Pediatric Guillain-Barré Syndrome

Introduction to Pediatric Geeland-Barre Syndrome Guillain-Barrésyndrome (GBS) is a multiplicity of nerve roots and peripheral neuropathy associated with infection and/or autoimmunity, with progressive, symmetrical and flaccid limb paralysis feature. Also known as acute infectious polyradiculoneuritis, also known as acute inflammatory demyelinating polyradiculoneuritis (acuteinflamatory demingeling polyradiculoneuritis). At present, GBS is considered to be an acute autoimmune disease mediated by both humoral and cellular immunity. It can occur at any age. In most cases, most of the children can recover gradually after a short period of quiescent period, and the prognosis is good. Different degrees of peripheral sensory disturbances, medullary lesions and respiratory muscle paralysis occur in severe cases. Cerebrospinal fluid changes to protein-cell separation. Treatment mainly includes general treatment and immunotherapy. Most of the children gradually recover after a short period of quiescent period, and the prognosis is good. basic knowledge Sickness ratio: 0.0001% Susceptible people: children Mode of infection: non-infectious Complications: diarrhea

The cause of pediatric Guillain-Barre syndrome

(1) Causes of the disease

The cause is unclear, but studies have shown that Campylobacter jejuni (4% to 66%), cytomegalovirus (5% to 15%), Epstein-Barr virus (2% to 10%), Mycoplasma pneumoniae (1% to 5%), these There is no specific correlation between pre-infection and clinical subtypes. In addition, the literature reports with herpes simplex and herpes zoster virus, influenza A and B, mumps, measles, coxsack, hepatitis A and hepatitis B virus. Smallpox is associated with infections such as human immunodeficiency virus.

(two) pathogenesis

Pathogenesis

The pathogenesis of GBS is still not very clear, mainly the following:

(1) Infection: Most patients with GBS have pre-infection, but severe axonal degeneration is more common after infection with Campylobacter jejuni, and severe sensory damage is more common after cytomegalovirus infection. At present, the correlation between Campylobacter jejuni and GBS has caused widespread concern. Campylobacter jejuni (CJ) is the main cause of acute gastroenteritis and the most common source of GBS pre-infection, by different CJ serotypes: 0:1, 0:2, 0:4, 0:10, Chemical analysis of the core oligosaccharides (OS) of lipopolysaccharides at 0:19, 0:23, 0:36 and 0:41 showed similar structures to human gangliosides GML, GDLa, GDa, GD3 and GM2.

Certain structures of microorganisms share a common epitope with certain structures of the host. The protective immune response against pathogenic microorganisms after infection causes cross-reaction in nerve tissue, destroying nerve structure function or causing functional changes. This is called "molecular simulation". In addition, microbes can also act as a polyclonal activator to stimulate B cell proliferation, produce antibodies; directly participate in cytokine release, synergistic immune response; activate oligoclonal reactions of T cells by so-called "microbial superantigens"; destroy immunocompetent cells, Interfering with immune regulation mechanisms, resulting in an autoimmune response.

The incidence of GBS is related to the characteristics of the source of infection and to the immune status of the patient.

(2) Anti-ganglioside antibodies: Many studies have shown that relatively specific anti-ganglioside antibodies can be found in each subtype of GBS, the most typical of which is Miller-Fisher syndrome (MFS), 90% of patients with MFS Anti-GQ1b and GT1a ganglioside antibody (IgG); anti-GML antibody (IgG type) found in all GBS subtypes, but acute motor axonal neuropathy (AMAN) compared with demyelinated GBS Anti-GML antibodies are more common in patients with acute motor-sensory axonal neuropathy (AMSAN).

Whether anti-ganglioside antibodies are directly involved in the pathogenesis is still inconclusive. Many experiments have shown that anti-GML antibodies can cause ion channel dysfunction. An early manifestation of AMAN is that complements on Langfei junctions are activated, and the possible mechanism of action is resistance. Ganglioside antibodies act directly on receptors at the Langue or junction, causing changes in ion channels by activating complement.

(3) Cellular immunity: T cells may be involved in the pathogenesis of GBS in most or all of the subtypes. T cells respond to any of the myelin proteins P2, P0 and PMP22 and are sufficient to elicit experimental autoimmune neuritis. Activated T cells are found in the humoral circulation of patients in the acute phase. It can up-regulate the matrix metalloprotein kinase and recognize it by binding to the antigens of the same family via the blood-nerve barrier. The study of these specific responses to T cells is still in its infancy.

(4) Vaccination: It has been reported that 4.5% of GBS is vaccinated after vaccination, which is more common in influenza vaccine, hepatitis vaccine and measles vaccine.

(5) Genetics: It has been reported that the frequency of A3 and B8 genes in GBS patients is significantly increased.

(6) Trace elements: It has been reported that there are trace elements of zinc, copper and iron metabolism in patients with GBS.

2. Pathological changes

Recent studies have shown that GBS includes many different subtypes, mainly acute inflammatory demyelinating polyradiculoneuritis (AIDP), acute motor axonal neuropathy (AMAN). Acute motor-sensory axonal neuropathy (AMSAN) and Miller-Fisher syndrome (MFS), more than 90% of patients with GBS are AIDP type, and the clinical and pathological characteristics of each subtype are different. However, the most important pathological changes are monocyte infiltration and segmental demyelination in the peripheral nerve.

(1) Acute inflammatory demyelinating polyradiculopathy (AIDP): pathological changes are mainly inflammatory demyelinating changes with focal and diffuse lymphocytic infiltration and a large number of lipid-rich macrophages, exercise And the sensory fiber is involved, the disease mainly involves the nerve root (especially the motor nerve root) and the adjacent nerve plexus. The early visible damage of the myelin nerve fiber is the vacuole-like change of the outer layer of the myelin sheath, but the outer layer of the affected fiber, The phenomenon of complement activation on the surface of Schwann cells appeared earlier, so some scholars speculated that the antibody activates complement by binding to the surface of Schwann cell membrane, and triggers a series of changes with the activation of complement, myelin vacuole Change, disintegrate and be swallowed by macrophages.

(2) Acute motor axonal neuropathy (AMAN): pathological changes are mild, and there is no inflammation. The main changes in nerve fibers are motor axonal degeneration, involving the dorsal and ventral nerve roots and peripheral nerves, immunopathology and electron microscopy. Studies have shown that AMAN's initial immune damage appears on the Langfei knot.

(3) Acute motor-sensation axonal neuropathy (AMSAN): The pathological process is complement activation, macrophages are in contact with the nerve nodes, the space around the axons is opened, and macrophages migrate away; followed by axonal Shrinkage, axonal degeneration can occur in some patients, Langfei knot and sensory nerves are extensively damaged, and these pathological changes are similar to AMAN.

(4) Miller-Fisher syndrome (MFS): There are few reports on its pathological changes, and its pathological changes are generally considered to be similar to AIDP.

Pediatric Guillain-Barre Syndrome Prevention

The cause of this disease is not fully understood. It is believed to be related to viral infection and immune response after infection. Therefore, it is necessary to actively prevent and treat various infectious diseases, such as doing vaccination work, especially the prevention and treatment of respiratory infectious diseases. Active prevention and treatment of Campylobacter jejuni should be carried out.

Pediatric Guillain-Barré syndrome complications Complications, diarrhea

May be secondary to infection.

Pediatric Guillain-Barre Syndrome Symptoms Common Symptoms Sensory Disorders Ataxia Arrhythmia Hearing Drops Sore Throat Consonants Facial Muscles Forceless Reflexes Disappear tachycardia Respiratory Failure

1. Acute inflammatory demyelinating polyradiculopathy (AIDP): More than 90% of GBS patients can affect patients of all ages. This type of symptoms appears faster, often within a few days, and can also be outbreaks. Sexuality, the most common manifestations are progressive, ascending, flaccid paralysis, with mild to moderate sensory disturbances, or with cranial nerve palsy (declined), severe patients can develop medullary paralysis and cause serious complications The most susceptible to the 7th, 9th, and 10th pairs of cranial nerves, followed by 2, 5, 12 pairs of cranial nerves, severe respiratory muscle paralysis within 24 to 48 hours, requiring immediate mechanical ventilation.

Sensory dysfunction includes numbness, sensation, acupuncture, burning sensation, usually no urination or defecation disorder, the autonomic nervous system damage of this disease is common, there may be symptoms of sympathetic and parasympathetic insufficiency, patients often have hands and feet less sweat Or hyperhidrosis, sinus tachycardia, unstable blood pressure, can be transient, urinary retention or incontinence.

The following indicators suggest clinical respiratory failure: rapid disease progression, medullary dysfunction, bilateral lateral muscle weakness, autonomic dysfunction, lung function indicators associated with respiratory failure: vital capacity <20ml / kg maximum inspiratory pressure <30cmH2O, maximum The expiratory pressure <40cmH2O, or the lung capacity, the maximum inspiratory pressure and the maximum expiratory pressure drop by more than 30%.

2. Acute motor axonal neuropathy (AMAN): The clinical manifestations are acute hemorrhoids, without sensory disturbances, and the recovery is slow. Patients often have hyperreflexia in the early recovery period.

3. Acute motor-sensory axonal neuropathy (AMSAN): This type is more common in adults and is a severe axonal destructive subtype characterized by simultaneous impairment of motor and sensory functions, with slower recovery and sensory impairment including Numbness, ant feeling, acupuncture, burning sensation.

4. Miller-Fisher syndrome (MFS): clinical features of different degrees of extraocular muscle paralysis, ataxia and sputum reflex disappeared, MFS is a variant of GBS, for the primary damage of oculomotor nerves, in some Patients may have brain stem or cerebellum directly damaged. Generally, MFS patients rarely involve limb muscle strength, autonomic nerve function, except for cerebral nerves outside the ocular nerve. MFS can still have peripheral and central hearing system and peripheral balance system. Impaired, manifested as hearing loss, balance dysfunction, when patients with medullary paralysis and autonomic dysfunction, may indicate poor prognosis, very few patients can relapse, that is, after a disease, after a long period of asymptomatic, again The appearance of MFS is similar to that of the first time. Some scholars believe that recurrence may be related to HLA-DR2.

5. Pediatric GBS features

(1) Pre-existing symptoms are more common in unexplained fever than diarrhea.

(2) The limbs are more asymmetrical in the upper and lower limbs.

(3) Cranial nerve palsy is rare.

(4) Feeling disorders are rare.

(5) Early muscle atrophy is less than that of adults.

(6) The condition changes rapidly, but the prognosis is better than that of adults.

(7) Cerebrospinal fluid protein-cell separation is not typical in adults.

6. GBS after infection with Campylobacter jejuni (CJ) is mainly characterized by: 1 more serious condition; 2 greater degree of axonal degeneration; 3 worse adverse prognosis; 4 high incidence of children; 5 greater proportion of specific HLA Type 6; more closely linked to anti-ganglioside antibodies and seasonality of onset.

Examination of children's Guillain-Barre syndrome

1. Blood test: More than half of the sick children have neutrophils, and the erythrocyte sedimentation rate is increased in critical cases.

2. Blood gas analysis: Blood gas analysis can understand respiratory function and respiratory acidosis, and blood oxygen saturation should be monitored regularly.

3. Antibody detection: A variety of anti-gangliosides gML, gMa, GDLa, GDLb and GQ1b antibodies can be detected in the serum of GBS patients, which are generally detected by ELISA. Many scholars have whether these antibodies are related to GBS subtypes. In addition, the anti-GQ1b antibody was closely related to MFS, and other GBS clinical subtypes and corresponding specific antibodies were not completely determined. The positive rate of double serum anti-neuroantiage 4 fold increase was about 75%, P2 protein. (Human peripheral neurophospholipid protein) antibody positive has a certain specificity for the diagnosis of this disease.

Antibodies and their possible related GBS subtypes:

(1) Anti-GML antibody: This antibody is present in about 30% of patients with AIDP and is non-specific.

(2) Anti-GDLa antibody: This antibody is specific in Chinese AMAN patients, but its sensitivity is 60% to 70%.

(3) Anti-GQLb antibody: 90%: This antibody is present in patients with MFS.

(4) Anti-GalNAc-GDLa antibody: This antibody is associated with the infection of Campylobacter jejuni. Studies have shown that GBS patients with this antibody can develop rapidly, very severe muscle weakness (mainly distal muscle group), but very There are few feelings of disappearance, abnormal feelings and brain nerve involvement.

(5) Anti-Gla and anti-GMLb antibodies: The presence of this antibody in patients with GBS needs to be alert to the occurrence of medullary paralysis.

4. Cerebrospinal fluid examination: Protein-cell separation is one of the characteristics of this disease. After several days of onset, the protein content begins to rise. The highest peak of protein content is 4-6 weeks after onset. Most patients have normal cell numbers, and the patient's cerebrospinal fluid can be found in the cerebrospinal fluid. Cloning zone.

Electrophysiological examination:

1.AIDP: Demyelinating changes, nerve conduction velocity is significantly slowed down, F wave disappears, some authors believe that the disappearance of H reflex is a more sensitive indicator for early diagnosis of GBS, and the amplitude of upper limb sensory nerve action potential (SNAP) is weakened or disappeared. Abnormal F waves are also an abnormal indicator of early GBS.

2. AMAN: The nerve conduction velocity is normal or slightly abnormal, and the amplitude of the composite motor action potential (CMAP) decreases, suggesting that the axonal cord is damaged, but there is no demyelination.

3.AMSAN: Axle damage is the same as AMAN.

4. MFS: Demyelination changes with AIDP.

Diagnostic identification of children's Guillain-Barre syndrome

Diagnostic criteria

1. Clinical symptoms: In 1996, Nomura et al summarized seven characteristics of GBS, of which the first five are clinical features:

(1) Patients often have pre-infections 1 to 3 weeks before the onset of neurological symptoms, the most common are sore throat, nasal congestion, fever or gastroenteritis caused by Campylobacter jejuni infection.

(2) Symmetrical sputum, generally with the weakness of both lower limbs, gradually increasing and upward development.

(3) The sputum reflection disappears.

(4) Symptoms and signs progress rapidly within a few days to 2 weeks, then enter a stable period, and finally gradually return to normal, which takes about several months.

(5) Most patients can resume function, usually 2 to 4 weeks after the cessation of progress, but also after a few months.

(6) The protein in cerebrospinal fluid is increased, the number of white blood cells is not high, and protein-cell separation is observed.

(7) The motor nerve conduction velocity is slowed down, and the F wave disappears.

2. Diagnostic criteria The new diagnostic criteria revised by Asbury (1990) propose the following conditions for GBS:

(1) Characteristics necessary for diagnosis:

1 More than one limb is progressively weak.

2 reflex loss, but if other features meet the diagnosis, distal sacral reflex loss and biceps tendon reflex and knee reflex reduction can also be diagnosed.

(2) Features that support high diagnostics:

1 clinical features:

A. Progress: Symptoms and signs appear rapidly and stop progressing by 4 weeks.

B. Relatively symmetric.

C. Feel the symptoms and signs are mild.

D. Cranial nerve involvement.

E. It usually recovers 2 to 4 weeks after the cessation of progression, and it does not begin to recover after a few months. Most of the patients return to normal function.

F. Autonomic dysfunction: arrhythmia, orthostatic hypotension, hypertension.

G. There is no fever when the neurological symptoms appear.

H. Variant:

a. fever occurs when neurological symptoms occur.

b. Severe sensory disturbance with pain.

c. Progress over 4 weeks, some patients may have a slight iteration.

d. Progress ceases but does not restore or leave a permanent functional defect.

e. Sphincter disorders, usually the sphincters are not tired, but have a transient bladder sphincter disorder at the onset of the disease.

f. Central nervous system involvement occasionally occurs.

Includes severe ataxia, dysarthria, extensor and foot reflexes, and unclear sensory planes that cannot be explained by sensory impairments. If other symptoms are met, the diagnosis of GBS cannot be denied.

2 Highly supported diagnostic cerebrospinal fluid characteristics:

A. Cerebrospinal fluid protein content can be increased in the first week of onset, and subsequent continuous measurements are elevated.

B. The number of white blood cells in the cerebrospinal fluid is 10 x 106 / L or less.

C. Variant: No protein content increased within 1 to 10 weeks after onset, and white blood cells were 11×106/L to 50×106/L.

3 Electrophysiological features that highly support diagnosis: Approximately 80% of patients have evidence of slowing or blockade of nerve conduction, conduction velocity is usually lower than normal 60%, but is patchy, not all nerves are involved, distal The latency is extended to three times normal, and the F-wave response of the proximal nerve trunk and nerve root conduction is a good indicator. About 20% of patients have normal conduction, and sometimes conduction abnormalities occur several weeks after onset.

Differential diagnosis

The peripheral nerve paralysis symptoms of this disease need to be differentiated from other peripheral neurological disorders.

Polio

More common in children not taking polio vaccine, characterized by asymmetrical paralysis, sensory presence, may have medullary paralysis, abdominal muscle paralysis, less cranial nerve involvement, general heat retreat after paralysis no longer develop, early cerebrospinal fluid The number of cells is often increased, and the phenomenon of protein-cell separation can be seen in the later stage. In children who have not taken polio vaccine, paralysis often has sequelae. The poliovirus can be isolated from feces and cerebrospinal fluid, and the serum specific antibody titer is increased during recovery.

2. Spinal cord tumor

3. Hypokalemia periodic paralysis

4. Other

Such as snoring sputum, etc., in addition, individual cases need to be differentiated from acute cerebellar ataxia, the symptoms of this disease are mainly cerebellar ataxia, manifested as gait instability, can not stand, refers to The nose test is unstable,

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