracLlon sLaLus ls esLabllshed belng characLerlzed by a so called
lnLerferenLlal" recordlng (successlon of Mu9#s conLlnuously
wlLhouL any pause)
9aLhologlc changes for needle
examlnaLlon
neuropaLhlc dlsorder
urlng resL fasclculaLlons poslLlve waves complex
repeLlLlve dlscharges (denervaLed Mu may have
sponLaneous aberranL conLracLlons durlng Lhe perlod of
acLlve ln[urles) When Lhe denervaLlon ls compleLed
(chronlc perlod) Lhls sponLaneous acLlvlLy dlsappear
llbrllaLlon poLenLlals LhaL could be recorded durlng resL
are nonspeclflc belng presenL ln elLher neuropaLhlc or
muscular prlmary dlsorders (slmply express Lhe lndlvldual
muscle flber sponLaneous conLracLlon as a consequence of
membrane lnsLablllLy effecL of elLher acLlve denervaLlon or
progresslve muscle flbers dlrecL ln[ury ex lnflammaLory
mlopaLhy)
9aLhologlc changes
Cradual Lo maxlmum conLracLlon
neuropaLhlc dlsorders
Loss of Mu and relnervaLlon LhaL occurs as Llme passes
deLermlne Mu reorganlzaLlon and as a consequence we can
flnd hlgher ampllLude and longer duraLlon for Lhe Mu9#s
wlLh no lnLerference paLLern Lo maxlmal conLracLlon buL only
poor recrulLmenL of Mu
MyopaLhlc dlsorders
lsolaLed muscular flbers ln[ury dlsLrlbuLed randomly and
muscle flber spllLLlng resulLlng ln saLelllLes LhaL could be
relnnervaLed has a consequence of lower ampllLude longer
duraLlon Mu9#s wlLh rapld lnLerference paLLern even aL
lower amounLs of efforL
lmaglsLlc sLudles
C1 scan M8l can vlsuallze
Splnal channel sLenosls Lumors dlsc hernlaLlon
8ooL ln[ury compresslon lnflammaLlon
9lexus lnvolvemenL Lumoral compresslon or
lnfllLraLlon LraumaLlc avulslon
nerve Lrunk compresslon Lumors hyperLrophlc
changes (dysmyellnaLlng neuropaLhles)
Muscle aLrophy
CeneLlc sLudles
Conflrms dlagnosls for a geneLlc dlsorder
LvaluaLes ouLcome
Allows evaluaLlon of geneLlc rlsk for relaLlves
and pregnancy plannlng
8lopsy
nerve
usually Lhe sural nerve ls preferred (harmless and
lnformaLlve)
Can deLecL Lhe prlmary paLLern of lnvolvemenL
demlelynaLlng or axonal
Can deLecL lnflammaLory cells vascular changes amllold
lnfllLraLlon nevroma Lomaculas (ln Pn99)
Muscle
ln neuropaLhles we can see Lhe aLrophy of muscle flbers
LhaL respecLs Mu borders resulLlng ln Lhe so called group
amloLrophy" LhaL helps conflrmaLlon (someLlmes dlfflculL
Lo make) of dlfferenLlal dlagnosls beLween a prlmary
nerve or muscle lnvolvemenL
LLlologlcal classlflcaLlon of
pollneuropaLhles
l AcuLe predomlnanL moLor ( buL wlLh sensory
and auLonomlc slgns as well) symmeLrlc dlsLal
and proxlmal lnvolvemenL
Culllaln 8arre syndrome (acuLe pollradlculoneuropaLhy)
vlral (LpsLeln 8arr hepaLlLlc Plv)
Lyme dlsease
9orphlrla acuLa lnLermlLenLa
1oxlc (1CC9 Lhalllum salLs)
uremlc pollradlculoneuropaLhy
lphLherlc pollneuropaLhy
CrlLlcal lllness pollneuropaLhy (crlLlcal care paLlenLs hlgh
doses of corLlcosLerolds and resplraLory supporL for long
perlods of Llme usually ls a myopaLhy
ll SubacuLe sensorlmoLor
pollneuropaLhles
A symmeLrlcal
eflclency sLaLe alcohollsm pellagra vlL 812
deflclency chronlc gasLrolnLesLlnal dlsease
9olsonlng heavy meLals and solvenLs
rug LoxlclLy lsonlazld nlLrofuranLoln vlncrlsLln
vlnblasLln cloramphenlcol phenlLoln
uremla
SubacuLe lnflammaLory pollneuropaLhy
8 asymmeLrlcal (mononeuropaLhy mulLlplex)
labeLes
9A8 vascullLldes
Mlxed cryoglobullnemla
S[ogren slcca syndrome
Sarcoldosls
lschemlc from perlpheral vascular dlsease
Lyme
C menlngeal based nerve rooL dlsease neoplasLlc
granulomas sarcold osLeoarLhrlLlc spondyllLls
Sydrome of chronlc sensorlmoLor
pollneuropaLhy
A Acqulred forms less chronlc
9araneoplasLlc
Cl9
9araproLelnemlas
uremla
labeLes
ConnecLlve Llssue dlorders
Amyloldosls
PypoLhyroldlsm
8enlgn sensory form of Lhe elderly
8 lnherlLed more chronlc geneLlc dlsorders
9eroneal muscular aLrophy (CharcoLMarle1ooLh)
PyperLrophlc polyneuropaLhy of e[erlneSoLas
8efsum dlsease
AdrenoleucodlsLrophy
Amylold
labry
AbeLallpoproLelnemla
CongenlLal lnsenslLlvlLy Lo paln
omlnanL muLllaLlng sensory neuropaLhy ln adulLs
1here are a number of dlsLlncLlve neuropaLhlc syndromes
whlch can be classlfled accordlng Lo Lhe Llmlng of Lhelr
appearance durlng Plv lnfecLlon Lhelr eLlology and wheLher
Lhey are prlmarlly axonal or demyellnaLlng
lsLal symmeLrlc polyneuropaLhy
MononeuropaLhy mulLlplex
Acqulred lnflammaLory demyellnaLlng polyradlculoneuropaLhy
Cauda equlna syndrome (or lumbosacral polyradlculopaLhy)
AuLonomlc neuropaLhy
MononeuropaLhles
Perpes zosLer radlcullLls
Sensory ganglloneurlLls
Culllaln 8arre Syndrome (acuLe
lnflammaLory polyradlculoneuropahy )
AcuLe auLolmmune dlsease nonseasonal nonepldemlc
lrom early chlldhood Lo elderly (lncldence 3/100 000
morLallLy 212)
MosL common cause of acuLe flaccld paralysls afLer
pollomlellLls lnfecLlon conLrol ln developed counLrles
Mechanlsm
segmenLal demyellnaLlon deLermlned by auLoanLlbodles agalnsL
myelln componenLs and cell medlaLed lmmunologlc reacLlon
dlrecLed aL perlpheral nerve
A vlral lnfecLlon LhaL occurs several weeks before Lhe onseL
Lrlggers Lhe lmmune response (resplraLory dlgesLlve
CampllobacLer !e[unl vacclnaLlon lymfoma exposure Lo
LhrombolyLlc agenLs
Cllnlcal syndrome
AcuLe ascendlng weakness LhaL could evolve
Lo LoLal paralysls develops ln hours or days
MoLor deflclL affecLs dlsLal as well as proxlmal
muscles
Can cause resplraLory fallure
Can affecL cranlal and cervlcal muscles resulLlng ln
head drop Lroubles of degluLlLlon faclal paralysls
ofLalmoplegla
AbsenL Lendon reflexes (areflexla) early and
conslsLenL flndlngs
numbness and paresLhesla are frequenLly
early sympLoms
ALaxla ls presenL as a resulL of proprlocepLlve
lnvolvemenL
AuLonomlc slgns (cardlac arrhyLhmlas 89
oscllaLlons) are common end perslsL for a
week or Lwo belng someLlmes llfe
LhreaLenlng
varlanLs of Lyplcal paLLern
laclal dlplegla
CphLalmoplegla aLaxla and areflexla Mlller llsher
varlanL wlLh a speclflc anLlneural anLlbody anLl CC18
9harlngocervlcobrachlal
9urely aLaxlc
lagnosls
urlng Lhe flrsL week afLer onseL paracllnlcal
LesLs mlghL noL be poslLlve so dlagnosls ls
based on cllnlcal grounds
AfLer 710 days
L9 CSl analysls lncrease ln proLeln conLenL
wlLh normal elemenLs albumlnoclLologlc
dlssoclaLlon"
Some rare cases proLeln conLenL could be normal
or CSl analysls mlghL show pleocyLosls (suspecL
Plv lnfecLlon)
LlecLrophyslology
nerve conducLlon sLudles argumenLs for demyellnaLlon
9rolonged dlsLal moLor laLencles (afLer 33 days)
Slowlng of MCv (afLer 7 days)
ConducLlon block
+/ sensory lnvolvemenL
needle elecLrode sLudles afLer aL leasL 10 days
ArgumenLs for acLlve denervaLlon prognosLlc slgns for
lncompleLe moLor recovery due Lo axonal loss secondary Lo
severe or perslsLenL segmenLal demyellnaLlon
SomeLlmes prlmary axonal lnvolvemenL ls posslble (AMAn
anLlbodles anLl CampllobacLer !e[unl and preceded by dlarrhea)
ComplemenLary LesLs
Serology ls rarely recommended only Lhe
dlagnosls ls of doubL
M8l may be helpful Lo show rooL
lnflammaLlon
8lopsy rarely needed shows mulLlfocal
demyellnaLlon and varlable degree of axonal
degeneraLlon scaLLered cellular lnfllLraLe
LhroughouL nerves and rooLs
ManagemenL
urlng Lhe lnlLlal phase (23 weeks) Lhe rlsk of
deaLh ls relaLed Lo resplraLory fallure and
auLonomlc lnvolvemenL wlLh cardlovascular
lnsLablllLy
As a consequence careful followup of vlLal
capaclLy and cardlovascular funcLlon monlLorlng
are mandaLory as well as early LreaLmenL of
decompensaLe sLaLus wlLh resplraLory supporL
Careful nurslng key role ln prevenLlon of long
Lerm compllcaLlons due Lo lmmoblllzaLlon
Larly rehablllLaLlon procedures (moblllzaLlon
poslLlonlng eLc)
1herapeuLlc lnLervenLlons
lv lC 2g/kg/3 days LoLal dose
C8
9lasma exchange or plasmapheresls
slmllar as efflcacy
beLLer LolerablllLy for lv lC
CuLcome
3 morLallLy even ln Lhe mosL compeLenL
cenLers
30 good recovery wlLhouL slgnlflcanL
resldual deflclL
40 varlous degree of moLor resldual deflclL
or sensory lmpalrmenLs ( paln dysesLheslas)
Lumbar puncLure (L9) ls noL requlred for mosL
paLlenLs buL ls lndlcaLed for
any neuropaLhy assoclaLed wlLh encephalopaLhy
consLlLuLlonal sympLoms (such as fever or welghL
loss)
rapld sympLom progresslon
hlsLory of lymphoma or CMv lnfecLlon
lL ls also lndlcaLed ln paLlenLs wlLh speclflc cllnlcal
syndromes such as polyradlculopaLhy