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Guillain-Barre Syndrome. סיפור מקרה בת שנתיים ושלושה חודשים, בריאה מיילדות תקינה, אחת מתאומות, התפתחות תקינה 5 ימים כאבים ברגל שמאל המעירים משינה בבכי.

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Presentation on theme: "Guillain-Barre Syndrome. סיפור מקרה בת שנתיים ושלושה חודשים, בריאה מיילדות תקינה, אחת מתאומות, התפתחות תקינה 5 ימים כאבים ברגל שמאל המעירים משינה בבכי."— Presentation transcript:

1 Guillain-Barre Syndrome

2 סיפור מקרה בת שנתיים ושלושה חודשים, בריאה מיילדות תקינה, אחת מתאומות, התפתחות תקינה 5 ימים כאבים ברגל שמאל המעירים משינה בבכי 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 חוסנה נגד שפעת חודש טרם קבלתה, קיבלה OPV אביה חלה ב - CMV כארבעה חודשים לפני כן נבדקה יום טרם קבלתה במיון ושוחררה

3 סיפור מקרה סימנים : חום 36.9, דופק 135, סטורציה 98% 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 גרמית נוירולוגית : רושם לטונוס תקין, הופקו רפלקסים גידיים, הליכה אטקטית / מתנדנדת

4 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 32.6 הדמיה : US פרקי ירכיים וברכיים : ללא נוזל וללא הרמת רצועה אילאופמורלית, ללא ממצא חריג אחר צילום ברכיים וירכיים תקין

5 סיפור מקרה יום לאחר אשפוזה החמרה - יושבת ומסרבת ללכת, ללא חום, אוכלת ומתנהגת כרגיל, שאר הסקירה המערכתית תקינה ניסיון הליכה - הולכת 2 צעדים ונופלת, ניסיון לקום - GOWER מניעה גפיים בחופשיות ובסימטריות בישיבה כוח גס בשתי הגפיים 5 / 4-3 החזרי פיקה – חלש בשמאל, לא הופק בימין רושם שהתחושה שמורה עצבים קרניאלים תקינים, פונדוסים תקינים ללא רגישות על פני עמוד השדרה, מפרקים חופשיים

6 סיפור מקרה CT מוח ועמוד שידרה : תקין ניקור מתני : חלבון מוגבר ( 120 ), גלוקוז 56.8, ללא תאים באבחנת גיאן ברה הוחל טיפול ב - IVIG, 2 גרם לק " ג מחולק ליומיים. ניטור דופק, ל " ד, סטורציה, מתן שתן

7 סיפור מקרה החמרה במהלך האישפוז עם חולשה בידיים, לא קמה משכיבה לישיבה, ישיבה לא יציבה. ללא מעורבות בולברית, ללא מעורבות אוטונומית, יציבה נשימתית 4 ימים לאחר סיום הטיפול הלכה ללא עזרה, אכלה ללא עזרה, עדיין לא הופקו רפלקסים גידיים. שוחררה למעקב מרפאתי

8 סיפור מקרה בירור נוסף : 2 דגימות צואה לפוליו - שליליות תרבית צואה שלילית CMV חיובי בשתן, שלילי בנוזל שדרה. סרולוגיה ל - CMV: IGG חיובי, IGM שלילי

9 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

10 THE ORIGINAL PAPER 1916 pport Group Downloads | Guestbook | Forum | Contact Us | Books & Shop| LinksGuestbookForumContact UsBooks & ShopLinks Home About Us About GBS & CIDP Support Members / Join Health Professionals Donate About Us About GBS & CIDP Support Members / Join Health Professionals Donate Return to the Home page. Information about the Charity: our history, constitution, location etc. Comprehensive information about GBS, CIDP and related conditions that you can view, download and print. We provide practical and emotional support (including home and hospital visits) to patients and families in the UK and Ireland. How to join the Support Group and Pages of information for our members. Further information for health professionals. Support us by making a financial donation to the Support Group. Home / About GBS & CIDP / The History of GBS & CIDPAbout GBS & CIDP The History of GBS & CIDP < Previous Page << Home Page Background Quick Guide See Also... Contact Us Contact Us GuestbookForumGuestbookForum Advanced Search Descriptions of progressive numbness and weakness over a short period followed by spontaneous recovery exist in medical papers of the early 19th century. However the peripheral nervous system was little understood so no informed explanation for the symptoms was possible. It was not until 1848 (Graves) that it was suggested that such paralysis came from within the nervous system. Landry's Ascending Paralysis Certainly the best description of 'ascending paralysis' in this period was made by a Frenchman named Jean Baptiste Octave Landry de Th é zillat in Landry's description was based on ten cases, five of his own and five from the medical literature. In one case, Landry gave a long description of a patient who eventually died of asphyxia. Landry's superior, a Dr Glauber who had admitted the patient, had diagnosed hysteria but Landry predicted the patient's demise at an early stage. Landry offered no explanation as no abnormality was found during a post mortem. Glauber added a note however, speculating a close connection between Landry's cases and the paralysis that follows dyptheria. While Guillain-Barr é syndrome was subsequently found to be unconnected with dyptheria, Glauber's observation was vindicated as both types of paralysis are due to a demyelinating neuropathy. A translation of Landry's paper reads: 'The sensory and motor systems may be equally affected. However the main problem is usually a motor disorder characterised by a gradual diminution of muscular strength with flaccid limbs and without contractures, convulsions or reflex movements of any kind. In almost all cases micturition and defaecation remain normal. One does not observe any symptoms referable to the central nervous system, spinal pain or tenderness, headache or delirium. The intellectual faculties are preserved until the end. The onset of the paralysis can be preceded by a general feeling of weakness, pins and needles and even slight cramps. Alternatively the illness may begin suddenly and end unexpectedly. In both cases the weakness spreads rapidly from the lower to the upper parts of the body with a universal tendency to become generalised. 'The first symptoms always affect the extremities of the limbs and the lower limbs particularly. When the whole body becomes affected the order of progression is more or less constant: (1) toe and foot muscles, then the hamstrings and glutei, and finally the anterior and adductor muscles of the thigh; (2) finger and hand, arm and then shoulder muscles; (3) trunk muscles; (4) respiratory muscles, tongue, pharynx, oesophagus, etc. The paralysis then becomes generalised but more severe in the distal parts of the extremities. The progression can be more or less rapid. It was eight days in one and fifteen days in another case which I believe can be classified as acute. More often it is scarcely two or three days and sometimes only a few hours. 'When the paralysis reaches its maximum intensity the danger of asphyxia is always imminent. However in eight out of ten cases death was avoided either by skillful professional intervention or a spontaneous remission of this phase of the illness. In two cases death occurred at this stage... When the paralysis recedes it demonstrates the reverse of the phenomenon which signaled its development. The upper parts of the body, the last to be affected, are the first to recover their mobility which then returns from above downwards.' The term 'Landry's ascending paralysis' was first used in 1876 (Westpahl). The usual treatment was with strychnine which probably did the unfortunate patients more harm than good. Landry contributed no more to neurology for he died of cholera just six years after publishing his paper. Acute Febrile Polyneuritis Acute febrile polyneuritis was one of six classes of polyneuropathy proposed by Ostler in Ostler considered that some of Landry's patients had fallen into this category while others had suffered from myelitis (inflammation of the myelon [spinal cord]). Ostler's description was of an illness similar to what we now call GBS but with the fundamental difference of showing a fever. In 1918, Bradford et al described 'acute infective polyneuritis'. They stated this to be the same as acute febrile polyneuritis, any fever having recovered before the onset of the neurological symptoms or being due to subsequent infection. In the meantime, developments had been published in France...

11 Epidemiology Annual incidence: 0.4 – 1.7 / Rare before 1 year of age M:F - 1.5:1

12 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

13 Guillain-barre Syndrome Clinical features Sensory disturbances: 40% pain or paresthesias Cranial nerves Autonomic disturbances – infrequent but life threatening % progress to respiratory failure.

14 Guillain-barre Syndrome diagnostic features

15

16 Clinical features Stages: Progression phase- days to weeks, max. 6 weeks. Period of major complications plateau phase - days to weeks Recovery – weeks to months

17 clinical variants

18 Laboratory findings: CSF normal cell count – up to 10 lymphocytes elevated protein – mg/dl, after 1 week

19

20 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)

21

22 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

23

24 Histology

25 Pathology Motor and motor-sensory variants: Axonal degeneration without an inflammatory response The immune process is directed at the nodes of Ranvier No demyelination

26 Pathophysiology Immune mediated disease Possible mechanism: autoAB bind to glycoproteins on peripheral myelin, causing a cascade of events which eventually destroy the myelin.

27 Pathophysiology 50 – 70% have an antecedent illness within the previous 4 weeks. URTI, gastroenteritis C. Jejuni – more severe symptoms CMV, EBV

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

29 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.

30 Guillain-barre Syndrome and Campylobacter Campylobacter associated GBS have a worse prognosis – slower recovery and greater residual neurological disability.

31 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)

32 J Infect Dis Feb 15;193(4): 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. Koga MGilbert MTakahashi MLi JKoike SHirata KYuki 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.

33

34 Pathophysiology Immunizations: “swine flu” vaccine (1976) Rabies vaccine

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

36 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 motor encephalopathy Ptosis, reflexes Constipation, swallow Proximal weakness

37 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.

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

39 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.

40 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.

41 GBS Motor Disability Scale gradeMotor disability 0Normal 1Minor symptoms 2Able to walk 5 meters unaided 3Able to walk 5 meters aided 4Bedridden 5Respiratory failure 6death

42 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.

43 Therapy

44 The Cochrane Database of Systematic Reviews 2002 Plasma exchange for Guillain-Barre syndrome Raphal 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."

45 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.

46 Therapy Small children: access problems (need central line – thrombosis, bleeding, infections), CVS instability after exchange No EBM regarding plasmapheresis in children

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

48 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

49 The Cochrane Database of Systematic Reviews 2006 Intravenous immunoglobulin for Guillain-Barre syndrome Hughes 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.

50 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 Jan;16(1):32-6. Shahar EShorer ZRoifman CMLevi Y Brand NRavid SMurphy EG 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.

51 Outcome of severe Guillain-Barre syndrome in children: comparison between untreated cases versus gamma-globulin therapy. Shahar E, Leiderman M. Clin Neuropharmacol Mar-Apr;26(2):84-7 Shahar ELeiderman M 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

52 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 Kanra GOzon AVajsar JCastagna LSecmeer 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

53 Prognosis 3% mortality Recovery- 1-6 months 80% have complete recovery in 12 months

54 תודה

55 CIDP

56 Pediatr Neurol Jul-Aug;4(4): Related Articles, Related Articles, Links 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. al-Qudah AAShahar ELogan WJMurphy EG


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