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Peripheral Neuropathies
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Peripheral Neuropathies
• Common disorder
• Prevalence of non-traumatic peripheral
neuropathies• 2.4% in general population
• 15% over the age of 40
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Peripheral Neuropathies
• Motor neuron disorders
• Radiculopathies
• Plexopathies
• Single and Multiple Mononeuropathies
• Symmetric Polyneuropathies
• Motor europathies• Sensor! "anglionopathies
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Symptoms
• Positive and negative phenomena
• Sensor! s!mptoms earl!
• #!picall! s!mmetric in onset• $eaness later
• &istal s!mptoms predominant
• $orse at night• 'ther S!mptoms-(m)alance* +atigue* +alls
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Early Signs
• &istal sensor! loss,• arge +i)ers
• loss of vi)ration )efore proprioception
• decreased anle reflexes• Small fi)ers
• oss of pinpric and temperature
• Stocing-glove distri)ution
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Early Signs
• &istal eaness
– #oe extensors
– +oot dorsiflexors
– +inger extensors
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Common Causes
• &ia)etes
• epros!
• /itamin 012 deficienc!
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Approach
• cute vs. chronic onset
– cute fulminant and live threatening
• xonal vs. dem!elinating – &em!elinating forms respond ell toimmunotherap!
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Acute Polyneuropathies
• "uillain-0arre S!ndrome or cute(nflammator! &em!elinating
Pol!radiculoneuropath!
• Porph!ria
• #oxic 3arsenic and thallium
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Chronic Polyneuropathies
• (nherited 3CM#* 5MS* 5PP
– +amil! 5istor!
– +oot &eformities
– +oot 6lcers
• c7uired
– 8M((9
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Acquire Polyneuropathy
8M((9
• Meta)olic
• (mmune• eoplastic
• (nfectious
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!eta"olic Causes
• &ia)etes
• 6remia
• lcohol a)use• 5!poth!roid
• /itamin 01 or 012 deficienc!
• /itamin 0: toxicit!• Medications;chemotherap!
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#mmune Causes
• /asculitis
• on-vasculitic – C(&P
– MM
– Sarcoid – S
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#nfectious Causes
• epros!
• 5epatitis C
• !me• 5(/
• $est ile
• S!philis• &iptheria
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Neoplastic Causes
• Paraneoplastic3Poems s!ndrome
• Paraproteinemic
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!$S
• !onoclonal gammopath! of unclear significance
• Prevalence,
– >% of persons ?@A !ears
– @% ?BA !ears
• 1% per !ear ris of progression to multiple m!eloma3MM or a related disorder
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Autonomic Symptoms
• ightheadedness or 8diiness9
• 0lurred vision
• &r! e!es* dr! mouth
• Cold feet
• Darl! satiet!* constipation* diarrhea
• 6rinar! retention* incontinence
• Drectile &!sfunction
• 5!pohidrosis
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&ysautonomias
• &ia)etes
• m!loidosis 3ac7uired and inherited
• Paraneoplastic• (nherited 35S
• S
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&ifferential &iagnosis
• Small fi)er neuropath!
• Plantar fasciitis
• 'steoarthritis• /ascular insufficienc!
• Cervical m!elopath!
• um)osacral radiculopath!
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Electromyography 'E!$(
• #o part test,
• erve conduction studies
• eedle electrom!ograph!
• Dsta)lish diagnosis of pol!neuropath!
• &istinguish dem!elinating from axonal
• &ifferentiate radiculopath!* plexopath!
• ormal in small fi)er and autonomic neuropath!
P EM YNDR ME
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P EM YNDR MEPOEMS syndrome, also known as
osteosclerotic myeloma, Takatsuki
syndrome, and Crow-Fukase syndrome, is a rare paraneoplastic syndrome
resulting from an underlying plasma
cell disorder T!ere acronym POEMSrefers to se"eral, #ut not all, of t!efeatures of t!e syndrome$polyradiculoneuropat!y,
organomegaly, endocrinopat!y,monoclonal plasma cell disorder, and
skin c!anges
G l
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Addition of newfeatures over time-Fre%uency$ &'(-More common wit! urine
M-proteinEventual severe
E!idemiolo"yMale +/0( to *0( 2 Female
#eo"ra!y5ig! incidence in 6apanese 78sian malesFre%uency in 6apan$ 0) per
&00,000
Onset a"e$ Mean .&years9 younger t!an myeloma
Pro"ression:sual$ O"er mont!s to yearsSome patients$ 8cute or
Su#acute
Generaly
T5E P8T5O;E
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T5E P8T5O;Eplatelets, is
known to target endot!elial cells, induce arapid and re"ersi#le increase in "ascular
permea#ility, and #e important inangiogenesis ?ot! 4@-&A and 4@-/ !a"e #een
s!own to stimulate =E;F production
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D$A#NOS%$C CR$E%ER$A
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$n te Mayo Clini& series of '' !atients( tefollowin" features were !resent )
BPolyneuropat!y &00 percentBMonoclonal plasma cell disorder &00 percentB4ncreased cere#rospinal Duid protein +2.0 mg>d@ &00percentBOsteosclerotic #one lesions * percentBSkin c!anges /' percentBEndocrinopat!y /* percentBOrganomegaly +!epatomegaly, splenomegaly,lymp!adenopat!y .0 percent
Beig!t loss +2&0 pounds )* percent
Peri!eral neuro!aty
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Peri!eral neuro!atySym!toms usually #egin in t!e feet and consist of tingling, parest!esias, andfeelings of coldness Motor in"ol"ement follows t!e sensory symptoms ?ot!
are distal, symmetric, and progressi"e, wit! a gradual proimal spread Se"ereweakness occurs in more t!an !alf of patients and results in an ina#ility to
clim# stairs, rise from a c!air, or grip o#Jects Krmly wit! t!e !ands, consistent
wit! a predominantly motor c!ronic inDammatory demyelinatingpolyneuropat!y T!e course is progressi"e and patients may #e conKned to a
w!eelc!air 8utonomic symptoms are not a feature
Pysi&al e*amination re"eals a symmetric sensorimotor neuropat!yin"ol"ing t!e etremities Muscle weakness is more marked t!an sensory loss Touc!, pressure, "i#ratory, and Joint position senses are often in"ol"ed @ess
fre%uently, loss of temperature discrimination and nociception occur Cranialner"es are not aHected
Ele&tromyo"ra!i& studies s!ow slowing of ner"e conduction, prolongeddistal latencies, and se"ere attenuation of compound muscle action
potentials Conduction #lock is rarely found , #ut slowing of motor conduction
is proportionately greater t!an t!e reduction in t!e compound muscle actionpotential amplitude 1istal K#rillation potentials are found on needleelectromyograp!y
+io!sy of te sural nerve usually s!ows #ot! aonal degeneration anddemyelination9 se"ere endoneurial edema may also #e seen, along wit! !ig!epression of =E;F in "asa ner"orum and some non-myelin-forming Sc!wann
cells Endoneurial deposits of immunoglo#ulins of t!e same type as in t!eserum !a"e #een reported in t!ree of four patients wit! POEMS syndrome
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14FFE3E
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Different neurological and physiological profiles in
POEMS syndrome and chronic inflammatory
demyelinating polyneuropathy
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%reatment a!!roa&
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CasesHistory physical exam
workuptreatment&management
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istory 8 ..-year-old , pre"iously !ealt!y patient
presented to a neurology ward #ecause of a &*-week !istory of progressi"e gait diculties
associated wit! distal parest!esias in !er upper andlower lim#s t!at gradually conKned !er to a
w!eelc!air Pysi&al e*amination found four-lim#areDeia, se"ere symmetrical motor deKcits of t!efour lim#s +predominantly in t!e legs, sensory
impairment of t!e four lim#s and disa#ility of t!earms and legs +functional disa#ility scale score out
of &0& T!e patient !ad no dermatological orendocrinological manifestations, organomegaly,papilledema, etra"ascular "olume o"erload, or
pulmonary manifestations
Neuro!ysiolo"i&al e*amination &' weeks after
http://www.nature.com/nrneurol/journal/v4/n12/full/ncpneuro0942.htmlhttp://www.nature.com/nrneurol/journal/v4/n12/full/ncpneuro0942.html
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! y "presentation re"ealed a frank decrease in t!e amplitude ofcompound muscle action potentials +CM8Ps and sensory
ner"e action potentials +Sl
and !ypoal#uminemia +G** g>l9 normal )..0 g>l$ntravenous immuno"lobulin 0$1$"2 34 "5day for 6 days7
was "iven for a su!!osed &roni& in8ammatory
demyelinatin" neuro!aty( witout any &lini&al bene9t. 3esults of a myelogram and #one-marrow #iopsy +includingimmuno!istoc!emical staining were normal T!oracic and
a#dominal CT scans demonstrated t!ree mainly lytic lesionswit! perip!eral sclerosis of t!e iliac #ones +Figure &
5istological eamination of CT-guided #one-marrow #iopsy
samples from t!e iliac #one lesions s!owed interstitial
C% s&an of te !elvis of
http://www.nature.com/nrneurol/journal/v4/n12/full/ncpneuro0942.htmlhttp://www.nature.com/nrneurol/journal/v4/n12/full/ncpneuro0942.html
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C% s&an of te !elvis oftis !atient
%e s&an sows a lyti&a&etabular lesion wit
irre"ular s&leroti&
mar"ins.
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COl engraftment occurred on days &G and G0,respecti"ely
• G' weeks after 85SCT , Clinical status impro"ed progressi"ely, and t!e patient resumedwalking, wit! normaliNation of t!e disa#ility scale score for t!e upper lim#s . weeks after85SCT MaJor impro"ement of neurop!ysiological parameters was also ac!ie"ed at t!at timealong wit! normaliNation of #iological parameters, platelet count and serum =E;F, as well asdisappearance of t!e monoclonal component
http://www.nature.com/nrneurol/journal/v4/n12/full/ncpneuro0942.htmlhttp://www.nature.com/nrneurol/journal/v4/n12/full/ncpneuro0942.html
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Follow-up of a patient with POEMS syndrome from week –9 before autologous
hematopoieti stem-ell transplantation to week !"9 thereafter
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Case G
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EFGHGI JKHL INOI GQQJ -QL TU M34L
FVQW GFONFFL KFKK EGQX OK YFXFJI FVOL
*t! d
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*t! day
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& Predominantly motor primary demyelinating polyneuropat!y wit! secondarylengt! dependent and somemultifocal aonal loss T!is conclusion is #ased on t!e slowed conduction "elocityand conduction #locksidentiKed in ner"es in t!e forearms, on t!e #ackground of relati"ely preser"ed
Songoingdener"ation in t!e etensor digitorum communis on t!e #ackground of relati"elypreser"ed radial S
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)*+,-.)/ +)
• #$%&'&()# *&&+ ', #$,$ %+ #'&.#
• #)&($+&)/'0 12 $(, 3. 4&'. *)35*) #$(0&6$&7 12 #'&.
36$ 8$/&63$#60$#) ' 8:3)' %&);# 81$$5&, *&5+ 8*&)+2 1$$(&6'5& 1$$($' 1$2%7- 256
6. 81$$'%6# *&2+: ', ?&G $/&($+&)&6*
• 1&637$' #)$:*) #7&)*# - 1&>$' POEMS
• *&)6' *&5$3?1$$7$2 :0&6 *5$3 ?' &5: - 6&6$ 1$',#' @')
1$7&)6 PET CT *&)6 ?4)/ 6>, =E;F
• '&0$( #:67> '&'>* A,)# '&0$(# *$7>&* 8#7$65 '&0$(# 1$$$
*$)+2 12+ .) *'*,#& $)($
• *$6&63 '56) 6=3
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Beuropathy features in patients with POEMS syndrome who were treated with
autologous hematopoieti stem-ell transplantation
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2eferences
• &ia)etes Statistics. http,;;.dia)etes.org;dia)etes-)asics;dia)etes-statistics;• 0ril / et al. Dvidence-)ased guideline, #reatment of painful dia)etic
neuropath!. eurolog!Z Pu)lished online )efore print pril 11* 2A11Z &'(1A.1212;$.A)A1>e>1[21::e)e
• 0ril /. #reatments for dia)etic neuropath!. \PS 2A12,1B3s2Z22]2B.• eishear ^ et al. Relationship 0eteen /itamin 012 and Sensor! and Motor
Peripheral erve +unction in 'lder dults. \"S 2A12,:A3:Z 1A@B]1A:>.• Dngland \& et al. Dvaluation of distal s!mmetric pol!neuropath!, the role of
autonomic testing* nerve )iops!* and sin )iops! 3an evidence-)ased revie.Muscle erve 2AA_ Z>_, 1A:]11@.
• Dngland \& et al. Dvaluation of distal s!mmetric pol!neuropath!, the role ofla)orator! and genetic testing 3an evidence-)ased revie. Muscle erve 2AA_
Z>_, 11:]12@.• Ser"ice de
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2eferences
• ^!le R* Ra_3@ZB>A]B4>.
• Mauermann M* 0urns #M. #he evaluation of chronic axonal pol!neuropathies. Semin eurol. 2AA[,2[32,1>>-@1.
• Ramaratnam S. eurologic Manifestations of epros!.http,;;emedicine.medscape.com;article;11:@41_-overvie`a2aa):):
• Rutove S0. 'vervie of pol!neuropath!. http,;;.uptodate.com;contents;overvie-of-pol!neuropath!6pto date