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Planul prezentarii
Poliradiculonevrita acuta (SGB)
Cadru nozologic
Etiologie
Patogenie
Morfopatologie
Simptomatologie
Forme clinice clasice si subtipuri electroimunochimice actuale
Explorari paraclinice
Diagnostic pozitiv si diferential
Evolutie si prognostic
Tratament
Poliradiculonevrite cronice
Cadru nozologic
Criterii de ncadrare
Limitele actuale ale conceptului de PRN cronica
Tratament
Concluzii
Bibliografie
MORFOPATOLOGIE
Topografia leziunii: rdcini, plexuri, trunchiuri nervoase, nervi
cranieni, ganglioni spinali i simpatici.
Tipul leziunii:
Inflamaie infiltrat interstiial predominent limfocitar.
Demielinizare n focare diseminate.
SIMPTOMATOLOGIE
- debut la 2-4 saptamani dupa un episod infectios
3 faze:
de extensie a paraliziei (4 sptmni),
de platou (2-4 sptmni),
de recuperare.
Tulburri senzitive subiective / obiective
Tulburri motorii
Paralizii de nervi cranieni: VII, IX, X, VI, III, IV
Tulburri vegetative (Sd Schwartz-Bartter - secreia ADH)
Rar semne centrale (Babinski, edem papilar, confuzie)
Tulburari de sensibilitate
- subiective = parestezii distale (MI>MS)
rahialgii/lombalgii iradiate in MI
- obiective = minime
Tulburari motorii
- debut la MI
- extindere ascendenta n ore/zile -> afectarea MS, nervilor cranieni
- cazuri grave afectarea musculaturii respiratorii
Examen clinic
- tetrapareza/tetraplegie simetrica
- deficit proximal+distal
- minime tulburari de sensibilitate (contrasteaza cu intensitatea tulburarilor subiective de
sensibilitate) + manevre de elongaie pozitive
- semne de disfunctie autonoma (50%)
aritmii cardiace (bradicardie, asitola, tahicardie sinusala, tahiaritmii atriale/ventriculare)
hipotensiune ortostatica
HTA tranzitorie/persistenta
ileus paralitic
disfunctie vezicala
transpiratii profuze
Forme clinice
Simptomatice:
Forma spinal (cu sindrom senzitivo-motor i disociaie albuminocitologic)
Forma mezocefalic (cu afectarea nervilor cranieni)
Forma mixt mezocefalo-spinal
Forma de mielopoliradiculonevrit
Encefalo-mielopoliradiculonevrit (realizate de extinderea leziunii la
mduv i encefal)
Forma pseudo-miopatic (cu tulburri de mers i static,
pseudomiopatice prin localizarea proximal a deficitului motor)
Forme de poliradiculonevrit acut inflamatorie fr disociaie
albumino-citologic;
Sindromul Miller Fisher (asociaz paralizii oculomotorii, ataxie i
areflexie osteotendinoas fr deficit motor la cele patru membre dar
cu disociaie albumino-citologic n LCR i evoluie benign)
Sindromul Young i Adams (cu disautonomie important)
Explorari paraclinice
Examene electrofiziologice cel mai sensibil/specific test ,
demonstreaz demielinizarea multifocal evolutiv
VCN
amplitudinii CAMP (compound action muscle potential),
reducerea raportului rspunsurilor motorii obinute la
stimularea proximal i distal = bloc de conducere n absena
unei pierderi axonale
alterarea morfologiei CAMP (aspectul normal bifazic -> aspect
polifazic) = semn precoce
duratei CAMP
alungirea latenelor distale (atingerea fibrelor rapide)
dispersie temporal anormal
anomalii ale reflexului H
alungirea latenelor undelor tardive F (demielinizare proximal)
Diagnosticul diferenial
1. Prima etapa -> eliminarea afeciunilor cu instalare acut ce
pot mima o PRN:
Poliomielita acut
Mielitele i mielopatiile acute necrozante
Meningoradiculita Lyme
Polineuropatiile acute
Polineuropatii multifocale cu bloc de conducie persistent
Distrofii musculare progresive / cortizonice
Diskaliemii (caracter familial i periodic)
Polimiozite,
Miastenie
Prognostic
- recuperare complet (majoritatea cazurilor)
- 5-10% sechele motorii/senzitive
- 3-8% deces prin complicatii:
detres respiratorie
sepsis
embolie pulmonar
stop cardiac
- elemente de prognostic nefavorabil:
vrsta naintat
evoluia rapid, sever
ventilaie mecanic prelungit (>1 lun)
areflexia completa precoce
anomalii severe persistente la examenele electrofiziologice (scderea
amplitudinii CAMP)
absena trat. cu IgG, plasmaferez
prodrom de diaree
2. Tratament patogenic
Imunglobuline i.v. (0,4 g/kg.corp/zi 5 zile) cel mai eficient n formele de SGB cu IgG vs GM1, GM1b,
GalNAc-GD1a gangliozide
ameliorarea superioar/mai rapid a deficitului motor
complicaii mai reduse
Combinarea celor 2 terapii NU este superioar
Corticoterapie (Metilprednisolon 0,5 g/zi i. v. , 5 zile , PDN 60mg/zi 8 zile cu progresiv) nu s-a
dovedit benefic
3. Tratament simptomatic
antialgice,
corectarea ileusului paralitic etc.
4. Tratament recuperator - precoce
POLIRADICULONEVRITELE CRONICE
Chronic inflammatory demyelinating polyneuropathy (CIDP) =
polyradiculonvrites inflammatoires dmylinisantes chroniques
Reunesc PRN recidivante i cele cu evoluie prelungit.
Criterii clinice:
deficit motor predominent proximal, mai ales la MI
deficit senzitiv pentru toate modalitatile, predominent
proprioceptiv, distal, neinvalidant
constant areflexie osteo-tendinoas,
amiotrofii,
tulburri respiratorii, dureri mai rar,
disautonomie excepional (tulburari mictionale).
Limitele conceptului
Electrofiziologice
- sensibilitate mica a criteriilor clasice
- dificultatea utilizrii n practic, mai ales la debutul afeciunii
- necesitatea dezvoltrii de noi tehnici
Terapeutice
- eficacitate 3 luni > limitarea duratei de tratament
- eficacitate restrnsa la doar cteva tratamente
- cunoastere limitata a eficientei noilor agenti imunosupresori
(mycofenolat, rituximab, etanercept, ciclosporina, interfron beta)
Histologice
- modificarile clasice (demielinizare segmentara recenta/veche,
demielinizare-remielinizare
cronica,
proliferarea
celule lor
Schwann in bulb de ceapa, edem endoneural, infiltrate celulare
inflamatorii perivasculare) nu se regasesc in toate cazurile
- propus studiul n ME
CIDP
Clinical Features
Electrophysiology
Motor + Sensory
change
Slow NCV
Conduction Block
Distal Latency: Long
Slow F-waves
Antibody
M-Protein*
Targets
-tubulin
Heparan
sulfate
15%
Class: IgM or
IgG
Treatment
T-cell
immunosuppression
Prednisone
Cyclosporine A
Methotrexate
HIG
Plasma Exchange
Frequency: 20%
Multifocal
CIDP
Also see:
CIDP varia
nts
MMN
Chronic
Motor > Sensory
Weakness:
Distal >
Proximal
Asymmetric
Arms > Legs
Onset: 15 to 75
yrs
Motor only
Distal >
Proximal
Arms > Legs
Asymmetric
Onset: 25 to 60
yrs
Slowly
progressive
Motor + Sensory
change
Slow NCV
Conduction Block
Distal Latency: Long
Slow F-waves
Motor only
Conduction Block
Axonal Loss
EMG: No paraspinous
denervation
T-cell
immunosuppression
Prednisone
HIG
20%
HIG
B-cell
immunosuppression
Plasma Exchange +
Cyclophosphamide
Rituximab
Targets
Co-GM1 or NP-9
Class: IgM
Frequency: 80%
Anti-MAG
GALOP
Gait Disorder
Sensory > Motor
Distal; Symmetric
Onset: > 50 yrs
Motor + Sensory
Distal Latency:
Long
Slow NCV
No conduction
block
Axonal Loss
Motor + Sensory
Distal Latency:
Long
Slow NCV
No conduction
block
Target: MAG
Class: IgM
85%
Frequency: 100%
Target
Sulfatide
in lipid membr
ane
B-cell
immunosuppression
Plasma Exchange +
Cyclophosphamide
Rituximab
? Fludarabine
80%
HIG
Plasma Exchange +
Cyclophosphamide
90%
HIG
Plasma Exchange +
Cyclophosphamide
Class: IgM
Anti-Sulfatide
Anti-GM2 &
GalNAc-GD1a
Slowly
progressive
Sensory > Motor
Distal; Symmetric
Onset: > 45 yrs
Distal Latency:
Long
Slow NCV
Axonal Loss
Slow NCV
Target
Sulfatide
Class: IgM
Targets
GM2
GalNAc-GD1a
Class: IgM
Common
HIG
Tratamentul patogenic
de elecie corticoterapia = prednison 60-100mg/zi 3-6 luni,
scadere progresiva a dozelor, doza minima de intretinere
eventual imunosupresoare (ciclosporina A, azatioprina)
imunglobuline intravenos (eficacitate comparabil cu
prednison) 2g/kg 3-5 zile, repetare dupa 3-8 saptamani
plasmaferez
Concluzii
Capitolul de patologie reprezentat de
poliradiculonevrite a suferit transformari
importante sub aspect nozologic .
Cercetarile imunologice castiga tot mai
mult teren in acest domeniu.
Pentru ca terapia sa fie eficienta, este
necesara cunoasterea aprofundata a
caracteristicilor
histoimunologice
ale
fiecarei forme de poliradiculonevrita.
BIBLIOGRAFIE
Bosch EP, Smith BE. Disoders of peripheral nerves. In: Neurology in Clinical Practice.
5th ed. Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds. Philadelphia, PA:
Butterhworth-Heinemann Elsevier. 2007:2336-2345.
Guillain-Barre
syndrome.
EBSCO
Dynamed
website.
Available
at:
http://www.ebscohost.com/dynamed/what.php . Accessed December 3, 2006.
Guillain-Barre syndrome fact sheet. National Institute of Neurological Disorders and
Stroke website. Available at: http://www.ninds.nih.gov/disorders/gbs/detail_gbs.htm .
Accessed December 3, 2006.
C. Caudie, C. Vial, P. Petiot, J. Bancel - Dtection des autoanticorps antigangliosides par
immunodot-blot : intrt dans le diagnostic des neuropathies priphriques autoimmunitaires, Annales de Biologie Clinique. Volume 57, Numro 5, 579-88, Septembre Octobre 1999
Comi, G., A. Quattrini, R. Fazio, and L. Roveri. "Immunoglobulins in Chronic
Inflammatory Demyelinating Polyneuropathy." Neurological Science (October 2003):
S246S250.
Fee, D. B., and J. O. Flemming. "Resolution of Chronic Inflammatory Demyelinating
Polyneuropathy-associated Central Nervous System Lesions after Treatment with
Intravenous Immunoglobulin." Journal of the Peripheral Nervous System (September
2003): 155158.
Katz, J. S., and D. S. Saperstein. "Chronic Inflammatory Demyelinating Polyneuropathy."
Current Treatment Options in Neurology (September 2003): 357364.
Adams and Victorss Principles of Neurology, 8th Ed. 2005, 1121-7