guillain-barre syndrome and myasthenia gravis [email protected] university of versailles...
TRANSCRIPT
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GUILLAIN-BARRE SYNDROMEand
MYASTHENIA GRAVIS
University of VersaillesRaymond Poincaré Teaching Hospital
Garches - France
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STATEMENTS
1. Guillain-Barré Syndrome (GBS) is the most frequent cause of acute paralysis.
2. GBS is secondary to an acute immune-mediated polyneuropathy
3. GBS can be differentiated in various clinical and electrophysiological sub-types
4. Its gravity includes respiratory failure, cardio-vascular autonomic dysfunction, and long-term disability
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PARALYSIE FLASQUE AIGUË
DEFICIT SENSITIVO-MOTEUR
DEFICIT MOTEUR PUR
1. ± IRM
2. LCR
3. VS-CRP-NFS
4. EMG (PN Axonale, PN Démyelinisante)
1. Kaliémie
2. LCR
3. EMG (Myopathie, Myasthénie, Neuropathy)
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Signes Pyramidaux
Niveau sensitif
Sd de la queue de cheval
IRM de la moelle
PARALYSIE AIGUË HYPOREFLEXIQUE SENSITIVO-MOTRICE
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PARALYSIE AIGUË HYPOREFLEXIQUE SENSITIVO-MOTRICE
VS NORMALE VS AUGMENTEE
LCR
NORMAL
TOXIQUE (Thallium, arsenic…)
METABOLIQUE (Gly, Vit …)
VASCULARITES (SLE…)
GBS IDIOPATHIQUE
VASCULARITES (SLE…)
HYPERCEL. MENINGORADICULITES MENINGORADICULITES
HYPERPROT.
GBS IDIOPATHIQUE
CANCER, LYMPHOME, VASCULARITES, DIPHTERIE, VIH.
CANCER, LYMPHOME, VASCULARITES, DIPHTERIE, VIH.
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DEFICIT MOTEUR PUR HYPOREFLEXIQUE
SIGNES K+ LCR EMG
PARALYSIE
PERIODIQUEEXERCICE MYOPATHIE
MYASTHENIEVARIATION
MVT OCUL. JONCTION NM
BOTULISMEINTOX. ALIM.
PUPILLES
JONCTION NM
POLIO
MYELITES
VOYAGE
DIARRHEE
CELL.
ANTERIOR HORN CELLS
PORPHYRIE
NEUROPATHIE
CONFUSION
DOULEUR
POLY NEUROPATHI
E
GBS IDIOPATHIQUE
INFECTION
ASCENDANT
PROTEINE
POLY NEUROPATHI
E
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DEFINITIONCRITERES REQUIS
1. Faiblesse progressive de plus de 2 membres
2. Areflexie
3. Progression en moins de 4 semaines
4. Autre cause exclue
CRITERES UTILES
1. Signes sensitifs
2. Hyperprotéinorachie
3. Bloc de conduction à l’ENMG
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INCIDENCE
1. INCIDENCE: 0.5–2.0 cas/100 000/an2. SEX RATIO (F/M): < 1.03. AGE RATIO (O/Y): > 1.04. EPIDEMIO: PAS DE VARIATION SAISONNIERE5. PRODROMES: 70-90%
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SOUS-TYPES
1. Acute inflammatory demyelinating polyneuropathy (AIDP)
2. Acute motor axonal neuropathy (AMAN)
3. Acute motor and sensory axonal neuropathy (AMSAM)
4. Miller Fisher syndrome (MFS)
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PUZZLEPathogen agent
1. C. jejuni
2. CMV
3. Others
Anti-Ganliosides
1. Ant-GM1
2. Anti-GM2
3. Anti-G1QB
Subtypes
1. AIDP
2. AMAN
3. AMSAM
4. MFS
Clinic
1. Sensorimotor
2. Pure motor
3. Cranial nerves
Severity
1. Aspiration
2. Intubation
3. Dysautonomia
Outcome
1. Recovery
2. Sensory sequellae
3. Motor sequellae
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PATHOPHYSIOLOGY
Hughes and Cornblath - Lancet - 2005
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ANTICIPER LA DETRESSE ANTICIPER LA DETRESSE RESPIRATOIRE AIGUËRESPIRATOIRE AIGUË
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VENTILATION MECANIQUE
1. Requise dans 20 à 30%
2. Délai médian entre admission et VM: 2 j
3. Durée médiane: 21 j
4. 1/3 sevrés de la VM dans les 3 premières semaines
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RISQUES A RETARDER LA VM
N= 87PAVM = 67 (75%)
Orlikowski et al - ICM - 2007
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MECANISMES
Diaphragme
Accessores
Abdominaux
DE
FIC
IT A
SC
EN
DA
NT
CV
Pimax
Pemax
toux
Atelectasie
Atteinte bulbaire
Inhalation
Phrenique
IX-X
Hypoventilation
Hypoxaemia
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AdmissionDébut VM
PREDICTEURS PRECOCES
CRITERES PRECOCES
CRITERES TARDIFS
PREDICTEURS TARDIFS
Arrêt Respiratoire
Réa Salle
VM
Pas de VM
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CRITERES D’INTUBATION
CRITERES MAJEURS1. Signes de détresse respiratoires
2. CV< 15 ml/kg, Pimax ou Pemax < 25 cm H2O
3. PaCO2 > 6,4 kPa
4. PaO2 < 7.5 kPa (FiO2= 0,21)
MINOR CRITERIA1. Toux inefficace2. Troubles sévères de la déglutition3. Atélectasies
Ropper - Neurology – 1985; Wijdicks-Neurology-1998
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PREDICTEURS PRECOCES
n= 196
OR 95% CI p
PROGRESSION < 7 jours
5.0 (1.4 – 5.7) < 0.003
Tête 5.0 (1.9 – 12.5) < 0.0011
CV < 60% 2.86 (2.4 – 10.0) < 0.0001
Sharshar et al - Crit Care Med - 2003
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CAPACITE VITALE
Durand et al – Neurology - 2005
MV
No MV
REPETER LA MESURE DE LA CAPACITE VITALE
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ELECTROPHYSIOLOGIE
• EMNG diaphragmatique– Not helpful (Durand – Neurology – 2005)
• ENMG Standard– Demyeliting form at higher risk (Durand–
Eur J Neurol 2003)
– No risk if no conduction block on common peroneal nerve + VC >80% (Durand – Lancet Neurology – 2006)
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CORTISOLEMIE
Variableno MV
(Group 2)MV > 24h(Group 3)
P*
n 60 17
[Cortisol]T0 (ng/ml) 20.4 ± 9.6 28.5 ± 12.1 0.01
[Cortisol]T60 (ng/ml) 42.4 ± 14.8 53.1 ± 16.8 0.05
Strauss et al – CCM - 2009
[Cortisol]T0 est corrélée avec la survenue de dysautonomie
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PEURN (%)
Marcadet et al - Submitted
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AdmissionOnset MV
EARLY PREDICTORS
Inability to lift head
VC < 60%
+/- High cortisolemia
CPN + VC > 80%
EARLY CRITERIAE
LATE CRITERIAE
LATE PREDICTORS
Fall in VC
Swallowing dysfunction
Respiratory arrest
ICU ward
MV
No VM
VERY EARLY PREDICTORS
Onset-Admission < 7 days
Uncertainty
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ADMISSION EN REA
1. Progression < 7 j2. Impossibilité à relever la tête3. Troubles de la déglutition4. CV < 60% oo 20 ml/Kg
Pimax ou Pemax< 30 to 40 cmH2O5. VC diiminuant de 30%
Pimax or Pemax diminuant de 30%6. Dysautonomie cardio-vasculaires
L’absence de ces signes ne doit pas dispenser d’une surveillance régulière neurologique et respiratoire
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TRACHEOSTOMIE
VM > 21 j1. Patients âgés2. Pathologie respiratoire pré-existante
3. Test pulmonaire dint/d12 ratio < 11. TP score = CV + Pimax + Pemax2. Sensibilité = 70%, specificité: 100%
Lawn and Wijdicks - Muscle Nerve - 1999, 2000
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VENTILATION MECANIQUE
Fourrier et al – Crit Care - 2010
Blocs de conduction moteurs: VM prolongée
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MORBIDITERESPIRATOIRE1. Tracheobronchitis 33 (29%)2. Pneumonia 27 (24%)3. Pneumothorax 6 (4%)
INFECTION1. Bacteremia 21 (15%)2. Catheter 3 (3%)3. Urinary tract 75 (66%)
THROMBOSES1. Deep venous thrombosis 5 (4%)2. Pulmonary embolism 3 (3%)
AUTRES1. Hyponatremie (< 130 mmol/L) 28 (25%)
Henderson et al – Neurology - 2003
n = 114
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FACTEURS DE RISQUE DE MORBIDITE
Characteristiques OR 95% CI p Value
Pathologie sous-jacente 2.8 (0.8 - 9.1) 0.10
Echanges plasmatiques 3.4 (1.1 – 10.5) 0.03
VM prolongée 12.4 (3.7 – 41.3) < 0.0001
Henderson et al - Neurology - 2003
n = 114
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TRAITMENT
1. Traitement symptomatique1. Thrombosis prophylaxis
2. Pain relief
3. Psychological support
4. Autonomic dysfunction: atropine, trandate …
5. Bladder catheter, nutrition, physiotherapy…
6. Intercurrent diseases
7. Non-Invasive ventilation: ?
2. Traitement spécifique1. Plasma exchange (PE)
2. High-dose intravenous immunoglobulin (IvIg)
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ECHANGES PLASMQTIQUES
Plasma exchange vs no treatment: DG at 4 weeks
Hughes et al - Brain - 2007
1. Benefit irrespective of severity2. Tested against placebo3. Tested up to 4 weeks after GBS onset
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IMMUNOGLOBULINES
Hughes et al - Brain - 2007
Plasma exchange vs IvIg: DG at 4 weeks
1. Tested against PE
2. Tested in mild or severe GBS
3. Tested within 2 weeks after GBS onset
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CORTICOSTEROIDES
Hughes et al - Brain - 2007
Corticosteroid vs placebo or no treatment: DG at 4 weeks
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CONTRE-INDICATIONS
EP
1. Infection
2. Hypotension
3. Haemorrhage
4. Clotting deficiency
IvIg1. IgA deficiency2. Allergy3. Renal failure
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ALGORITHMES
Disability
Stage
Marche
(DG < 4)
Allité (DG =4)
VM (DG = 5)
Initial* 2 EP 4 EP or IvIg**
Deterioration + 2 EP Rien
Relapse Rien ou même traitment
Rien ou même traitment
*Aussitôt que le diagnostique est confirmé** 0.4g/kg/ daily for 5 days
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MYASTHENIE
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1. Prévalence 4-6 / 100 000
2. Incidence 2-5/million/an
3. 2 F / 1 H1. F: 30 ans
2. H: 30 et 60 ans
EPIDEMIOLOGIE
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MOTS CLES
1. Pathologie de la jonction neuromusculaire
2. Pathologie auto-immune
3. Pathologie thymique
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1. FATIGUABILITE
2. VARIABILITE
à court (jour) et long terme (mois-année)
3. MOTEUR PERIPHERIQUE PUR
Absence d’aréflexie
Absence de myalgie
Absence d’atrophie
4. MUSCLES SQUELETTIQUES Oculomoteurs Myasthénie oculaireFaciauxOropharyngés Myasthénie bulbaire MyasthénieRespiratoires généraliséeDes membres et axiaux
JONCTION NEUROMUSCULAIRE
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SCORE DE LA FORCE MUSCULAIRE (1)
Membres supérieurs étendus à l’horizontale en antéroposition1 point pour 10 s Max. 15
Min. 0
Membres inférieurs, patient en décubitus dorsal, cuisses fléchies
à 90° sur le bassin, jambes à 90° sur les cuisses1 point pour 5 s Max. 15
Min. 0
Flexion de la tête en décubitus dorsalContre résistance 10
Sans résistance 5
Impossible 0
Passage de la position couchée à la position assisePossible sans l’aide des mains 10
Impossible sans l’aide des mains 0
Oculomotricité extrinsèqueNormal 10
Ptosis isolé 5
Diplopie 0
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SCORE DE LA FORCE MUSCULAIRE (2)
Occlusion palpébraleComplète 10
Signe de Souques 7,5
Incomplète (mais avec recouvrement cornéen) 5
Nulle (sans recouvrement cornéen) 0
MasticationNormale 10
Diminuée 5
Nulle 0
DéglutitionNormale 10
Dysphagie sans fausse route 5
Dysphagie avecfausse route 0
PhonationVoix normale 10
Voix nasonnée 5
Aphonie 0
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ATTEINTE OCULAIRE
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ELECTROPHYSIOLOGIE
Décrément
Stimulations répétées à 3HZ
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PATHOLOGIE AUTO-IMMUNE
1. Auto-Anticorps - Thymus
2. Evolution par poussées
3. Autre(s) pathologie(s) auto-immune(s)
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POUSSEE OU CRISE MYASTHENIQUE
PREMIERE ETAPE1. Vérification des critères diagnostiques
1. Anticorps anti-Rach + 2. Ou EMG + et Test à la Prostigmine +3. Sinon Biopsie musculaire
2. Vérification de l’existence d’un thymome1. Si oui, scanner systématique
3. Vérification d’une pathologie auto-immune1. Dysthyroïdie, etc…
4. Récapitulation anamnestique5. Récapitulation des traitements utilisés
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DEUXIEME ETAPE1. Recherche d’un facteur déclenchant (70%)
1. Infection (30-40%)
2. Intervention chirurgicale3. Grossesse
1. 19% d’aggravation) 2. 1er et 3eme trimestre
4. Traitement contre-indiqué1. A interrompre si possible
5. Surdosage en anticholinestérasique1. 4% des cas de poussée2. Ne pas faire de test à la Prostigmine
6. Institution d’une corticothérapie1. Survenue d’une poussée dans 48% des cas
7. Décroissance du traitement immunosuppresseur
POUSSEE OU CRISE MYASTHENIQUE
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EXACERBATION or CRISIS
Lacomis et al – NeuroCritCare - 2005
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EXACERBATIONS
Lacomis et al – NeuroCritCare - 2005
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POUSSEE OU CRISE MYASTHENIQUE
TROISIEME ETAPE1. Evaluation de la gravité
1. Atteinte oropharyngée
2. Atteinte respiratoire
• Difficilement détectable
• Mesure de la CV indispensable
• La normalité des GDS ne doit pas rassurer
2. Si Aggravation rapide, fluctuation clinique, atteinte axiale, oropharyngée, respiratoire ou CV < 60%
1. Transport médicalisé
2. Hospitalisation en réanimation
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POUSSEE OU CRISE MYASTHENIQUE
QUATRIEME ETAPE
1. Evoquer un diagnostic différentiel1. Autre cause d’insuffisance respiratoire2. Autre pathologies contingentes ou associées
• Dysthyroïdies etc..
3. Effets secondaires des traitements • Diabète, Insuffisance surrénalienne etc…
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CRISE MYASTHENIQUE
1. Incidence1. 16% de la population générale myasthénique
2. 23 et 70% dans les 6 à 36 premiers mois
2. Mortalité1. 6% (40% avant l’assistance respiratoire)
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POUSSEE ou CRISE
FIRST STEP1. Diagnostic criteria for MG
2. Thymoma 1. If yes, thoracic CT scan
3. Other auto-immune disease
4. Course of MG
5. Recapitulation of MG specific treatment
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POUSSEE ou CRISE
FIRST STEP1. Diagnostic criteria for MG
2. Thymoma 1. If yes, thoracic CT scan
3. Other auto-immune disease
4. Course of MG
5. Recapitulation of MG specific treatment
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MORTALITE
Alsheklee et al – Neurology - 2009
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MECHANISMS
Diaphragm
Accessory
Abdominal
AS
CE
ND
ING
PA
RE
SIS
CV
Pimax
Pemax
cough
Atelectasia
Bulbar dysfunction
Aspiration
Phrenic
IX-X
Hypoventilation
Hypoxaemia
Laryngeal spasm
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MYASTHENIEVENTILATION MECANIQUE
• Polypnée - orthopnéee - toux inefficace• CV inférieure à 15 ml/kg (25 %)• Pression statique maximale inspiratoire < 20
cmH2O• Pression statique maximale expiratoire < 40
cmH2O• Ne jamais attendre une hypercapnie
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SEVRAGE
• Onset of weaning procedure– Improvement of muscle strength– VC > 30% of predicted value
– FiO2 < 50% and Peep < 5 cm H2O
• Extubation – VC > 50% and VC at 4h of T-Piece < 20%– No hypercapnia at the end of T pieceT-Piece > 8
hours in a row– Take care of swallowing disturbances
(n° of succion)
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Arch Neurol - 2008
Neurology - 2002
BE CAREFULNeuroCrit Care - 2002
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TREATMENT OF EXACERBATION
Non specific treatment
Triggering factor ? Treatment
overdose Chol I? reduction
Swallowing dysfunction ? NGT
Respiratory failure ? MV
Specific treatment
Improvement
PE or IvIg CS or IS
yes no
CS dose x2 CS: 1 mg/kg I.S. no change + I.S.
Survey – No change in specific treatment
Failure
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BLOOD GASES « FAUX-AMIS »
1. Presence of hypercapnia or hypoxemia indicates a severe respiratory muscle weakness and misgives an impeding respiratory arrest
2. Conversely, absence of blood gases abnormality does not rule out severe respiratory muscle weakness
3. Unexplaines hypoxemia:thinks pulmonary embolism
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This presentation is dedicated to Professor Jean-Claude Raphael
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0
5
10
15
20
25
30
35
40
45
50
0 2 4 6 8 10 12 14 16
PE (2)IVIG (0.5g/kg/d)
Days Gajdos et al Ann Neurol 1997
Clin
ical
res
pon
ses
(% o
f b
asel
ine)
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0
5
10
15
20
25
30
35
40
45
50
0 2 4 6 8 10 12 14 16
Days
PE3 days-IVIg5 days-IVIg
Gajdos et al Ann Neurol 1997
Cli
nica
l res
pon
ses
(% o
f ba
seli
ne)
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ADMISSION
1. ASCENDANT WEAKNESS (70%) – Flaccid symetric areflexic weakness– Pure motor deficit: 18%
2. SENSORY SYMPTOMS (50 à 80 %)– Paresthesia, pain– Superficial and deep sensory impairment
3. CRANIAL NERVES– VII: 24-55%; IX-X: 6-46%; III-IV-VI: 5-13%– XII: 1 - 13%
4. DYSURIA (10 - 30%)
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GUILLAIN-BARRE SYNDROME COURSE
GUILLAIN-BARRE SYNDROME COURSE
Day 0Day 0 TimeTime
Motor deficit Extension Recovery
PlateauPlateau
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ELECTROPHYSIOLOGY
Durand et al – Neurology - 2005
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PHRENIC NERVE ELECTROPHYSIOLOGY
Durand et al – Neurology - 2005
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DIAPHRAGM ELECTROPHYSIOLOGY
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Durant et al – Lancet Neurology - 2006
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ELECTROPHYSIOLOGY
Durant et al – Lancet Neurology - 2006
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ELECTROPHYSIOLOGY
Durant et al – Lancet Neurology - 2006
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ELECTROPHYSIOLOGY
Durant et al – Lancet Neurology - 2006
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CPN p/d: conduction block on common peroneal nerve (%)VC: vital capacity (%)
Durand et al – Lancet Neurol - 2006
ELECTROPHYSIOLOGYP
RE
DIC
TIV
E M
OD
EL
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EARLY PREDICTORS
INABILITY OR 95% CI p
PROGRESSION < 7 days
2.51 (1.7 – 3.8) < 0.0001
STAND 2.53 (1.4 – 3.3) < 0.0005
RISE ELBOW 2.99 (1.8 – 4.9) < 0.0001
RISE HEAD 4.34 (2.7 – 6.7) < 0.0001
COUGH 9.09 (4.0 – 20.00) < 0.0001
CYTOLYSIS 2.09 (1.4 – 3.2) < 0.0005
n = 722
Sharshar et al - Crit Care Med - 2003
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REPEATED ASSESSMENT
1. 30 to 50% fall in VC
or VC < 20 ml/kg
2. 30% fall in Pimax or Pemax
or Pimax ou Pemax< 30 to 40 cmH2O
3. Bulbar dysfunction
Hahn et al - Arch Neurol - 2001
Chevrolet et al - Rev Respir Dis - 1991
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MECHANICAL VENTILATION
Walgaard et al – Ann Neurol - 2010
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OTHER ABNORMALITIES
HYPONATREMIAE1. Hyponatremia < 133 mmol/L : 31%2. Pseudohyponatremia due to IvIg: 46%3. Hyponatremia: worst outcome ?
LIVER DYSFUNCTION
1. Cytolysis: 25%
2. Secondary to CMV: 25%
3. Predictors of MV
Colls Int Med J 2003; Oomes et al Neurology 1996;
Sharshar et al Crit Care Med 2003
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AN
XIE
TY
n 105
Age, yr / Women, % 50 [34-62] / 38 (36)
Delay from onset to inclusion, days 6 [4-9]
Arm grade > 2/5/ Disability grade > 3/5 48 (46)/ 38 (36)
Vital Capacity (%) 72 [52-89]
Mechanical ventilation (MV), % 23 (22)
STAI (from 20 to 80) 47.2 [36.2-57.0]
VAS-breathlessness (from 0 to 10) 2 [0-4]
VAS-anxiety(from 0 to 10) 5 [3-8]
Main cause of distress, %
-breathing
-uncertainty
-weakness
-pain
3 (3)
25 (24)
49 (47)
28 (27)Marcadet et al - Submitted
STAI: State-Trait Anxiety Inventory
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MECHANISMS OF RESPIRATORY FAILURE
• Weakness of inspiratory muscles (hypoventilation)• Weakness of expiratory muscles (cough impairment)• Bulbar weakness (aspiration)• Pneumonia• Laryngeal spasm• No-obstructive sleep apnea (due to diaphragm
weakness )
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MECHANISMS OF RESPIRATORY FAILURE
• Invasive thymoma
• Complication of radiotherapy
• Complication of thymectomy
• Other auto-immune disease (sclerodermia…)
• Paraneoplastic encephalopathy
• Other cause (Pneumothorax, PE…)
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SEVERITY AT ADMISSION
1. SWALLOWING IMPAIRMENT - In 6 to 46%
2. RESPIRATORY DYSFUNCTION- Respiratory symptoms in 40 to 60% - Vital capacity < 1 L in 16%
3. CV AUTONOMIC DYSFUNCTION- in about 15% - Correlated with weakness
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GUILLAIN-BARRE SYNDROME
• A « spinal cord » syndrome must be always rule out clinically (sensory level etc…)
• If any doubt, do an MRI of the spine
JUST A WORD BEFOREHAND….