Monoclonal gammopathy with peripheral neuropathy
Introduction
Introduction to monoclonal gamma globulin disease with peripheral neuropathy Monoclonal gamma globulin disease with peripheral neuropathy, also known as paraproteinemicperipheralneuropathy. When there is too much M protein in the blood, it is called monoclonal globopathy, also known as immunoglobulin disease, paraprotein disease or plasma cell disease. The pathological significance of the appearance of M protein in the blood to peripheral nerves is that the M protein contains one or more antibodies against myelin or axonal membranes, such as anti-MAG, GM1 and GD1 antibodies. Among the unexplained idiopathic peripheral neuropathy, 10% have monoclonal propoferopathy, and 29% to 71% of the monoclonal globopathy of undetermined significance (MGUS) is associated with peripheral neuropathy, suggesting that monoclonal C Ball disease is associated with peripheral neuropathy. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific people Mode of infection: non-infectious complication:
Cause
Monoclonal gamma globulin disease with etiology of peripheral neuropathy
(1) Causes of the disease
Monoclonal gamma globulin disease, immunoglobulin disease, paraproteinemia, and intrahepatic protein abnormality. This group of diseases, also known as plasma cell disease, is a group of neoplastic or tumor-prone diseases. The monoclonal plasma cells of the immune B cell line are abnormally proliferated and secreted by immunoglobulin.
Peripheral nerve myelin is composed of a variety of glycolipids and glycoproteins, of which glycosphingolipids and myelin sheath associated glycoprotein (MAG) are considered to be immune-mediated peripheral neuropathy-associated antigens.
Glycosphingolipids include gangliosides (GM) and sulfated glycosides. The former can be divided into GM1, GM2, GD1a, GD1b, GT1B, GQ1b and LM1 depending on the amount of sialic acid contained. The latter includes brain sulphur and SGPG (sultate-3-glucuronyl paragloboside), in which SGPG is most closely related to immune-mediated peripheral neuropathy. Due to the different spatial distribution of different antigens, the clinical characteristics of each mediated peripheral neuropathy are different. For example, GM1 is mainly distributed in exercise. On the myelin sheath of the nerve, the peripheral neuropathy mediated by it is a simple motor neuropathy, and GD1b is mainly distributed on the myelin sheath of the sensory nerve, thus mediated the generation of sensory peripheral neuropathy.
MAG is a small glycoprotein on the myelin sheath, which is concentrated on the periorbital Schwann cell membrane and the paraspinal sac of the myelin sheath. It is an adhesion molecule that connects axons and Schwann cells. It contains five types of immunity. A globulin region and a carbon water antigenic determinant that can react with HNK-1 adhesion molecules.
(two) pathogenesis
An important pathological mechanism of monoclonal neuronopathy combined with peripheral neuropathy is that M protein contains a variety of antibodies that act directly on myelin and axonal membranes. For example, MAG antibodies can be inserted into tightly packed myelin lamellar structures and combined with MAG. , thereby destroying the integrity and stability of the myelin structure, leading to demyelinating peripheral neuropathy, immunohistochemical studies have found deposition of immunoglobulins and complement on the peripheral nerves, animal experiments have also confirmed the application of MAG, GM and SGPG Animal models of demyelinating neuropathy can be successfully established by passive transfer of serum from immunized animals or applied patients.
Cochlear nerve biopsy can be seen in segmental demyelination or axonal degeneration, sometimes see Schwann cell proliferation to form onion-like structure, a few cases have mononuclear cell infiltration, immunofluorescence and immunocytochemistry studies can be found in the margin of the myelin sheath IgM-kappa chain deposition.
Prevention
Monoclonal gamma globulin disease with peripheral neuropathy prevention
There are no good preventive measures for autoimmune diseases, mainly symptomatic treatment and intensive care.
Complication
Monoclonal gamma globulin disease with peripheral neuropathy complications Complication
After long-term follow-up (2 to 22 years), about one-fourth of patients can develop malignant plasma cell disease. Multiple myeloma is the most common, but there is no simple and reliable detection method to predict, radionuclide labeling index Increased DNA rate in plasma cells is a strong evidence for the development of malignant plasma cell disease, but clinically no practical significance.
Symptom
Monoclonal gamma globulin disease with peripheral neuropathy symptoms common symptoms demyelinated gait instability block ataxia
At the same time, there are two sets of clinical symptoms and signs, that is, the manifestations of multi-system lesions caused by monoclonal neuron disease, peripheral motion caused by peripheral nerve damage, and sensory autonomic dysfunction. This is one of the clinical features of this disease. .
Clinical, pathological, and electromyographic features of malignant or specific monoclonal-gammaglobulin associated with peripheral neuropathy.
MGUS or benign monoclonal propofol combined with peripheral neuropathy is mainly seen in children over 50 years old, insidious onset, clinical manifestations of foot numbness, paresthesia, balance disorder and gait instability, deep feeling and tactile involvement are obvious, half of patients have pain Discomfort, the course of the disease often lasts for several years to several decades. In the late stage, there is weakness in the distal extremities and atrophy in different degrees, but it is rarely bedridden due to muscle weakness.
A small number of patients can be expressed as simple motor neuropathy, similar to the performance of motor neuron disease.
Electrophysiological examination sometimes has localized motor block, cerebrospinal fluid protein is often increased, IgM type M protein patients, limb tremor, deep sensory loss and ataxia are more serious, distal limb weakness is late, electrophysiological examination is obvious The demyelination changes, and the electrophysiological examination of patients with IgG type M protein is axonal neuropathy.
Examine
Monoclonal gamma globulin disease with peripheral neuropathy
1. Serum protein electrophoresis For the unexplained idiopathic peripheral neuropathy, serum protein electrophoresis and immunoelectrophoresis should be routinely performed to detect the presence or absence of M protein.
2. In the urine, the peri-protein is sometimes negative in the serum M protein, but the light chain of the M protein can enter the urine, which is called the peri-protein. The urine-peripheral protein has the same clinical significance as the serum M protein. Therefore, urine testing must be performed simultaneously with serum.
3. Cerebrospinal fluid protein is often increased.
Electrophysiological examinations sometimes have localized motor block, and the type of M protein is associated with clinical manifestations and electromyography characteristics of peripheral neuropathy.
Diagnosis
Diagnosis and identification of monoclonal gamma globulin disease with peripheral neuropathy
Diagnostic criteria
1. Clinical manifestations such as sensation of peripheral neuropathy and dyskinesia.
2. For idiopathic peripheral neuropathy with unknown causes, protein and electrophoresis of urine and serum should be routinely performed to detect the presence or absence of M protein.
3. Cerebrospinal fluid protein is often increased.
4. Electrophysiological examination may have localized motor block.
5. If necessary, the sural nerve biopsy can be seen in segmental demyelination or axonal degeneration.
Differential diagnosis
The relationship between monoclonal neuronopathy peripheral neuropathy and chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy is currently undetermined.
The clinical manifestations and electrophysiological characteristics of CIDP and some types of monoclonal neuron disease peripheral neuropathy are very similar, and both have elevated cerebrospinal fluid protein, and 1/4 of CIDP can be combined with monoclonal gamma disease.
20% to 84% of patients with multifocal motor neuropathy can detect GM1 antibodies in serum, 20% of which are monoclonal, and the rest are polyclonal. It is speculated that CIDP and multifocal motor neuropathy and monoclonal neuron disease peripheral neuropathy may be There are common pathophysiological mechanisms.
