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GUILLAIN-BARRE SYNDROME and MYASTHENIA GRAVIS

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1 GUILLAIN-BARRE SYNDROME and MYASTHENIA GRAVIS
University of Versailles Raymond Poincaré Teaching Hospital Garches - France

2 STATEMENTS Guillain-Barré Syndrome (GBS) is the most frequent cause of acute paralysis. GBS is secondary to an acute immune-mediated polyneuropathy GBS can be differentiated in various clinical and electrophysiological sub-types Its gravity includes respiratory failure, cardio-vascular autonomic dysfunction, and long-term disability

3 PARALYSIE FLASQUE AIGUË DEFICIT SENSITIVO-MOTEUR
DEFICIT MOTEUR PUR ± IRM LCR VS-CRP-NFS EMG (PN Axonale, PN Démyelinisante) Kaliémie LCR EMG (Myopathie, Myasthénie, Neuropathy)

4 PARALYSIE AIGUË HYPOREFLEXIQUE SENSITIVO-MOTRICE
Signes Pyramidaux Niveau sensitif Sd de la queue de cheval IRM de la moelle

5 PARALYSIE AIGUË HYPOREFLEXIQUE SENSITIVO-MOTRICE
VS NORMALE VS AUGMENTEE LCR NORMAL TOXIQUE (Thallium, arsenic…) METABOLIQUE (Gly, Vit …) VASCULARITES (SLE…) GBS IDIOPATHIQUE HYPERCEL. MENINGORADICULITES HYPERPROT. CANCER, LYMPHOME, VASCULARITES, DIPHTERIE, VIH.

6 DEFICIT MOTEUR PUR HYPOREFLEXIQUE
SIGNES K+ LCR EMG PARALYSIE PERIODIQUE EXERCICE  MYOPATHIE MYASTHENIE VARIATION MVT OCUL. JONCTION NM BOTULISME INTOX. ALIM. PUPILLES POLIO MYELITES VOYAGE DIARRHEE  CELL. ANTERIOR HORN CELLS PORPHYRIE NEUROPATHIE CONFUSION DOULEUR POLY NEUROPATHIE GBS IDIOPATHIQUE INFECTION ASCENDANT  PROTEINE

7 DEFINITION CRITERES REQUIS Faiblesse progressive de plus de 2 membres
Areflexie Progression en moins de 4 semaines Autre cause exclue CRITERES UTILES Signes sensitifs Hyperprotéinorachie Bloc de conduction à l’ENMG

8 INCIDENCE INCIDENCE: 0.5–2.0 cas/100 000/an SEX RATIO (F/M): < 1.0
AGE RATIO (O/Y): > 1.0 EPIDEMIO: PAS DE VARIATION SAISONNIERE PRODROMES: 70-90%

9 SOUS-TYPES Acute inflammatory demyelinating polyneuropathy (AIDP)
Acute motor axonal neuropathy (AMAN) Acute motor and sensory axonal neuropathy (AMSAM) Miller Fisher syndrome (MFS)

10 PUZZLE Clinic Sensorimotor Pure motor Cranial nerves Pathogen agent
C. jejuni CMV Others Subtypes AIDP AMAN AMSAM MFS Severity Aspiration Intubation Dysautonomia Anti-Ganliosides Ant-GM1 Anti-GM2 Anti-G1QB Outcome Recovery Sensory sequellae Motor sequellae

11 PATHOPHYSIOLOGY Hughes and Cornblath - Lancet

12 ANTICIPER LA DETRESSE RESPIRATOIRE AIGUË

13 VENTILATION MECANIQUE
Requise dans 20 à 30% Délai médian entre admission et VM: 2 j Durée médiane: 21 j 1/3 sevrés de la VM dans les 3 premières semaines

14 RISQUES A RETARDER LA VM
N= 87 PAVM = 67 (75%) Orlikowski et al - ICM

15 MECANISMES DEFICIT ASCENDANT Atteinte bulbaire IX-X Inhalation
Hypoxaemia  toux  Atelectasie Accessores  CV  Pimax  Pemax I will now focus our talk on the respiratory dysfunction. The weakness of abdominal muscles, the diaphragm and respiratory accessory muscles produces a decrease in VC that at a certain level will induce an hypoventilation. Bulbar dysfunction put at risk of aspiration that, in combination with cough impairment and atelectasia, will worsen the VC decrease. Diaphragme Hypoventilation Phrenique Abdominaux

16 Pas de VM Arrêt Respiratoire Début Admission VM
CRITERES TARDIFS CRITERES PRECOCES VM PREDICTEURS TARDIFS Pas de VM PREDICTEURS PRECOCES Mechanical ventilation is a serious event that requires appropriate and on-going consideration in order to avoid an intubation in a hasardous situation. This requires clinical predictors of subsequent respiratory failure in order to appropriately stratify risk. Réa Salle Arrêt Respiratoire Début Admission VM

17 CRITERES D’INTUBATION
CRITERES MAJEURS Signes de détresse respiratoires CV< 15 ml/kg, Pimax ou Pemax < 25 cm H2O PaCO2 > 6,4 kPa PaO2 < 7.5 kPa (FiO2= 0,21) MINOR CRITERIA Toux inefficace Troubles sévères de la déglutition Atélectasies Ropper - Neurology – 1985; Wijdicks-Neurology-1998

18 PROGRESSION < 7 jours
PREDICTEURS PRECOCES n= 196 OR 95% CI p PROGRESSION < 7 jours 5.0 (1.4 – 5.7) < 0.003 Tête (1.9 – 12.5) < CV < 60% 2.86 (2.4 – 10.0) < Sharshar et al - Crit Care Med

19 REPETER LA MESURE DE LA CAPACITE VITALE
No MV MV REPETER LA MESURE DE LA CAPACITE VITALE Durand et al – Neurology

20 ELECTROPHYSIOLOGIE EMNG diaphragmatique ENMG Standard
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)

21 CORTISOLEMIE Variable no 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 [Cortisol]T0 est corrélée avec la survenue de dysautonomie Strauss et al – CCM

22 PEUR N (%) Marcadet et al - Submitted

23 Swallowing dysfunction Onset-Admission < 7 days
LATE CRITERIAE EARLY CRITERIAE EARLY PREDICTORS Inability to lift head VC < 60% +/- High cortisolemia CPN + VC > 80% MV LATE PREDICTORS Fall in VC Swallowing dysfunction No VM VERY EARLY PREDICTORS Onset-Admission < 7 days Uncertainty Mechanical ventilation is a serious event that requires appropriate and on-going consideration in order to avoid an intubation in a hasardous situation. This requires clinical predictors of subsequent respiratory failure in order to appropriately stratify risk. ICU ward Respiratory arrest Onset Admission MV

24 ADMISSION EN REA Progression < 7 j Impossibilité à relever la tête
Troubles de la déglutition CV < 60% oo 20 ml/Kg Pimax ou Pemax< 30 to 40 cmH2O 5. 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

25 TRACHEOSTOMIE VM > 21 j Patients âgés
Pathologie respiratoire pré-existante Test pulmonaire dint/d12 ratio < 1 TP score = CV + Pimax + Pemax Sensibilité = 70%, specificité: 100% Lawn and Wijdicks - Muscle Nerve , 2000

26 VENTILATION MECANIQUE
Blocs de conduction moteurs: VM prolongée Fourrier et al – Crit Care

27 MORBIDITE n = 114 RESPIRATOIRE Tracheobronchitis 33 (29%)
Pneumonia (24%) Pneumothorax (4%) INFECTION Bacteremia (15%) Catheter (3%) Urinary tract (66%) THROMBOSES Deep venous thrombosis (4%) Pulmonary embolism (3%) AUTRES Hyponatremie (< 130 mmol/L) 28 (25%) Henderson et al – Neurology

28 FACTEURS DE RISQUE DE MORBIDITE
n = 114 Characteristiques OR 95% CI p Value Pathologie sous-jacente 2.8 ( ) 0.10 Echanges plasmatiques 3.4 (1.1 – 10.5) 0.03 VM prolongée 12.4 (3.7 – 41.3) < Henderson et al - Neurology

29 TRAITMENT Traitement symptomatique Traitement spécifique
Thrombosis prophylaxis Pain relief Psychological support Autonomic dysfunction: atropine, trandate … Bladder catheter, nutrition, physiotherapy… Intercurrent diseases Non-Invasive ventilation: ? Traitement spécifique Plasma exchange (PE) High-dose intravenous immunoglobulin (IvIg) Treatment consist of specific treatment and supportive therapy that include:… Benefit of Early non-invasive MV in patients at high risk of respiratory failure need to be assessed.

30 ECHANGES PLASMQTIQUES
Plasma exchange vs no treatment: DG at 4 weeks Hughes et al - Brain Benefit irrespective of severity Tested against placebo Tested up to 4 weeks after GBS onset

31 IMMUNOGLOBULINES Plasma exchange vs IvIg: DG at 4 weeks
Hughes et al - Brain Tested against PE Tested in mild or severe GBS Tested within 2 weeks after GBS onset

32 CORTICOSTEROIDES Corticosteroid vs placebo or no treatment: DG at 4 weeks Hughes et al - Brain

33 CONTRE-INDICATIONS EP Infection IvIg Hypotension IgA deficiency
Haemorrhage Clotting deficiency IvIg IgA deficiency Allergy Renal failure Contra-indication for PE are… and for IvIg are…

34 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 Patients who are able to walk should be treated by 2 PE that should be repeated if the patients deteriorate. Bedbound or ventilated patients can be treated either by 4PE or five days of IvIg. There is no benefit to increase the number of PE if they deteriorate or to combine PE with IvIg. The treatment should be started as soon as the diagnosis is confirmed. Two other randomised trials are expected: - one asseses the benefit of combining of IvIg with setroids - the other one, coordinated by Pr Raphael from Garches, compare PE to IvIg at different level of severity. t *Aussitôt que le diagnostique est confirmé ** 0.4g/kg/ daily for 5 days

35 MYASTHENIE

36 EPIDEMIOLOGIE Prévalence 4-6 / 100 000 Incidence 2-5/million/an
2 F / 1 H F: 30 ans H: 30 et 60 ans

37 MOTS CLES Pathologie de la jonction neuromusculaire
Pathologie auto-immune Pathologie thymique

38 JONCTION NEUROMUSCULAIRE
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 oculaire Faciaux Oropharyngés Myasthénie bulbaire Myasthénie Respiratoires généralisée Des membres et axiaux

39 SCORE DE LA FORCE MUSCULAIRE (1)
Membres supérieurs étendus à l’horizontale en antéroposition 1 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 cuisses 1 point pour 5 s Max. 15 Flexion de la tête en décubitus dorsal Contre résistance Sans résistance Impossible Passage de la position couchée à la position assise Possible sans l’aide des mains 10 Impossible sans l’aide des mains Oculomotricité extrinsèque Normal Ptosis isolé Diplopie

40 SCORE DE LA FORCE MUSCULAIRE (2)
Occlusion palpébrale Complète Signe de Souques ,5 Incomplète (mais avec recouvrement cornéen) 5 Nulle (sans recouvrement cornéen) 0 Mastication Normale Diminuée Nulle Déglutition Dysphagie sans fausse route Dysphagie avecfausse route Phonation Voix normale Voix nasonnée Aphonie

41 ATTEINTE OCULAIRE

42 ELECTROPHYSIOLOGIE Stimulations répétées à 3HZ Décrément

43 PATHOLOGIE AUTO-IMMUNE
Auto-Anticorps - Thymus Evolution par poussées Autre(s) pathologie(s) auto-immune(s)

44 POUSSEE OU CRISE MYASTHENIQUE
PREMIERE ETAPE Vérification des critères diagnostiques Anticorps anti-Rach + Ou EMG + et Test à la Prostigmine + Sinon Biopsie musculaire Vérification de l’existence d’un thymome Si oui, scanner systématique Vérification d’une pathologie auto-immune Dysthyroïdie, etc… Récapitulation anamnestique Récapitulation des traitements utilisés

45 POUSSEE OU CRISE MYASTHENIQUE
DEUXIEME ETAPE Recherche d’un facteur déclenchant (70%) Infection (30-40%) Intervention chirurgicale Grossesse 19% d’aggravation) 1er et 3eme trimestre Traitement contre-indiqué A interrompre si possible Surdosage en anticholinestérasique 4% des cas de poussée Ne pas faire de test à la Prostigmine Institution d’une corticothérapie Survenue d’une poussée dans 48% des cas Décroissance du traitement immunosuppresseur

46 EXACERBATION or CRISIS
Lacomis et al – NeuroCritCare

47 EXACERBATIONS Lacomis et al – NeuroCritCare

48 POUSSEE OU CRISE MYASTHENIQUE
TROISIEME ETAPE Evaluation de la gravité Atteinte oropharyngée Atteinte respiratoire Difficilement détectable Mesure de la CV indispensable La normalité des GDS ne doit pas rassurer Si Aggravation rapide, fluctuation clinique, atteinte axiale, oropharyngée, respiratoire ou CV < 60% Transport médicalisé Hospitalisation en réanimation

49 POUSSEE OU CRISE MYASTHENIQUE
QUATRIEME ETAPE Evoquer un diagnostic différentiel Autre cause d’insuffisance respiratoire Autre pathologies contingentes ou associées Dysthyroïdies etc.. Effets secondaires des traitements Diabète, Insuffisance surrénalienne etc…

50 CRISE MYASTHENIQUE Incidence Mortalité
16% de la population générale myasthénique 23 et 70% dans les 6 à 36 premiers mois Mortalité 6% (40% avant l’assistance respiratoire)

51 POUSSEE ou CRISE FIRST STEP Diagnostic criteria for MG Thymoma
If yes, thoracic CT scan Other auto-immune disease Course of MG Recapitulation of MG specific treatment

52 POUSSEE ou CRISE FIRST STEP Diagnostic criteria for MG Thymoma
If yes, thoracic CT scan Other auto-immune disease Course of MG Recapitulation of MG specific treatment

53 MORTALITE Alsheklee et al – Neurology

54 MECHANISMS ASCENDING PARESIS Bulbar dysfunction IX-X Aspiration
Hypoxaemia  cough  Atelectasia Laryngeal spasm Accessory  CV  Pimax  Pemax I will now focus our talk on the respiratory dysfunction. The weakness of abdominal muscles, the diaphragm and respiratory accessory muscles produces a decrease in VC that at a certain level will induce an hypoventilation. Bulbar dysfunction put at risk of aspiration that, in combination with cough impairment and atelectasia, will worsen the VC decrease. Diaphragm Hypoventilation Phrenic Abdominal

55 VENTILATION MECANIQUE
MYASTHENIE VENTILATION 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

56 SEVRAGE Onset of weaning procedure Extubation
Improvement of muscle strength VC > 30% of predicted value FiO2 < 50% and Peep < 5 cm H2O Extubation VC > 50% and DVC 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)

57 Arch Neurol Neurology NeuroCrit Care BE CAREFUL

58 TREATMENT OF EXACERBATION
Survey – No change in specific treatment Improvement Non specific treatment Specific treatment Failure Triggering factor ?  Treatment overdose Chol I?  reduction Swallowing dysfunction ?  NGT Respiratory failure ?  MV PE or IvIg CS or IS yes no CS dose x2 CS: 1 mg/kg I.S. no change I.S.

59 BLOOD GASES « FAUX-AMIS »
Presence of hypercapnia or hypoxemia indicates a severe respiratory muscle weakness and misgives an impeding respiratory arrest Conversely, absence of blood gases abnormality does not rule out severe respiratory muscle weakness Unexplaines hypoxemia:thinks pulmonary embolism

60 This presentation is dedicated to Professor Jean-Claude Raphael

61 Clinical responses (% of baseline)
5 1 2 3 4 6 8 PE (2) I V G (0.5g/kg/d) Days Clinical responses (% of baseline) Gajdos et al Ann Neurol 1997

62 5 10 15 20 25 30 35 40 45 50 2 4 6 8 12 14 16 Days PE 3 days-IVIg 5 days-IVIg Clinical responses (% of baseline) Gajdos et al Ann Neurol 1997

63 ADMISSION ASCENDANT WEAKNESS (70%) Flaccid symetric areflexic weakness
Pure motor deficit: 18% SENSORY SYMPTOMS (50 à 80 %) Paresthesia, pain Superficial and deep sensory impairment CRANIAL NERVES VII: 24-55%; IX-X: 6-46%; III-IV-VI: 5-13% XII: % DYSURIA ( %)

64 GUILLAIN-BARRE SYNDROME COURSE
Extension Recovery Motor deficit Plateau Day 0 Time

65 ELECTROPHYSIOLOGY Durand et al – Neurology

66 PHRENIC NERVE ELECTROPHYSIOLOGY
Durand et al – Neurology

67 DIAPHRAGM ELECTROPHYSIOLOGY

68 Durant et al – Lancet Neurology - 2006

69 ELECTROPHYSIOLOGY Durant et al – Lancet Neurology

70 ELECTROPHYSIOLOGY Durant et al – Lancet Neurology

71 ELECTROPHYSIOLOGY Durant et al – Lancet Neurology

72 ELECTROPHYSIOLOGY PREDICTIVE MODEL
CPN p/d: conduction block on common peroneal nerve (%) VC: vital capacity (%) Durand et al – Lancet Neurol

73 EARLY PREDICTORS n = 722 INABILITY OR 95% CI p PROGRESSION < 7 days
2.51 (1.7 – 3.8) < STAND 2.53 (1.4 – 3.3) < RISE ELBOW 2.99 (1.8 – 4.9) RISE HEAD 4.34 (2.7 – 6.7) COUGH 9.09 (4.0 – 20.00) CYTOLYSIS 2.09 (1.4 – 3.2) Sharshar et al - Crit Care Med

74 REPEATED ASSESSMENT 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 Chevrolet et al - Rev Respir Dis Hahn et al - Arch Neurol

75 MECHANICAL VENTILATION
Walgaard et al – Ann Neurol

76 OTHER ABNORMALITIES HYPONATREMIAE Hyponatremia < 133 mmol/L : 31%
Pseudohyponatremia due to IvIg: 46% Hyponatremia: worst outcome ? LIVER DYSFUNCTION Cytolysis: 25% Secondary to CMV: 25% Predictors of MV GBS can be complicated by hyponatremia and liver dysfunction in 31% and 25%, respectively. Hyponatremia is related to IVIg administration in 46% of cases and liver dysfunction to CMV in 25%. Interestingly, we found that liver dysfunction is an independent risk factor of MV Colls Int Med J 2003; Oomes et al Neurology 1996; Sharshar et al Crit Care Med 2003

77 ANXIETY 105 50 [34-62] / 38 (36) 6 [4-9] 48 (46)/ 38 (36) 72 [52-89]
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 [ ] 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) ANXIETY Marcadet et al - Submitted STAI: State-Trait Anxiety Inventory

78 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 )

79 MECHANISMS OF RESPIRATORY FAILURE
Invasive thymoma Complication of radiotherapy Complication of thymectomy Other auto-immune disease (sclerodermia…) Paraneoplastic encephalopathy Other cause (Pneumothorax, PE…)

80 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

81 GUILLAIN-BARRE SYNDROME
JUST A WORD BEFOREHAND…. A « spinal cord » syndrome must be always rule out clinically (sensory level etc…) If any doubt, do an MRI of the spine


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