Guillain-Barré syndrome
Introduction
Introduction to Guillain-Barre Syndrome Guillain-Barréssyndrome (GBS), also known as acute infectious polyneuritis, is an autoimmune disease caused by viral infection or infection and other causes, the main pathological changes Extensive inflammatory demyelination for the peripheral nervous system. Clinically, the symmetry flaccid paralysis of the limbs is the main manifestation. basic knowledge The proportion of illness: the incidence rate is about 0.001% - 0.0005% Susceptible people: no specific population Mode of infection: non-infectious Complications: dysphagia, high blood pressure
Cause
Cause of Guillain-Barre syndrome
(1) Causes of the disease
It is not yet fully understood. It has been thought to be related to viral infections, respiratory infections, diarrhea, etc. It is now clear that GBS is a disease caused by cellular and humoral immunity, and antibodies caused by peripheral nerve-specific antigens cause loss of myelin.
The infection rate of Campylobacter jejuni (CJ) before GBS is high, which is an important factor in promoting this disease.
(two) pathogenesis
Early serological studies found that 48% of patients were positive for various viral complement binding tests, such as cold and paracolitis virus, monocytic virus, adenovirus, etc. In recent years, GBS patients were found to have a prevalence of Campylobacter jejuni infection before and after the disease. high.
Asbury et al. compared the clinical pathology of patients with this disease with the performance of experimental allergic neuritis (EAN), and concluded that the two were very similar. Domestic scholars tested cerebrospinal fluid and peripheral blood lymphocytes in patients with acute GBS. The group found that the increase of T cells in the cerebrospinal fluid and the decrease of T cells in the peripheral blood (Zhou Shanren et al., 1983), recently found that the plasma levels of tumor necrosis factor- (TNF-) and interleukin-2 (IL-2) were found in patients. Significantly elevated, and in the recovery period of glucocorticoid treatment improved, both concentrations decreased and near normal, suggesting that TNF- and IL-2 secreted by macrophages and antigen-activated T cells may directly Indirectly involved in the pathological damage of peripheral nerve demyelination (Ocean et al., 2000).
Other studies have detected anti-myelin IgM antibodies that bind to complement and various high-titer anti-sphingolipid antibodies and anti-GM1 antibodies in the serum of patients. In addition, IgG, IgA in cerebrospinal fluid are elevated, and oligoclonal IgG appears. Recent studies suggest that autoimmune T cells and activated monocytes and macrophages enter the peripheral nerve from the blood to participate in the process of damaging the peripheral nerves. Intercellular adhesion molecules (ICAM-1) play a role.
Although some experimental studies are not completely consistent, it has been established that GBS is mediated by cellular and humoral immunity, and antibodies caused by peripheral nerve-specific antigens cause diseases of myelin loss.
Neuropathological changes are mainly in the peripheral nervous system, which is the spinal nerve root system and extensive segmental demyelination and inflammatory cell infiltration. The axonal changes are relatively light, only swelling and distortion.
The anterior root of the spinal nerve is damaged and heavier than the posterior root. The proximal part of the peripheral nerve is heavier and the distal end is relatively light. The early stage of the disease is mainly neuroedema. The lymphocytes and macrophages form the perivascular sleeve, the spinal nerve root and the peripheral nerve. There is also mononuclear cell infiltration and increase. Monocytes and macrophages destroy Schwann cell basement membrane and cause extensive segmental demyelination. Occasionally, the spinal cord can be involved. In the middle stage of the disease, neuroendothelial fibroblasts can be obviously proliferated. Visible Schwann cell proliferation.
In some cases of explosive disease, axonal injury, rupture or even granulation in the acute phase due to a strong immune response, a few cases of severe axonal degeneration during the recovery period (6-7 weeks).
The GBS variant found in the northern part of China in the early 1990s is acute motor axonal neuropathy (AMAN). The pathological changes are mainly in the axon, Wallerian-like degeneration of anterior root and peripheral nerves. The myelin loss is mild, the inflammatory lesions are rare, and the diseased fibers have no lymphocyte infiltration, but macrophages can be found in the degenerated area, and this type is particularly closely related to CJ infection (Li Chunyan et al., 1993).
Prevention
Guillain-Barre Syndrome Prevention
There are no good preventive measures for autoimmune diseases. Pay attention to the following points in clinically cured patients to prevent recurrence:
1. Strengthen nutrition, enhance physical fitness, and prevent colds.
2. Practice correct coughing and coughing methods to prevent secondary infections in the lungs.
3. Vaccination, pregnancy, surgery can induce this disease.
4. Critically ill patients, sudden loss of mobility, prone to anxiety, tension and other emotions, should be appropriate psychological counseling.
Complication
Complications of Guillain-Barre syndrome Complications, dysphagia, high blood pressure
Often accompanied by facial nerve spasm, difficulty swallowing, vocal weakness and respiratory muscle spasm, pulmonary infection, a few extraocular tendons, tachycardia, hyperhidrosis, sudden hypertension or bladder sphincter disorders and other autonomic nervous system dysfunction Severe cases can be life-threatening due to respiratory muscle spasm.
Symptom
Symptoms of Guillain-Barre syndrome Common symptoms Diarrhea, chest tightness, breath sounds, weakened air, cranial nerves, paralysis, dysfunction, coma, symmetry, tachycardia
1. Aura symptoms often precede the onset of upper respiratory tract or digestive tract infections such as fever, diarrhea and so on.
2. Movement disorders
(1) limb paralysis: the limbs are symmetric under the motor neuron spasm, and often start from the lower limbs, gradually affecting the upper limbs, but also from one side to the other side, very few patients are first limited to the lower limbs, usually in 1 Within 2 weeks, the condition develops to the highest peak, and then tends to be stable. The tendon is generally heavier at the proximal end, the muscle tension of the limbs is low, the tendon reflex is weakened or disappeared, the abdominal wall and the cremaster reflex are normal, and a few may appear due to the involvement of the pyramidal bundle. Pathological reflex signs, muscle atrophy gradually appeared after 2 to 3 weeks of onset.
(2) trunk muscle spasm: cervical tendon can not raise the head, intercostal muscles, tendon tendon can appear respiratory muscle paralysis (20% ~ 30%), manifested as chest tightness, shortness of breath, low voice (like meow), cough Inability to lie down, chest or abdominal breathing decreased (generally intercostal muscle paralysis is earlier than the diaphragm) and respiratory sounds are weakened. In severe cases, coma and death may occur due to hypoxia or respiratory complications.
(3) cranial nerve palsy: about half of the patients may have cranial nerve damage, with tongue pharynx, vagus and peripheral paralysis of one or both sides of the nerve are more common, followed by eye movement, trochle, nerve expansion, occasional optic disc edema May be caused by inflammatory changes in the optic nerve itself or cerebral edema; it may also be associated with a significant increase in cerebrospinal fluid protein, obstructing arachnoid villi, affecting the absorption of cerebrospinal fluid.
3. Sensory disturbances are often the first symptom. Subjective sensory disturbances are mainly caused by numbness and acupuncture at the end of the extremities. Pulling the nerve roots during examination often exacerbates the pain (such as positive Kernig sign), and the muscles may be obvious. The tenderness (especially on the bilateral gastrocnemius muscles), objective examination may have the feeling of loss of gloves, sock-like and/or trigeminal innervation, or no sensory disturbance, and the sensory disturbance is much lighter than the movement disorder, which is one of the characteristics of this disease. .
4. Autonomic dysfunction often has excessive sweating and odor at the initial stage or recovery period. It may be the result of sympathetic stimulation. A small number of patients may have short-term urinary retention at the beginning, which may be temporarily misaligned or dominated by autonomic function that governs the bladder. The spinal nerves of the external sphincter are damaged. Some patients may have cardiovascular dysfunction such as blood pressure instability, tachycardia and abnormal ECG.
5. Laboratory examination of cerebrospinal fluid protein protein separation occurred 1 to 2 weeks after onset, and most significant in the second to eighth week, and gradually recovered, the number of white blood cells does not exceed 10 × 106 / L, cytological classification of lymphocytes and Mononuclear cells are predominant, and macrophages are visible, protein content is significantly increased, and sugar and chloride are normal.
6. Electrophysiological examination The motor nerve conduction velocity is obviously slowed down. The prolongation or disappearance of F wave latency is seen in demyelinating GBS. If it is AMAN, the motor nerve conduction velocity is normal or slightly slow, and the F wave latency of sensory fiber is normal or light. Degree is extended.
GBS can occur again. Generally, it is called recurrent GBS after several months to several years. Symptoms are often more serious than the first episode when recurrence. Fisher syndrome is another variant of GBS. This type of onset is acute, mainly for the eye. External tendon, ataxia and sputum reflex disappeared triad, occasionally accompanied by limb paralysis and CSF protein cell separation. The pathogenesis of these variants of GBS is different from those with myelin loss.
Examine
Guillain-Barre syndrome examination
1. Cerebrospinal fluid has a protein-cell separation phenomenon with increased protein and normal or near normal cells. Another feature of this disease is that cerebrospinal fluid protein often begins to increase 7 to 10 days after onset (the range of increase varies), 4 After 5 weeks, the peak was reached, and gradually decreased after 6-8 weeks. The increase of cerebrospinal fluid protein content was not related to the condition. After recovery of limb paralysis in a few patients, the protein content of cerebrospinal fluid was still high. Some patients had normal protein content, cerebrospinal fluid and Immunological examination of blood is often abnormal.
2. The total number of white blood cells and blood sedimentation increases and the erythrocyte sedimentation rate increases, which indicates that the condition is serious or there are pulmonary complications.
Electromyography examination is related to the severity of the disease and the course of the disease. In the acute phase (within 2 weeks after the disease), the motor unit potential is often reduced, the amplitude is reduced, the motor nerve conduction velocity is normal, and some may have prolonged latent time, but often F-wave (proximal motor fiber conduction) latency extension or waveform disappearance, can also be measured by motor evoked potential (MEP), to understand the degree of damage to the proximal nerve roots and peripheral nerves, the abnormal rate can reach 74.1%, gradually appear after 2 weeks Denervation potential [such as fibrillation and / or positive sharp wave], when the disease course enters the recovery period or later, it can be seen that the multi-phase potential increases, a small motor unit potential (neonatal potential) appears, and the motor nerve conduction velocity is often slowed down. , and the terminal latency is prolonged, and the sensory nerve conduction velocity can also be slowed down.
Diagnosis
Diagnosis and identification of Guillain-Barre syndrome
Diagnostic criteria
In 1993, the editorial board of the Chinese Journal of Neuropsychiatry has developed the diagnostic criteria for GBS in China with reference to international data. Generally, it has symptoms of upper respiratory tract or gastrointestinal infection before the disease, and rapidly develops into lower extremity after 1 to 2 weeks. Neuronal spasm, severe cases of medullary paralysis and respiratory muscle spasm should be considered.
If CSF is separated by protein cells, electrophysiological examination of the sacral limbs suggests that the proximal or distal nerve damage of the peripheral nerve trunk is more helpful in confirming the diagnosis.
Differential diagnosis
1. When polio is onset, there is more fever, muscle spasm is segmental, and asymmetry, no sensory disturbance, cerebrospinal fluid white blood cell count often increases.
2. Although acute myelitis is also flaccid paralysis in the acute phase, it often has pyramidal tract signs and transverse sensory disturbances, and sphincter dysfunction is more obvious, cerebrospinal fluid protein and cells are slightly increased or normal.
3. Periodic paralysis (periodic paralysis) Acute onset, may be symmetrical flaccid paralysis of the limbs, a few cases may also have respiratory muscle paralysis, but often serum potassium levels and low potassium electrocardiogram changes, short course, after potassium supplementation Can be quickly restored, more than a few hours to 3 to 4 days to heal.
Attention should be paid to the identification of acute toxic multiple peripheral neuropathy and cancerous multiple peripheral neuropathy caused by lead and arsenic.
