DR GUILLAIN - Gurrain barren syndrome.pptx

GUILLAIN-BARRE SYNDROME
DR SINGOYI
KASAMA GENERAL HOSPITAL

OUTLINE
• VIGNETTE
• INTRODUCTION
• CLASSIFICATION
• PATHOPHYSIOLOGY
• CLINICAL FEATURES
• INVESTIGATIONS
• COURSE AND PROGNOSIS
• MANAGEMENT
• DDX

VIGNETTE
M/46
• Referal form Mungwi District Hospital
for further investigations and management
c/o .rubbery legs 2/7
Hxpc:
patient was in his usual normal state of health until 3 weeks
ago when ,he experienced several episodes of
diarrhea,which resolved spontaneously

• O/E
• neulogical exam:bilateral lower-extremity weakness and a
loss of reflexes were noted.

INTRODUCTION
Guillain-Barre Syndrome(Gian Bari) also known as an
acute inflammatory or post-infective demyelinating
polyneuropathy.
develops 1-3 weeks after respiratory infection or
diarhearin more than 70% cases
COMMON CAUSATIVE AGENTS
Epstein Barr Virus
CMV
Compylobactor jejuni

• in some patients,it occurs after surgery or immunization
• Age is usually 20-50 years. Predominantly males

CLASSIFICATIONS
• 1)ACUTE INFLAMMATORY DEMYELINATING
POLYNEUROPATHY.
-Most common
Auto-immune responce against schwann cells.
• 2) MILLER FISCHER SYNDROME
rare variant
manifests as descending paralysis
usually affects the eye muscle and presents with

with the triad of Opthalmoplegia,Ataxia and areflexia
• 3)ACUTE MOTOR AXONAL NEUROPATHY
aka Chinese Paralytic Syndrome
Attacks motor nodes of ranvier and is prevelent in Mexico
• 5) ACUTE PANAUTOMATIC NEUROPATHY
Most rare variant of GBS,sometimes accompanied by
encephalopathy; inpaired sweat,lack of tear
formation,photophobia,dry nasal and oral mucosa,itchy
peeling skin

• nausea and dysphagia
• 5)BICKERSTAFF BRAIN STEM ENCEPHALITIS
• Acute onset of opththalmoplegia,Ataxia,disturbance of
consciousness,hyperreflexia

PATHOPHYSIOLOGY
• Demyelination of spinal of nerve roots or peripheral
nerves which is immunologically mediated

CLINICAL FAETURES
Rapid progression of muscle weakness,ascending from
lower to upper limbs and more markedly distally
proximally (Ascending paralysis)
Distal paraesthesia and limb pains often precede the
weakness.
most patients muscle weakness progresses for 1-3
weeks,but rapid deterioration with respiratory muscle
paralysis can occur within hours

in 20% of cases there is involvement of bulbar,facial or
respiratory muscles.
o/e weakness and absent reflexes.There may be sensory
loss at the periphery in an ascending pattern from fingers
and toes
Autonomicneuropathy(hypo/hypertension,tachy/brady-
cardia

INVESTIGATION
• 1.NCV- nerve conduction velocity : slowing of nerve
conduction or conduction block consistent with underlying
segmental demyelination
• 2.CSF- raised protein(maybe normal in the first 2- 10
days).No rise in cell number.lymphocytosis more than
50/mm suggestive of alternative dx

• 3.ECG:
 ST segment depression
T wave inversion
QT prolongation
Tachycardia

COURSE AND PROGNOSIS
UNTREATED CASES
recovery begins after 3 weeks.Pt may need ventilatory
support
Assesment: PFT,vital capacity and ABGs
80% of pts recover completely within 3-6 months
4% die
and remainder suffer residual neurological disability which
can be severe

MANAGEMENT
1.Nursing care
nutritional support
monitoring of ventilatory function
2.Plasmapharesis(Plasma Exchange)
very effective if initiated within 2 weeks of illness
(usually 4-5 sessions are required)
3.Immunoglobulin: High dose IV immuniglobulin 2g/kg x
5/7)as effective as plasmapharesis

N.B No role for corticosteroid here

DDX
• OTHER PARAPLEGICS
• 1.TOXINS
• Drugs:
Isoniazid,Ethambutol,chloroquin,metronidazole,lithium,ph
ytoin,vincristin,amiodaron
• Alcohol
• Hearvy metals:lead,gold,arsenic,mercury,organic solvents

• 2.VITAMIN DEFICIENCY
• vitamin A,B2,B6,B12,B9 and E
• 3.METABOLIC AND ENDOCRINE DISORDERS
• DM,Renal Failure,hepatic failure,hypothyroidism and gout
• 4.CONNECTIVE TISSUE
• Rheumatoid arthritis,SLE,polyarteritis nodosa
• 5.MALIGNANT DISEASES
• bronchogenic ca,lymphoma,leukemia,multiple myeloma

• 6.INFECTIONS
• leprosy,typhoid,TB(potts Dzz),meningitis,acute infective
polyneuritis,HIV neuropathy
• 7. STRUCTUAL NEUROPATHIES
• hereditary motor and sensory neuropathies
• 8.SPINAL NEUROPATHIES
• nerve entrapment syndromes
• traumatic -e.g

REFRENCES
• 1.WWW.scribs.com/presentation
• 2www.medicine.arums.ac.ir
• Kaplan’s Internal medicine USMLE step 2
• Manual of Diagnostics

DR GUILLAIN - Gurrain barren syndrome.pptx

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