Definition

acquired disorder of the peripheral nerve, progressive, symmetrical, usually ascending weakness

Described best as a polyradiculoneuropathy

Guillain-Barr syndrome (GBS) is a rare autoimmune neurological disease in which the bodys immune system produces antibodies against its own nerves, resulting in damage to them.

Epidemiology
13/100 000 population according to epidemiological studies from Europe, USA, and Australia

male-to-female ratio of 1.5:1

incidence in children is lower, with estimates between 0.4 and 1.3 cases per 100,000 per year

GBS can occur at any age but is rare in children under the age of 2 years

Etiology
Post-infectious
immune-mediated disease targeting peripheral nerves virulence of C jejuni is thought to result from the presence of specific antigens in its capsule that are shared with nerves CMV infections present as upper respiratory tract infections, pneumonias, and nonspecific flulike illnesses

Administration of certain vaccinations

Clinical manifestation
evolving areflexic motor paralysis with or without sensory disturbance

bulbar weakness with difficulty handling secretions and maintaining an airway

Autonomic involvement fluctuation in blood pressure, postural hypotension, and cardiac dysraythmias

Subtypes of GBS
Acute inflammatory demyelinating polyradiculoneuropathy
commonest type of Guillain-Barr syndrome lymphocytic infiltration of the peripheral nerves and macrophage mediated segmental demyelination

Acute motor axonal neuropathy

Wallerian-like degeneration of motor axons exclusively rapidly progressive weakness, often with respiratory failure and usually good recovery. muscle action potential amplitudes are reduced

Acute motor sensory axonal neuropathy

axonal degeneration with no demyelination or inflammation

Miller fisher syndrome

ataxia, areflexia, and ophthalmoplegia Anti-GQ1b antibodies were found oculomotor nerve contained the highest concentration of GQ1b gangliosides conduction block is the most likely mechanism of ophthalmoplegia.

Pathogenesis

classic pathological findings in acute inflammatory

IgG and activated complement bind to the axolemma of motor fibers at the nodes of Ranvier, followed by formation of the membrane-attack complex. nodal lengthening is followed by axonal degeneration of motor fibers with neither lymphocytic inflammation nor demyelination

Follow up

January, 10th 2013

S o

A P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 22222/222222 22222/22222 Respiratory failure ec Guillain-Barr Syndrome with unstable respiratory system, infection and musculoskeletal system Management: T-piece attached O2 8L/I SpO2 92-94% IVFD D5% NaCl 0,9% Inj Ranitidine 25 mg/12 hr/IV Inj Meropenem 800 mg/12 hr/ IV (day 15th) Inj Farmadol 220 mg/ IV (if needed) Inj dexamethasone 11 mg/8hr/IV Ambroxol syr 3 x 5cc Zamel syr 1 x cth Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr CVP/6hr Chest physiotherapy Diagnostic Planning: Lumbal puncture Check albumin, T3, T4, TSH

Blood Gas Analysis pH pCO2 pO2 Bicarbonat Total CO2 Base Excess O2 Saturation

Result 7.459 32.0 134.7 22.2 23.2 -1.3 99.1

Unit

Referral 7.35-7.45

mmHg mmHg mmol/L mmol/L mmol/L %

38-42 85-100 22-26 19-25 (-2) - (+2) 95 100

January, 11th 2013

S o

A P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 22222/222222 22222/22222 Respiratory failure ec Guillain-Barr Syndrome with unstable respiratory system, infection and musculoskeletal system Management: T-piece attached O2 8L/I SpO2 92-94% IVFD D5% NaCl 0,9% + Ca. glukonas 10 mEq 20 gtt/I micro Inj Ranitidine 25 mg/12 hr/IV Inj Meropenem 800 mg/12 hr/ IV (day 15th) Inj Farmadol 220 mg/ IV (if needed) Inj dexamethasone 11 mg/8hr/IV Ambroxol syr 3 x 5cc Zamel syr 1 x cth Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr CVP/6hr Chest physiotherapy

Lumbar puncture Color LDH Total protein Total leucocyte Total erythrocyte Glucose pH MN cell PMN cell

Result Serous 31 36.00 0.001 0.000 96 7.5 100.0 0.0

Unit

Referral Serous

U/L mg/dL 10/uL 10/uL mg/dL

< 200 < 45 <3

40 76 78

% %

Result immunoserology Total T3: 0,60 ng/ml (0,8-2) Total T4: 8,63 g/dl (5-14) TSH: 0,478 IU/ml (0,27-4,2)

January, 12th 2013 and January, 13th 2013

S o

A P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 22222/222222 22222/22222 Respiratory failure ec Guillain-Barr Syndrome with unstable respiratory system, infection and musculoskeletal system Management: T-piece attached O2 8L/I SpO2 92-94% IVFD D5% NaCl 0,9% Inj Ranitidine 25 mg/12 hr/IV Inj Meropenem 800 mg/12 hr/ IV (day 15th) Inj Farmadol 220 mg/ IV (if needed) Inj dexamethasone 11 mg/8hr/IV Ambroxol syr 3 x 5cc Zamel syr 1 x cth Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr CVP/6hr Chest physiotherapy

January, 14th 2013

S o

A P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 22222/222222 22222/22222 Respiratory failure ec Guillain-Barr Syndrome with unstable respiratory system, infection and musculoskeletal system Management: IVFD D5% NaCl 0,9% Inj ceftazidine 500 mg/8hr/IV (day 1st) Inj Farmadol 220 mg/ IV (if needed) Ambroxol syr 3 x 5cc Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr Diagnostic Planning: Check electrolite

January, 15th 2013

S o

A P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 22222/222222 22222/22222 Guillain-Barr Syndrome Management: IVFD D5% NaCl 0,9% Inj ceftazidine 500 mg/8hr/IV (day 1st) Inj Farmadol 220 mg/ IV (if needed) Ambroxol syr 3 x 5cc Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr Result of electrolyte: Ca: 7,7 mg/dl Na: 132 mEq/l K: 3,6 mEq/l

January, 16th 2013 and January, 17th 2013

S o

A
P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 33333/33333 22222/22222 Guillain-Barr Syndrome Management: IVFD D5% NaCl 0,9% Inj ceftazidine 500 mg/8hr/IV (day 1st) Inj Farmadol 220 mg/ IV (if needed) Ambroxol syr 3 x 5cc Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr

January, 18th 2013 - January, 20 th 2013

S o

A
P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 33333/44444 22222/22222 Guillain-Barr Syndrome Management: IVFD D5% NaCl 0,9% Inj ceftazidine 500 mg/8hr/IV (day 1st) Ambroxol syr 3 x 5cc Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr

January, 21st 2013 - January, 22nd 2013

S o

A
P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 44444/44444 22222/22222 Guillain-Barr Syndrome Management: IVFD D5% NaCl 0,9% Inj ceftazidine 500 mg/8hr/IV (day 9th) Ambroxol syr 3 x 5cc Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr Physiotheraphy Diagnostic Planning: Check blood routine, electrolyte, LFT, RFT, glucose ad random

Complete Blood Count Hb WBC RBC Hematocrite PLT MCV MCH MCHC RDW MPV PCT PDW Neutrophil Lymphocyte Monocyte Eosinophil Basophil Liver Total bilirubin Direct bilirubin AST ALT Kidney Ureum Creatinine Uric acid Electrolyte Sodium Calcium Pottasium Phosphor Chloride Magnesium

Result 10.40 8.33 3.97 31.90 387 80.40 26.20 32.60 17.00 8.80 0.34 9.0 44.70 39.50 13.90 1.3 0.6 Result 0.34 0.09 26 18 Result 13.10 0.24 2.9 Result 133 9.2 4.1 6.2 113 2.35

Unit gr% x 103/mm x 106/mm % x 10/mm fL Pg g% % fL

Referral 11.3 14.1 4.5 13.5 4.40 4.48 37 41 150 450 81 95 25 29 29 31 11.6 14.8 7.0 10.2 37 80 20 40 28 16 01 Referral <1 0 0.2 < 38 < 41 Refferal < 50 0.32 0.59 < 7.0 Referral 135-155 9.2 11.0 3.5-5.5 3.4 6.2 96-106 1.4 1.9

% % % % % Unit mg/dL mg/dL U/L U/L Unit mg/dL mg/dL mg/dL Unit mEq/L mg/dL mEq/L mEq/L mEq/L mEq/L

January, 23rd 2013 - January, 25th 2013

S o

A
P

Weakness of both upper and lower extremities (+), fever (-) CNS Stable, sens: compos mentis, Eye: isochoric pupil, light reflex (+/+), 2 mm Cardiovascular Stable, HR: 118 bpm, regular, murmur (-), CRT < 3, MAP: 93 mmHg, adequate p/v, warm acral Respiratory Unstable, thorax: simetris fusiformis, epigastrial retraction (-), ETT attached, RR: 22x/i, Tpiece O2 8L/I, SpO2 92-94% Infection Ustable, fever (-), temp: 37,1C, leukocyte: 14.950/mm3 culture bacillus aureus (+) and providential retgen Gastrointestinal Stable, soepel, normoperistaltic, clear NGT Hematology Unstable, Hb/Ht/L/Tr: 10,4/31,8/14.950/213000 Musculoskeletal Stable, edema (-) physiology reflex: APR/KPR: +/+ pathology reflex: (-) muscle strength: 44444/44444 22222/22222 Guillain-Barr Syndrome Management: IVFD D5% NaCl 0,9% Inj ceftazidine 500 mg/8hr/IV (day 9th) Ambroxol syr 3 x 5cc Nebule NaCl 0,9% 2,5cc/8hr Diet 70 cc/kgBW/day 190 cc/3hr Physiotheraphy

 Patient was discharged from hospital on 25th January 2013 because his condition of stability was improved. NGT was changed. Family patient has been educated regarding to the method of using the NGT at home. Patient has been told to be controlled at the policlinic of neurology pediatric and get the physiotherapy once per week.