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Guillian- Barre
Syndrome
Acute ascending flaccid paralysis
Assisstant Professor
Dr.Ahmed Abd Ahdi
 In 1859 The first cases were described by Landry, who
noted that the disease could produce motor and sensory
engagement, which committed the distal portion of
extremities and in some cases progressed in
caudocephalic or ascending direction to generalized
involvement .
 In 1916, Guillain and Barre emphasized the importance
of albumin-cytological dissociation for clinical diagnosis
‫اكدو‬
- It is the most frequent form of polyneuritis seen in pediatric
it’s etiology is unknown.
 - It is thought to be due to viral infection as it may follow measles,
chicken box and mumps( Post infectious polyneuritis).
 Roughly two thirds of patients have a history of an antecedent GI or
RT infection.
 Pathogenic triggers of GBS include EBV, CMV, the enteroviruses,
hepatitis A and B, varicella M.pneumoniae and Campylobacter
jejuni, which is perhaps the most common
 These pathogens are believed to activate CD4+ helper-inducer T
cells, which are particularly important mediators of disease
-The condition may follow vaccination ( as rabies vaccine) or serum
sickness.
*Pathology :
There is demyelination of multiple peripheral nerves which is maximal
where the anterior and posterior roots fuse, the damage is mainly motor.
‫االتحاد‬ ‫منطقة‬ ‫عند‬ ‫شيء‬ ‫اكثر‬
Brain; meningitis encephalitis
Spinal cord: infection abcess truma
Anterior horn: Nerve injury muscle
Muscle junction
Muscle: myopathy hypokalemia myositis
hoshion syndrome
Nerve
Lower limp weakness
Parstgesia in lower limp
‫الطوارئ‬ ‫مال‬ presentation‫ال‬
Usually in
motor nerve
Clinical Picture:
 1- Most often an unremarkable infection, such as upper respiratory infection,
often predates the onset of GBS by 14 days.
 Many antecedent infections have been identified, including Campylobacter jejuni,
cytomegalovirus (CMV), Mycoplasma pneumonia, Epstein–Barr virus, influenza virus,
JEV.
 Surgery, immunization, and parturition have also been associated with GBS
2- The symptom may occur insidiously one week or more after acute infection of
respiratory or GIT and consists of:
- Motor manifestations (predominant):
Bilateral symmetrical weakness which begins usually in the lower limbs and progresses
to involve the trunk, upper limb and the bulbar muscles ( ascending paralysis).
- Sensory manifestations : tender muscle and paraesthesia in some cases.
- autonomic manifestations: in some cases as transient urinary incontinence or
retention postural hypotension or hypertension.
Japanese encephalitis
‫ماضي‬
‫الوالدة‬ ‫مخاض‬
Tachycardia, bradycardia
 Neurological examination is characterized by distal
and often proximal, relatively symmetrical,
weakness.
 Although GBS is essentially a motor neuropathy,
sensory dysfunction is seen in a few patients. It is
seen more in a demyelinating form of GBS.
 Sensory examination is often normal in the early
phase of disease. Facial or pharyngeal weakness is
commonly seen in GBS.
 Diaphragmatic weakness due to phrenic nerve
involvement is also common.
Examination
Bilateral symmetrical weakness
Usually anreflexia
Sign of autonmic nerve ( tachycardia ,
bradycardia ,
Paralysis
Cranial nerve involves
‫كلهن‬ ‫منهن‬ ‫االهم‬
diaphragmatic paralysis
‫هو‬ ‫نعرف‬ test in pediatric ‫نسوي‬
delloved area ‫ال‬ ‫علو‬ ‫ايدنه‬ ‫نخلي‬ ‫من‬
acsesive crying ‫يصير‬
Early sign in
 Approximately one third of hospitalized GBS patients
require mechanical ventilation due to respiratory
muscle or oropharyngeal weakness.
 Tachycardia is common but more serious autonomic
nervous system dysfunction may occur, including life-
threatening arrhythmias, hypotension, hypertension,
and gastrointestinal dysmotility. nitroprusside
* Course :
1-Regressive in more than 90% of cases.
Recovery begins in 2-3 weeks in a descending
manner. Most pt. regain full muscle strength, some
left with residual weakness.
2- Progressive in few cases. Bulber
involvement leads to dysphagia and aspiration.
Involvement of respiratory muscles may lead to
respiratory failure.
3- A chronic relapsing form exist in 7% of the
cases: Weakness recurs intermittently or does not
improve over periods of months or yrs.
‫يتكرر‬
Slow
Diagnosis :-
a- History of antecedent infection, or injection of
sera may be of diagnostic help.
b-Clinical examination of paralytic
muscles.areflexai
c- CSF examination : Characterized by the presence
of Protein cell Dissociation ( Froin syndrome) i.e. there is
an increase in proteins without corresponding increase in
cells; However, protein cell dissociation is not Path
genomic of Guillian Barre since it may occur in other
disease as :
- Poliomyelitis; Infratentorial tumors; venous sinus
thrombosis ; lead poisoning; Spinal cord compression;
Diphtheria.
d- Nerve conduction velocity is decreased (
Diagnostic Investigation).
14 days.
‫واحد‬ ‫اهم‬
‫اضافات‬ ‫بعد‬ ‫اكو‬
DIFFERENTIAL DIAGNOSIS
(AFP)
 Cerebral causes : encephalitis,bilateral strokes,acute cerebellar
ataxia syndrome posterior fossa structural lesion
 Spinal cord lesion compression, transverse myelitis, vascular
injury)
 AH lesion e.g. poliomyelitis and SMA
 Peripheral neuropathy Chronic inflammatory demyelinating
polyneuropathy , (HIV) , Neurotoxic fish or shellfish poisoning
,Paraneoplastic neuropathy,Porphyria polyneuropathy, Toxic
neuropathies ( arsenic, thallium, organophosphates, lead)
Vasculitic neuropathies, Vitamin deficiency (B-12, folate,
thiamine)
 Motor end plate disease Myasthenia Gravis, Tick paralysis
 Muscle disease Myopathy hypokaemia and myositis
 Conversion disorder/hysterical paralysis
Treatment :
1- Hospitalization: The pt should be admitted once
diagnosed, even if general condition is good;pt may need
mechanical ventilation at any time.
2- Care of feeding and avoidance of complications of being
bed ridden as bed sores.
3- Specific therapy : One of the following or both:
a- Intravenous Immunoglobulin's: 0.4 mg/kg /day over 5
days.( total dose = 2g).
b- Plasma Exchange ( Plasmapherisis): 5 exchanges of 50
ml/kg over 7-10 days.
4- Physiotherapy after recovery of the respiratory muscles.
chart ‫نسويله‬ admitted ‫نسويلهم‬ ‫من‬
vita sign ‫ال‬ ‫بيها‬
HR RR PB
Progress of disease
Spo2
Tenderness + numbness
Automatic nerve involvement ; tachycardia ,
bradycardia
Early sign of diaphragmatic involvement
+ difficult to feeding ‫يصير‬
‫يحتاجون‬ ‫كلهم‬ ‫مو‬
‫ال‬ ‫بس‬ admitted
agressive type
immunoglobulin
mainly IGG ‫لكن‬ ‫كلهن‬
Monoclono antibody
‫بس‬ ‫واحد‬ immunoglobulin ‫اذا‬
Steroid use
‫يصير‬ ‫من‬
‫نسوي‬ ‫حتى‬
dose‫ال‬ ‫مطلوب‬
ED MANAGEMENT
Intubation
if there is hypoxia ,rapidly declining respiratory function,
poor or weak cough reflex , and suspected aspiration.
Typically, intubation is indicated when the forced vital
capacity (FVC) is less than 15 mL/kg. .
 Dysautomia
1. hypertension short acting b blocker or nitroprusside
2. hypotension-- ivf and supine position
3. bradycardia --- atropine
4. antiarrhythmic drugs or cardioversion may be indicated
ICU ‫يحتاج‬ ‫ملن‬ mechanical inventelation
sign ‫ال‬ ‫نفس‬ ‫خالل‬ ‫من‬ ‫نعرف‬
pulmonary ‫هي‬ ‫الثانيه‬ indication ‫وال‬
function test
Force vital capacity ‫ال‬ ‫اذا‬
ml ١٥ ‫من‬ ‫اقل‬
emergency or ICU ‫بال‬ ‫نعالجه‬
according severity ‫ال‬ ‫حسب‬ ‫هذا‬
autonomic nerve ‫الن‬
‫يصيرون‬ ‫فممكن‬ involvement
arrhythmia
ICU MANAGEMENT
1-Respiratory support
 Respiratory support: serial assessment of ventilatory
status is needed, including measurements of vital
capacity and pulse oximetric monitoring.
 Respiratory assistance should be considered when the
expiratory vital capacity decreases to less than 18
mL/kg or when a decrease in oxygen saturation is
noted (arterial PO2< 70 mm Hg).
 Trachesotomy may be required in a patient with
prolonged respiratory failure, especially if mechanical
ventilation is required for more than 2 weeks.
2-Management Of
Dyautonomia
 Close monitoring of heart rate, blood pressure, and
cardiac arrhythmias allows early detection of life-
threatening situations. Critically ill patients require
close medical supervision in an ICU setting.
 Antihypertensives and vasoactive drugs should be
used with caution in patients with autonomic
instability. Hemodynamic changes related to
autonomic dysfunction are usually transitory, and
patients rarely require long-term medications to treat
blood pressure or cardiac problems.
All joint involved except
one joint is elbow joint
usually 80%
part ‫اول‬ healing ‫ال‬
‫هو‬ involves t healing
involved ‫بي‬ ‫صار‬ ‫واحد‬ ‫اخر‬
‫اول‬ ‫هو‬ ‫يتشافى‬ ‫واحد‬ ‫واخر‬
‫تاثر‬ ‫مفصل‬
Hypertension , hypotension
Nitroglerride
‫بال‬ HTN ‫لل‬ ‫نستخدمه‬ ‫الي‬
ER ‫بال‬ pediatric

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guillian barre.pdf

  • 1. Guillian- Barre Syndrome Acute ascending flaccid paralysis Assisstant Professor Dr.Ahmed Abd Ahdi
  • 2.  In 1859 The first cases were described by Landry, who noted that the disease could produce motor and sensory engagement, which committed the distal portion of extremities and in some cases progressed in caudocephalic or ascending direction to generalized involvement .  In 1916, Guillain and Barre emphasized the importance of albumin-cytological dissociation for clinical diagnosis ‫اكدو‬
  • 3. - It is the most frequent form of polyneuritis seen in pediatric it’s etiology is unknown.  - It is thought to be due to viral infection as it may follow measles, chicken box and mumps( Post infectious polyneuritis).  Roughly two thirds of patients have a history of an antecedent GI or RT infection.  Pathogenic triggers of GBS include EBV, CMV, the enteroviruses, hepatitis A and B, varicella M.pneumoniae and Campylobacter jejuni, which is perhaps the most common  These pathogens are believed to activate CD4+ helper-inducer T cells, which are particularly important mediators of disease -The condition may follow vaccination ( as rabies vaccine) or serum sickness. *Pathology : There is demyelination of multiple peripheral nerves which is maximal where the anterior and posterior roots fuse, the damage is mainly motor. ‫االتحاد‬ ‫منطقة‬ ‫عند‬ ‫شيء‬ ‫اكثر‬ Brain; meningitis encephalitis Spinal cord: infection abcess truma Anterior horn: Nerve injury muscle Muscle junction Muscle: myopathy hypokalemia myositis hoshion syndrome Nerve Lower limp weakness Parstgesia in lower limp ‫الطوارئ‬ ‫مال‬ presentation‫ال‬ Usually in motor nerve
  • 4. Clinical Picture:  1- Most often an unremarkable infection, such as upper respiratory infection, often predates the onset of GBS by 14 days.  Many antecedent infections have been identified, including Campylobacter jejuni, cytomegalovirus (CMV), Mycoplasma pneumonia, Epstein–Barr virus, influenza virus, JEV.  Surgery, immunization, and parturition have also been associated with GBS 2- The symptom may occur insidiously one week or more after acute infection of respiratory or GIT and consists of: - Motor manifestations (predominant): Bilateral symmetrical weakness which begins usually in the lower limbs and progresses to involve the trunk, upper limb and the bulbar muscles ( ascending paralysis). - Sensory manifestations : tender muscle and paraesthesia in some cases. - autonomic manifestations: in some cases as transient urinary incontinence or retention postural hypotension or hypertension. Japanese encephalitis ‫ماضي‬ ‫الوالدة‬ ‫مخاض‬ Tachycardia, bradycardia
  • 5.  Neurological examination is characterized by distal and often proximal, relatively symmetrical, weakness.  Although GBS is essentially a motor neuropathy, sensory dysfunction is seen in a few patients. It is seen more in a demyelinating form of GBS.  Sensory examination is often normal in the early phase of disease. Facial or pharyngeal weakness is commonly seen in GBS.  Diaphragmatic weakness due to phrenic nerve involvement is also common. Examination Bilateral symmetrical weakness Usually anreflexia Sign of autonmic nerve ( tachycardia , bradycardia , Paralysis Cranial nerve involves ‫كلهن‬ ‫منهن‬ ‫االهم‬ diaphragmatic paralysis ‫هو‬ ‫نعرف‬ test in pediatric ‫نسوي‬ delloved area ‫ال‬ ‫علو‬ ‫ايدنه‬ ‫نخلي‬ ‫من‬ acsesive crying ‫يصير‬ Early sign in
  • 6.  Approximately one third of hospitalized GBS patients require mechanical ventilation due to respiratory muscle or oropharyngeal weakness.  Tachycardia is common but more serious autonomic nervous system dysfunction may occur, including life- threatening arrhythmias, hypotension, hypertension, and gastrointestinal dysmotility. nitroprusside
  • 7. * Course : 1-Regressive in more than 90% of cases. Recovery begins in 2-3 weeks in a descending manner. Most pt. regain full muscle strength, some left with residual weakness. 2- Progressive in few cases. Bulber involvement leads to dysphagia and aspiration. Involvement of respiratory muscles may lead to respiratory failure. 3- A chronic relapsing form exist in 7% of the cases: Weakness recurs intermittently or does not improve over periods of months or yrs. ‫يتكرر‬ Slow
  • 8. Diagnosis :- a- History of antecedent infection, or injection of sera may be of diagnostic help. b-Clinical examination of paralytic muscles.areflexai c- CSF examination : Characterized by the presence of Protein cell Dissociation ( Froin syndrome) i.e. there is an increase in proteins without corresponding increase in cells; However, protein cell dissociation is not Path genomic of Guillian Barre since it may occur in other disease as : - Poliomyelitis; Infratentorial tumors; venous sinus thrombosis ; lead poisoning; Spinal cord compression; Diphtheria. d- Nerve conduction velocity is decreased ( Diagnostic Investigation). 14 days. ‫واحد‬ ‫اهم‬ ‫اضافات‬ ‫بعد‬ ‫اكو‬
  • 9. DIFFERENTIAL DIAGNOSIS (AFP)  Cerebral causes : encephalitis,bilateral strokes,acute cerebellar ataxia syndrome posterior fossa structural lesion  Spinal cord lesion compression, transverse myelitis, vascular injury)  AH lesion e.g. poliomyelitis and SMA  Peripheral neuropathy Chronic inflammatory demyelinating polyneuropathy , (HIV) , Neurotoxic fish or shellfish poisoning ,Paraneoplastic neuropathy,Porphyria polyneuropathy, Toxic neuropathies ( arsenic, thallium, organophosphates, lead) Vasculitic neuropathies, Vitamin deficiency (B-12, folate, thiamine)  Motor end plate disease Myasthenia Gravis, Tick paralysis  Muscle disease Myopathy hypokaemia and myositis  Conversion disorder/hysterical paralysis
  • 10. Treatment : 1- Hospitalization: The pt should be admitted once diagnosed, even if general condition is good;pt may need mechanical ventilation at any time. 2- Care of feeding and avoidance of complications of being bed ridden as bed sores. 3- Specific therapy : One of the following or both: a- Intravenous Immunoglobulin's: 0.4 mg/kg /day over 5 days.( total dose = 2g). b- Plasma Exchange ( Plasmapherisis): 5 exchanges of 50 ml/kg over 7-10 days. 4- Physiotherapy after recovery of the respiratory muscles. chart ‫نسويله‬ admitted ‫نسويلهم‬ ‫من‬ vita sign ‫ال‬ ‫بيها‬ HR RR PB Progress of disease Spo2 Tenderness + numbness Automatic nerve involvement ; tachycardia , bradycardia Early sign of diaphragmatic involvement + difficult to feeding ‫يصير‬ ‫يحتاجون‬ ‫كلهم‬ ‫مو‬ ‫ال‬ ‫بس‬ admitted agressive type immunoglobulin mainly IGG ‫لكن‬ ‫كلهن‬ Monoclono antibody ‫بس‬ ‫واحد‬ immunoglobulin ‫اذا‬ Steroid use ‫يصير‬ ‫من‬ ‫نسوي‬ ‫حتى‬ dose‫ال‬ ‫مطلوب‬
  • 11. ED MANAGEMENT Intubation if there is hypoxia ,rapidly declining respiratory function, poor or weak cough reflex , and suspected aspiration. Typically, intubation is indicated when the forced vital capacity (FVC) is less than 15 mL/kg. .  Dysautomia 1. hypertension short acting b blocker or nitroprusside 2. hypotension-- ivf and supine position 3. bradycardia --- atropine 4. antiarrhythmic drugs or cardioversion may be indicated ICU ‫يحتاج‬ ‫ملن‬ mechanical inventelation sign ‫ال‬ ‫نفس‬ ‫خالل‬ ‫من‬ ‫نعرف‬ pulmonary ‫هي‬ ‫الثانيه‬ indication ‫وال‬ function test Force vital capacity ‫ال‬ ‫اذا‬ ml ١٥ ‫من‬ ‫اقل‬ emergency or ICU ‫بال‬ ‫نعالجه‬ according severity ‫ال‬ ‫حسب‬ ‫هذا‬ autonomic nerve ‫الن‬ ‫يصيرون‬ ‫فممكن‬ involvement arrhythmia
  • 12. ICU MANAGEMENT 1-Respiratory support  Respiratory support: serial assessment of ventilatory status is needed, including measurements of vital capacity and pulse oximetric monitoring.  Respiratory assistance should be considered when the expiratory vital capacity decreases to less than 18 mL/kg or when a decrease in oxygen saturation is noted (arterial PO2< 70 mm Hg).  Trachesotomy may be required in a patient with prolonged respiratory failure, especially if mechanical ventilation is required for more than 2 weeks.
  • 13. 2-Management Of Dyautonomia  Close monitoring of heart rate, blood pressure, and cardiac arrhythmias allows early detection of life- threatening situations. Critically ill patients require close medical supervision in an ICU setting.  Antihypertensives and vasoactive drugs should be used with caution in patients with autonomic instability. Hemodynamic changes related to autonomic dysfunction are usually transitory, and patients rarely require long-term medications to treat blood pressure or cardiac problems. All joint involved except one joint is elbow joint usually 80% part ‫اول‬ healing ‫ال‬ ‫هو‬ involves t healing involved ‫بي‬ ‫صار‬ ‫واحد‬ ‫اخر‬ ‫اول‬ ‫هو‬ ‫يتشافى‬ ‫واحد‬ ‫واخر‬ ‫تاثر‬ ‫مفصل‬ Hypertension , hypotension Nitroglerride ‫بال‬ HTN ‫لل‬ ‫نستخدمه‬ ‫الي‬ ER ‫بال‬ pediatric