Peripheral Neuropathies (1)Mmmmpo2222445345

8/9/2019 Peripheral Neuropathies (1)Mmmmpo2222445345

1/48

Peripheral Neuropathies


2/48


• Common disorder

• Prevalence of non-traumatic peripheral

neuropathies• 2.4% in general population

• 15% over the age of 40


3/48


• Motor neuron disorders

• Radiculopathies

• Plexopathies

• Single and Multiple Mononeuropathies

• Symmetric Polyneuropathies

• Motor europathies• Sensor! "anglionopathies


4/48

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


5/48

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


6/48

Early Signs

• &istal eaness

– #oe extensors

– +oot dorsiflexors

– +inger extensors


7/48

Common Causes

• &ia)etes

• epros!

• /itamin 012 deficienc!


8/48

Approach

• cute vs. chronic onset

– cute fulminant and live threatening

• xonal vs. dem!elinating – &em!elinating forms respond ell toimmunotherap!


9/48

Acute Polyneuropathies

• "uillain-0arre S!ndrome or cute(nflammator! &em!elinating

Pol!radiculoneuropath!

• Porph!ria

• #oxic 3arsenic and thallium


10/48

Chronic Polyneuropathies

• (nherited 3CM#* 5MS* 5PP

– +amil! 5istor!

– +oot &eformities

– +oot 6lcers

• c7uired

– 8M((9


11/48

Acquire Polyneuropathy

8M((9

• Meta)olic

• (mmune• eoplastic

• (nfectious


12/48

!eta"olic Causes

• &ia)etes

• 6remia

• lcohol a)use• 5!poth!roid

• /itamin 01 or 012 deficienc!

• /itamin 0: toxicit!• Medications;chemotherap!


13/48

#mmune Causes

• /asculitis

• on-vasculitic – C(&P

– MM

– Sarcoid – S


14/48

#nfectious Causes

• epros!

• 5epatitis C

• !me• 5(/

• $est ile

• S!philis• &iptheria


15/48

Neoplastic Causes

• Paraneoplastic3Poems s!ndrome

• Paraproteinemic


16/48

!$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


17/48

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


18/48

&ysautonomias

• &ia)etes

• m!loidosis 3ac7uired and inherited

• Paraneoplastic• (nherited 35S

• S


19/48

&ifferential &iagnosis

• Small fi)er neuropath!

• Plantar fasciitis

• 'steoarthritis• /ascular insufficienc!

• Cervical m!elopath!

• um)osacral radiculopath!


20/48

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


21/48

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


22/48

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


23/48

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


24/48

D$A#NOS%$C CR$E%ER$A


25/48

$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


26/48

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


27/48


28/48

14FFE3E


29/48

Different neurological and physiological profiles in

POEMS syndrome and chronic inflammatory

demyelinating polyneuropathy


30/48


31/48

%reatment a!!roa&


32/48


33/48


34/48

CasesHistory physical exam

workuptreatment&management


35/48

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


36/48

! 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



37/48

C% s&an of te !elvis oftis !atient

%e s&an sows a lyti&a&etabular lesion wit

irre"ular s&leroti&

mar"ins.


38/48

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



39/48

Follow-up of a patient with POEMS syndrome from week –9 before autologous

hematopoieti stem-ell transplantation to week !"9 thereafter


40/48


41/48

Case G


42/48

EFGHGI JKHL INOI GQQJ -QL TU M34L

FVQW GFONFFL KFKK EGQX OK YFXFJI FVOL

*t! d


43/48

*t! day


44/48

& 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


45/48

)*+,-.)/ +)

• #$%&'&()# *&&+ ', #$,$ %+ #'&.#

• #)&($+&)/'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


46/48

Beuropathy features in patients with POEMS syndrome who were treated with

autologous hematopoieti stem-ell transplantation


47/48

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


48/48

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

Date post:	01-Jun-2018
Category:	Documents
Upload:	mohamed-azaiza
View:	223 times
Download:	0 times

Peripheral Neuropathies (1)Mmmmpo2222445345

Documents