Guillain Barre Syndrome

Topics: Nervous system, Immune system, Action potential Pages: 6 (830 words) Published: September 9, 2010
Guillain-Barre Syndrome (GBS)
Iman binti Jeffrey 0611750

Definition Epidemiology Etiology Pathogenesis & Pathology Clinical features Investigations Diagnosis Differential diagnosis Treatment Prognosis


Postinfectious polyneuropathy involving mainly motor but sometimes also sensory and autonomic nerves Affects people of all ages and is not


Most patients have a demyelinating neuropathy, but primarily axonal degeneration is documented in some cases. also known as: Acute inflammatory demyelinating polyneuropathy (AIDP)

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usually in children over 4-9 years overall frequency is 1.9 cases per 100,000 population follows infection or immunization by 10 days follows respiratory tract infection or gastrointestinal infection

GIT infection Campylobacter jejuni (26-41%) Cytomegalovirus (10-22%) Respiratory tract infection Mycoplasma pneumoniae Ebstein-Barr virus (10%) Vaccines  

Rabies Avian-flu influenza

Molecular mimicry
Cross-reactive immune attack by host Ab & T cell with are directed against the pathogen & nerve components.

Eg: Campylobacter jejuni
Immune response directed against capsular LPS producing Ab cross-reacting with myelin to cause demyelination (mimics the gangliosides)

Presentation of Ag to naïve T cell → activation ↓ Activated T cells attach to venular endothelium of peripheral nerves ↓ Migrate through endothelial lining to perivascular location ↓ Sensitized → contact myelin → segmental demyelination

Inflammatory lesions scattered throughout the peripheral nervous system Circumscribed areas of myelin loss associated with presence of lymphocytes and macrophages Initial lesion: nodes of Ranvier Myelin damage: penetration of macrophages into basement membrane around nerve fibers & strip myelin away Severe cases: interruption of axon & wallerian degeneration

Clinical Features
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Onset is gradual and progresses over weeks Lower extremities (unable/refusal to walk)  trunks  upper limbs  bulbar muscles  flaccid tetraplegia = Landry Ascending Paralysis Proximal and distal muscles are involved relatively symmetrically, but asymmetry is found in 9% of patient

Muscle tenderness – At the onset Paraesthesias – in some cases Areflexia (83%)

Clinical Features
Bulbar involvement (50%)
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Dysphagia and facial weakness – signs of impending respiratory failure Interfere with eating. Increase risk of aspiration

Cranial nerve involvement (50%)
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Facial nerve Oculomotor nerve

Autonomic involvement
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Lability of blood pressure Postural hypotension Profound bradycardia Occasional asystole Urinary retention or incontinence (20% of cases, usually transient)

Clinical Features

Symptoms of viral meningitis / meningoencephalitis In young children CNS involvement ataxia papilledema Miller-Fisher syndrome External ophtalmoplegia ataxia areflexia

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Table 1:Clinical features in 49 children with GBS*
Features Age Male/female ratio Weakness Pain Ataxia Paraesthesias Shortness of breath

Prevalence 1.2:1 73% 55% 44% 18% 4%

7.1years (mean)

* Data from unpublished observation of John Sladky. Two patients had consistent findings of Fisher syndrome

Initial phase  Gradually increasing involvement lasts 10-30 days (less than 4 weeks) Plateau phase  Short phase (within 2 weeks)  Long plateau phase → poor prognosis Recovery phase  Within months  Usually complete  Motor sequelae (5-25%)  Relapse & late recurrences (3%)

Lumbar puncture – cerebrospinal fluid (CSF)
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Elevation of CSF protein (more than twice upper limit of normal) Cell content of CSF is normal (4 weeks)

Acute motor-axonal neuropathy (AMAN) Miller-Fisher syndrome Chronic IDP (CIDP)

Required for diagnosis Supportive
Progressive motor weakness involving >1...

References: 

Menkes & Sarnat: Child Neurology, 6th ed. USA, Lippincott Williams & Wilkins, 2000 Aicardi, Jean: Clinics in Developmental Medicine: Diseases of The Nervous System in Childhood, 2nd ed. London, Mac Keith Press, 1998 Behrman, Kliegman, Jenson: Nelson Textbook of Pediatrics, 17th ed. China, Elsevier Saunders, 2004
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