An Interesting Case of Guillain-Barre Syndrome

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Information about An Interesting Case of Guillain-Barre Syndrome
Health & Medicine

Published on March 11, 2014

Author: mahmoodi2000



PEDIATRIC SEMINAR Seyed Morteza 2005M29

CASE 1 History  14-year-old girl, 10-day history of progressive weakness  3 weeks before, rhinorrhea, cough, malaise  8 days after, lower-extremity weakness and difficulty walking  Diffuse muscle pain and progressive weakness that extended to upper extremities 3 days before this, developed a hoarse voice and SOB Difficulty urinating and decreased oral intake No fever, cough, vomiting, diarrhea

EXAMINATION  Afebrile, ill-appearing  Heart rate: 118 bpm  Respiratory rate: 28 breaths/min  Blood pressure: 168/122 mm Hg  Oxygen saturation: 93% at room air  Lungs auscultation: diffuse, poor aeration  Heart sounds: normal, no murmur

NEUROLOGIAL EXAM.  Muscle strength symmetric but diminished  Lower extremities: 2/5, upper extremities: 4/5  Sensation intact to light touch, loss of vibratory sense  Lower extremities: No DTR, upper extremities: diminished (1+), absent plantar reflexes  Cranial nerves II-XII intact; weak cough and gag reflex, impaired handling of secretions.  The remainder of her examination is unremarkable.


INVESTIGATIONS  CBC and a basic metabolic panel: within normal limits  LP: opening pressure of 15 cm H20  CSF analysis:  2 WBC/HPF  4 RBC/HPF  No organisms  Protein 96 mg/dL (960 mg/L)  Glucose 72 mg/dL (3.99 mmol/L)  MRI brain and spine  Transported to the ICU, Intubated

DIAGNOSIS Spinal epidural abscess Guillian-Barre syndrome Transverse myelitits Multiple sclerosis

DIAGNOSIS Spinal epidural abscess Guillian-Barre syndrome Transverse myelitits Multiple sclerosis

Introduction Pathophysiology Epidemiology Diffrential diagnosis Investigations Treatment

Georges Guillain Jean-Alexandre Barré

Revue Neurologique 1916


Two types of reactions are seen in peripheral nerve injury Injury of the neuron and its axon leads to axonal degeneration, a. neurone cell body (neuropathy) b.or its axon (axonopathy) •Dysfunction of the Schwann cell or damage to myelin sheath leads to a loss of myelin lead to (segmental demyelination).

Epidemiology  Frequency  Age  Race  Sex  Mortality and morbidity  Recurrence


Clinical Picture  Weakness  Reduced or absent reflexes  Sensory disturbance  Pain  Cranial nerve involvement  Respiratory dysfunction  Dysautonomia

 CSF analysis  Opening pressures  WBC  Protein  Albuminocytologic dissociation  MRI  Vitals  Vital capacity / arterial O2 pressure

VARIANTS  Miller Fisher syndrome (MFS)  Acute motor axonal neuropathy (AMAN)  Acute motor sensory axonal neuropathy (AMSAN)  Acute panautonomic neuropathy  Bickerstaff’s brainstem encephalitis (BBE),

Miller Fisher syndrome (MFS)  Classic triad of ophthalmoplegia, ataxia, and areflexia  Diplopia usually initial symptom, followed by limb or gait ataxia  Descending paralysis  Elevated antibodies to GQ1b


Serum anti-ganglioside Antibodies Anti-GM1, GM1b, GD1a, GalNAc-GDIa

TREATMENT  Supportive Care  Ventilatory Support  Autonomic dysfunction  Noscomial infections  Venous thromobosis  Nutritional support  Immune therapy  Plasma exchange (PE)  Intravenous immunoglobulin (IVIg)  Corticosteroids

Prognosis  Nadir of clinical deficits at 4 weeks  5-20% have fulminant course  Residual deficit  Recovery at 1 year follow-up: 62% had recovered completely, 14% could walk but not run, 9% could not walk without assistance, 4% remained bed bound or ventilated, 8% died

CORTICO-SPINAL Tracts (Pyramidal T.)

Anterior Horn Cell Autosomal recessive inheritance mutation on chromosome 5q13 S---- M------- A------

Anterior Horn Cell Type 1 (Werdning- Hoffman disease) • Clinically apparent at birth • Tongue fasciculations, floppiness, absent tendon reflexes and gen. muscle atrophy, • Recurrent chest infections and death before the age of 2 yrs. due to resp. failure. Spinal Muscular Atrophy

Anterior Horn Cell Poliomyelitis

Peripheral Nerve  Guillain-Barré syndrome  Tick paralysis  Hereditary  Vitamin E, B12, and B1 deficiencies  Toxins Lead, thallium, arsenic, mercury, Hexane Acrylamide, Organophosphates  Diphtheria  Collagen vascular disease  Porphyria  Paraneoplastic  Drugs Amitriptyline Dapsone Hydralazine Isoniazid Nitrofurantoin Vincristine

Neuromuscular Junction Myasthenia gravis

Neuromuscular Junction Botulism

Muscle Dystrophy

 Muscle dystrophies  Myotonic myopathies  Inflammatory myopathies  Toxic myopathies  Metabolic myopathies  Endocrine myopathies

CASE 2  Six-year-old girl, unable to walk without assistance for few hours  Tingling sensation in her finger toes yesterday  Six hours later stagger and fall  Next day unable to walk

PHYSICAL EXAMINATION  Alert, afebrile  Truncal instability, wide-based, ataxic gait  Neurological examination:  Muscle strength in the legs and arms 4/5  Dysmetria on finger-to-nose testing  DTR diminished at the left knee, normal elsewhere  Sensations and cranial nerves normal, as was the rectal-sphincter tone

INVESTIGATIONS  Chest x-ray  Routine laboratory studies  Tests for toxic substances  Stool culture  MRI head and neck no abnormalities  CSF analysis:  Protein 29 mg/dl  Glucose 71 mg/dl (3.9 mmol per liter)  Lymphocyte count of 2 per cubic millimeter.  Gram's staining and culture negative  Nerve-conduction studies

COURSE IN HOSPITAL  48 hours later lethargic and irritable  Symmetric leg weakness increased, with muscle strength of 3/5.  DTR at the knees and ankles were absent  Due to ascending paralysis and hypoventilation, transferred to the ICU for intubation  By 72 hours, motor strength 2/5  Listlessness, slurred speech, and bilateral ptosis


 Spinal cord lesions  Guillian-Barre syndrome  Poliomyelitis  Tick paralysis  Botulism

 Spinal cord lesions  Guillian-Barre syndrome  Poliomyelitis  Tick paralysis  Botulism



Neural Tube Defects  Spina bifida oculta  Meningocele  Meningomyelocele  Encephalocele  Syringomyelia  Dermal sinus  Thered cord  Anencephaly  Diestematomyelia

Neuronal Migration Disorder  Lissencephaly  Schizencephaly  Porencephaly  Holoprosencephaly  Pachigyria  Micropolygyria

 Agenesis of corpus callosun  Agenesis of cranial nerves




Cranial Anomalies

Arnold chiary malformation

b w N S Generalized Seizures Encephalopathic Coma with or without Seizures All disorders that cause hypoglycemia Maple syrup urine disease Most hepatic glycogen storage diseases Nonketotic hyperglycinemia Galactosemia, hereditary fructose intolerance Diseases producing extreme hyperammonemia Fructose-1,6-bisphosphatase deficiency Disorders of the urea cycle Disorders of the propionate pathway Disorders of the propionate pathway HMG-lyase deficiency Disorders of beta oxidation Disorders of beta oxidation Congenital lactic acidosis (PCD) Pyruvate carboxylase deficiency (PCD) Maple syrup urine disease

Blood and Plasma Urine Arterial blood gas Glucose Electrolytes-anion gap pH Glucose Ketones Ammonia Reducing substances Liver enzymes Organic acids Complete blood count, differential,† and platelet count Acylcarnitine Orotic acid Lactate, pyruvate Organic acids Amino acids Carnitine

Disease Affected Enzyme Organs Affected Clinical Syndrome Neonatal Manifestations Type I: von Gierke Glucose-6- phosphatase Liver, kidney, GI tract, platelets Hypoglycemia, lactic acidosis, ketosis, hepatomegaly, hypotonia, slow growth, diarrhea, bleeding disorder, gout, hypertriglyceridemia, xanthomas Hypoglycemia, lactic acidemia, liver may not be enlarged Type II: Pompe Lysosomal α- glucosidase All, notably striated muscle, nerve cells Symmetric profound muscle weakness, cardiomegaly, heart failure, shortened P-R interval May have muscle akness, cardiomegaly, or both Type III: Forbes Debranching enzyme Liver, muscles Early in course hypoglycemia, ketonuria, hepatomegaly that resolves with age; may show muscle fatigue Usually none Type IV: Andersen Branching enzyme Liver, other tissues Hepatic cirrhosis beginning at several months of age; early liver failure Usually none Type V: McArdle Muscle phosphorylase Muscle Muscle fatigue beginning in adolescence None Type VI: Hers Liver phosphorylase Liver Mild hypoglycemia with hepatomegaly, ketonuria Usually none Type VII: Tarui Muscle phosphofructok inase Muscle Clinical findings similar to type V None Type VIII Phosphorylase kinase Liver Clinical findings similar to type III, without myopathy None

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