Guillain-Barre Syndrome

סיפור מקרהבת שנתיים ושלושה חודשים, בריאה •מיילדות תקינה, אחת מתאומות, התפתחות תקינה • ימים כאבים ברגל שמאל המעירים משינה בבכי5•ביממה אחרונה מתנדנדת וצולעת בהליכה, משחקת •

בישיבה, בלילה טרם קבלתה התעוררה כל שעה בבכי בגלל כאב

שוללים טראומה, מחלות במשפחה•חודש טרם קבלתה מחלת שלשולים•OPVחוסנה נגד שפעת חודש טרם קבלתה, קיבלה • כארבעה חודשים לפני כן CMVאביה חלה ב- •נבדקה יום טרם קבלתה במיון ושוחררה •

סיפור מקרה

98%, סטורציה 135, דופק 36.9סימנים: חום •ערנית וחיונית, ללא מצוקה נשימתית•אישונים שוים ומגיבים לאור•ללא קשיון עורף או סימני גירוי מנינגיאלי•אא"ג, ריאות, לב , בטן – ב.מ.פ•עור ללא פריחה או כתמים•שלד- ללא נפיחות או אודם במפרקים, טווחי תנועה •

שמורים, ללא רגישות גרמיתנוירולוגית: רושם לטונוס תקין, הופקו רפלקסים •

גידיים, הליכה אטקטית/ מתנדנדת

סיפור מקרה

מעבדה: ספירת דם תקינה:•

WBC 13.4, HGB 13.8, PLT 375•CRP 0.5, ESR 10•GLU 65, UREA 23.5, CREAT 0.21, NA 140, K 4.8,

CPK 44.3, LDH 448, GOT 32.6הדמיה:

•US פרקי ירכיים וברכיים: ללא נוזל וללא הרמת רצועה אילאופמורלית, ללא ממצא חריג אחר

צילום ברכיים וירכיים תקין •

סיפור מקרה

יום לאחר אשפוזה החמרה- יושבת ומסרבת ללכת, ללא •חום, אוכלת ומתנהגת כרגיל, שאר הסקירה המערכתית

תקינה צעדים ונופלת, ניסיון לקום - 2ניסיון הליכה- הולכת •

GOWERמניעה גפיים בחופשיות ובסימטריות בישיבה • 4-3 / 5כוח גס בשתי הגפיים •החזרי פיקה – חלש בשמאל, לא הופק בימין•רושם שהתחושה שמורה •עצבים קרניאלים תקינים, פונדוסים תקינים•ללא רגישות על פני עמוד השדרה, מפרקים חופשיים•

סיפור מקרה

•CT:תקין מוח ועמוד שידרה(, גלוקוז 120ניקור מתני: חלבון מוגבר )•

, ללא תאים56.8 IVIG, 2 באבחנת גיאן ברה הוחל טיפול ב-•

גרם לק"ג מחולק ליומיים. ניטור דופק, ל"ד, סטורציה, מתן שתן•

סיפור מקרה

החמרה במהלך האישפוז עם חולשה •בידיים, לא קמה משכיבה לישיבה, ישיבה

לא יציבה. ללא מעורבות בולברית, ללא מעורבות •

אוטונומית, יציבה נשימתית ימים לאחר סיום הטיפול הלכה ללא 4•

עזרה, אכלה ללא עזרה, עדיין לא הופקו רפלקסים גידיים.

שוחררה למעקב מרפאתי•

סיפור מקרה

בירור נוסף: דגימות צואה לפוליו- שליליות2•תרבית צואה שלילית••CMV .חיובי בשתן, שלילי בנוזל שדרה שליליIGM חיובי, CMV: IGGסרולוגיה ל- •

Guillain-Barre Syndrome

• An acquired disease of the peripheral nervous system

• Major features: weakness and areflexia• The most common cause of acute

flaccid paralysis in all ages. • No specific test to confirm the

diagnosis• A syndrome rather than a disease

THE ORIGINAL PAPER 1916

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Epidemiology

• Annual incidence: 0.4 – 1.7 / 100.000

• Rare before 1 year of age• M:F - 1.5:1

Guillain-barre Syndrome Clinical features

• Progressive weakness and diminished deep tendon reflexes in a symmetric distribution.

• Ascending progression – most common

• 5 – 10% upper >> lower• 5-10% proximal >> distal

Guillain-barre SyndromeClinical features

• Sensory disturbances: 40% pain or paresthesias

• Cranial nerves• Autonomic disturbances –

infrequent but life threatening. • 15-20% progress to respiratory

failure.

Guillain-barre Syndromediagnostic features

Clinical features

Stages:• Progression phase- days to weeks,

max. 6 weeks. Period of major complications

• plateau phase - days to weeks• Recovery – weeks to months

clinical variants

Laboratory findings:

CSF• normal cell count – up to 10

lymphocytes• elevated protein – 80-200 mg/dl ,

after 1 week

Laboratory findings

Electrophysiological tests:• 80% abnormal studies• Multiple nerves must be studied• Evidence of multifocal demyelination in motor

and sensory nerves. • prolonged distal latency• Reduction of the F WAVE response and H-

REFLEX. • H REFLEX – single most sensitive test for

early GBS, absent in 97% of pts in the first week )Gordon at al. neurol 2001(

Pathology

Depend upon the form of GBS. AIDP and MILLER-FISHER:• Inflammation and demyelination• More severe inflammation at the

junction of dorsal and ventral roots at the site of the dural attachment.

• Secondary axonal degeneration

                                                 

Histology

Pathology

Motor and motor-sensory variants: • Axonal degeneration without an

inflammatory response• The immune process is directed at

the nodes of Ranvier• No demyelination

Pathophysiology

• Immune mediated disease• Possible mechanism: autoAB bind

to glycoproteins on peripheral myelin , causing a cascade of events which eventually destroy the myelin.

Pathophysiology

• 50 – 70% have an antecedent illness within the previous 4 weeks.

• URTI, gastroenteritis• C. Jejuni – more severe symptoms• CMV, EBV

The Lancet; Nov 5-Nov 11, 2005; 366, 9497;

• Campylobacter is the most commonly identified precipitant of GBS

• Demonstrated in up to 30% of cases• 27 / 103 pts )26%( with GBS had

evidence of C.jejuni infection compared with 1% of controls.

• 70% of those infected with C.jejuni reported diarrheal illness within 12 weeks before the onset of GBS.

Guillain-barre Syndromeand Campylobacter

• Campylobacter associated GBS have a worse prognosis – slower recovery and greater residual neurological disability.

• The risk of developing GBS during the 2 months after C.jejuni infection is 100-fold higher than the risk in the general population!

)30.4/100,000 compared with 0.3/100,000(

J Infect Dis. 2006 Feb 15;193(4):547-55.  Comprehensive Analysis of Bacterial Risk Factors for

the Development of Guillain-Barre Syndrome after Campylobacter jejuni Enteritis.

Koga M, Gilbert M, Takahashi M, Li J, Koike S, Hirata K, Yuki N.

• Specific strains )class A, serotype HS:19( were found more frequently )78% vs 17%( in patients with GBS than in patients with enteritis.

• Class A strain carries gangliosid -like lipo-oligosaccharide

• Increased risk of producing antiganglioside autoantibodies and developing GBS.

Pathophysiology

Immunizations:

• “swine flu” vaccine (1976)

• Rabies vaccine

AAP News Vol. 26 No. 11 November 2005, p. 6© 2005 American Academy of Pediatrics

AAP News Vol. 26 No. 11 November 2005, p. 6© 2005 American Academy of Pediatrics

Guillain-Barré cases among recipients of meningococcal conjugate vaccine

The Food and Drug Administration (FDA) and the Centers for Disease Control and Prevention (CDC) are investigating six cases of Guillain-Barré syndrome that occurred after receipt of meningococcal conjugate vaccine (MCV4, Menactra – sanofi pasteur). This number of cases of Guillain-Barré syndrome is not greater than would be expected in an unvaccinated teenage population. However, the onset of neurologic symptoms within two to five weeks following vaccination is a reason to gather further information.

The AAP Committee on Infectious Diseases continues to recommend MCV4 for adolescents "Anyone who has ever had Guillain-Barré syndrome should talk with their doctor before getting MCV4."

Differential Diagnosis• Cerebral• Cerebellar• Spinal• Anterior horn cell• Peripheral nerve

• Neuromascular junction

• muscle

– Bilateral strokes– Acute cerebellar ataxia– Transverse myelitis– Compressive myelopathy– Poliomyelitis )wild/ vaccine(– Toxic neuropathy– Diphtheria– Porphyria– Tick paralysis– Botulism– Myasthenia gravis– Neuromascular blocking agents– Acute inflammatory myopathies – Metabolic: periodic paralysis

Hypotonia, pleocytosis

level

Asymmetric,pure motorencephalopat

hy

Ptosis, reflexes

Constipation, swallow

Proximal weakness

Therapy

• Supportive• Monitoring: BP, HR, RR, TEMP. • Frequent physical examinations to

establish a trend. • Serial lung function testing• If dysphagia or shoulder

weakness: respiratory assistance may be necessary.

Therapy

Indications for transfer to ICU: • Respiratory failure• Autonomic dysfunction• Bulbar dysfunction• Bilateral facial weakness• aspiration

Therapy

• Anticipate the need for mechanical ventilation – better to intubate electively!

• Vital capacity < ½ normal for age or <15-20 ml /kg

• Serial measurements of pulmonary function tests were most helpful in detecting the risk of respiratory failure )Arch Neurol 2001(

• Children < 6 years – monitor fatigue: blood gases, chest x-ray.

Therapy

American Academy of Neurology recommendations for treatment of GBS: indications for treatment:

• Rapidly progressing weakness• Worsening respiratory status or need

for mechanical ventilation• Significant bulbar weakness• Inability to walk unaided.

GBS Motor Disability Scale

grade Motor disability

0 Normal

1 Minor symptoms

2 Able to walk 5 meters unaided

3 Able to walk 5 meters aided

4 Bedridden

5 Respiratory failure

6 death

Therapy

Corticosteroids:• First immunotherapy for GBS • “Corticosteroid medications do not seem to

help improve symptoms or lessen nerve damage from Guillain-Barre syndrome” - The Cochrane Database of Systematic Reviews 2000 (6 RCT’s, 195 pts treated with steroids vs 187 pts with supportive care)

• No indication as monotherapy, evidence of benefit if added to IVIG.

Therapy

The Cochrane Database of Systematic Reviews 2002

Plasma exchange for Guillain-Barre syndromeRaphal JC, Chevret S, Hughes RAC, Annane

"Plasma exchange is the first and only treatment that has been proven to be superior to supportive treatment alone in Guillain-Barre syndrome. Consequently, plasma exchange should be regarded as the treatment against which new treatments, such as intravenous immunoglobulin, should be judged."

Therapy

Plasma exchange• reduce length of stay in the ICU and in hospital• Reduce need for and period of ventilation• Reduce length to unaided walking and neurological sequele. • 4 double-volume exchanges on alternate days over 1 week.• exchange with albumin 5%• more beneficial when started within seven days of the

disease onset.

Therapy

• Small children: access problems )need central line – thrombosis, bleeding, infections(, CVS instability after exchange

• No EBM regarding plasmapheresis in children

Therapy

Use of plasma exchange:• severe disease• Failure of IVIG treatment• Previous adverse reaction to IVIG

Therapy

IVIG• Probably same effects as plasma

exchange or even superior • More available, less side effects• 2 g/ Kg over 2-4 days• Caution: early transient relapse

after IVIG administration

The Cochrane Database of Systematic Reviews 2006Intravenous immunoglobulin for Guillain-Barre

syndromeHughes RAC, Raphael J-C, Swan AV, van Doorn PA

• 6 RCT’s, 536 patients )mostly adults(• Plasma exchange vs IVIG• No statistically significant difference in

primary and secondary outcome• Regimen: no significant difference

between 5 days and 2 days• 3 studies including 75 children

suggested that IVIG significantly hasten recovery compared with supportive care.

Immune globulins are effective in severe pediatric Guillain-Barre syndrome.

Shahar E, Shorer Z, Roifman CM, Levi Y, Brand N, Ravid S, Murphy EG.

Pediatr Neurol. 1997 Jan;16(1):32-6.

• 26 children,Prospective, no control • All bedridden, 2 needed artificial ventilation• All treated with IVIG 2g/kg in 2 days• No adverse effects• 25 improved in 1-2 grades in GMDS within 2

weeks• 18 recovered by 2 weeks• The rest recovered within 4 months• Rapid improvement compared with the

natural history of GBS.

Outcome of severe Guillain-Barre syndrome in children: comparison between untreated cases

versus gamma-globulin therapy.Shahar E, Leiderman M.

Clin Neuropharmacol. 2003 Mar-Apr;26(2):84-7• Retrospective, 23 children• 15 treated with IVIG• 5 supportive care, 3 oral steroids• Improvement by 1 grade in the GMDS

mean 10 days in the IVIG group vs 30 days

• Walk unaided: mean 20 days vs 100 days

Eur J Paediatr Neurol. 1997;1(1):7-12.Intravenous immunoglobulin treatment in

children with Guillain-Barre syndrome.Kanra G, Ozon A, Vajsar J, Castagna L, Secmeer G,

Topaloglu H

• 47 children treated with IVIG compared with 28 treated with supportive care

• Retrospective, case-control

• Mean time to improve in 1 grade in GMDS was 20 days in the IVIG group vs 62 days in the control

Prognosis

• 3% mortality• Recovery- 1-6 months• 80% have complete recovery in

12 months

תודה

CIDP

Neonatal Guillain-Barre syndrome.

al-Qudah AA, Shahar E, Logan WJ, Murphy EG.

Department of Pediatrics; Hospital for Sick Children, Toronto, Ontario, Canada.

A term female infant had the clinical manifestations and accompanying electrophysiologic studies to fulfill the criteria of Guillain-Barre syndrome. At birth, she presented with generalized hypotonia, paucity of lower limb movements, and diminished muscle stretch reflexes. At 3 weeks of age, motor nerve conduction studies demonstrated evidence of demyelination and axonal involvement. Progressive clinical improvement was observed beginning at the age of 2 weeks with subsequent normalization of clinical examinations and nerve conduction studies. To our knowledge, this patient is the youngest reported with Guillain-Barre syndrome.