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*Only for medical professionals to read for reference.
At the Rheumatology and Immunity Conference, I listened to a neurology doctor talking about peripheral neuropathy.
.
.
20 March 20-21, 2021.
The 3rd Peking University Rheumatism and Immunity Hot Forum was successfully held.
In the conference, many speakers shared some cases of peripheral neuropathy.
What is the connection between connective tissue disease and peripheral neuropathy? Let's listen to Dr.
Meng Lingchao from the Department of Neurology of Peking University First Hospital tell the story of "When the Department of Rheumatology and Immunology encountered peripheral neuropathy".
Figure 1: Screenshot of the meeting conducted by Dr.
Meng Lingchao.
Dr.
Meng Lingchao said at the beginning, "Peripheral neuropathy actually seems to be quite simple.
"
Because of the peripheral nerves, the motor nerve, sensory nerve and autonomic nerve fibers are from large to small, and their respective functions can be ascertained from their names.
The structure of all nerves is very uniform, composed of cell bodies, axons, and myelin sheaths.
If you want to name the peripheral neuropathy, you can easily solve it with the time dimension (acute, subacute, chronic) plus the spatial dimension (upper extremity, lower extremity, distal end, proximal end, symmetry, and asymmetry).
However, it is really difficult to put it clinically.
.
.
Because the peripheral nerves are easily damaged, their causes are diverse.
However, peripheral neuropathy lacks examinations with the diagnostic significance of the gold standard.
Therefore, Dr.
Meng Lingchao emphasized that it is necessary to be cautious to establish any relationship between the cause of the disease and peripheral neuropathy.
In this case, how do we understand the relationship between connective tissue disease and peripheral neuropathy? Dr.
Meng Lingchao listed the following 5 possibilities: Peripheral neuropathy is part of the systemic damage of connective tissue disease; Peripheral neuropathy and connective tissue disease are both immune-mediated but relatively independent; Peripheral neuropathy is a certain systemic damage of connective tissue disease Secondary disease; Peripheral neuropathy is an adverse reaction to the treatment of connective tissue disease; it just happens to happen, it doesn't matter. In order to visualize these possibilities, Dr.
Meng Lingchao enumerated the relationship between several common connective tissue diseases and peripheral neuropathy.
The first is vasculitic peripheral neuropathy that is easiest to think of.
Vasculitis peripheral neuropathy, usually acute or subacute onset, is painful sensory or sensorimotor neuropathy.
It often begins with pain and weakness in a single nerve area, and gradually progresses to multiple mononeuropathy, or it is symmetrical, with less cranial nerve involvement.
In the auxiliary examination of vasculitic peripheral neuropathy: EMG shows axonal neuropathy, asymmetry, sensory or sensory + motor nerve involvement; laboratory tests (ANCA, etc.
); sural nerve biopsy: vasculitis, active axonal disease , Heterogeneity; nerve + muscle combined biopsy increases the positive rate.
The pathological manifestations of vasculitic peripheral neuropathy are mostly typical vasculitis.
However, if vasculitis is not cut on the cut surface, it can also be diagnosed as vasculitic peripheral neuropathy based on the pathological changes of nerves + clinical manifestations.
Figure 2: Sural nerve biopsy of vasculitis peripheral neuropathy.
Primary Sjogren’s syndrome (pSS) is also closely related to peripheral neuropathy.
Studies have shown that the proportion of pSS combined with peripheral neuropathy can be as high as 60%.
And the types of peripheral neuropathy associated with pSS are diverse, including distal sensory polyneuropathy, sensory neuron neuropathy, axonal sensorimotor polyneuropathy, mononeuropathy, chronic inflammatory demyelinating polyneuropathy , Guillain Barre syndrome, simple cranial neuropathy, small fiber neuropathy.
Among them, sensory neuron neuropathy is located in the cell body of sensory neuron; it is manifested as sensory neuropathy, especially deep sensory disorders and sensory ataxia.
When sensory neuron neuropathy occurs, in addition to considering pSS, we must also be vigilant about whether it is a paraneoplastic syndrome.
The electromyography of sensory neuron neuropathy checks that the motor nerve conduction is normal, and the sensory nerve conduction generally cannot be induced by the action potential wave amplitude.
The pathological examination of sensory neuron neuropathy revealed fiber loss, active axon, and lack of regeneration.
Figure 3: Pathological examination of sensory neuron neuropathy The pathological feature of chronic inflammatory demyelinating multiple radiculopathy (CIPD) is chronic demyelination, which is mainly manifested by numbness and weakness of the limbs; electromyography indicates sensory motor neuropathy.
Mainly demyelination; lumbar puncture has separation of protein cells; pathological examination is mainly demyelination (onion skin-like changes), and there may be uneven distribution of lesions.
Figure 4: Pathological features of CIPD.
Under the electron microscope, macrophages can be seen attacking myelin sheath.
Figure 5: Electron micrograph of CIPD.
Imaging shows thickening of nerve roots.
Figure 6: Imaging pictures of CIPD (CIPD patients on the left and normal people on the right) B-ultrasound can show segmental thickening of nerves.
Figure 7: B-ultrasound image of CIPD.
Small fiber neuropathy, mainly involving small pain-temperature sensory fibers and autonomic nerve fibers.
Often manifested as pain, numbness and autonomic neuropathy (perspiration, cardiovascular, gastrointestinal, genitourinary problems).
Routine electromyography may be normal, and skin biopsy can be used to evaluate small fibers.
Figure 8: PGP 9.
5 staining of skin biopsy of small fiber neuropathy.
Sweat test, upright tilt test, urine flow rate, urodynamics, esophageal pressure measurement, and gastric emptying radionuclide imaging can also help diagnose small fiber neuropathy.
Finally, Dr.
Meng Lingchao made a summary of the treatment of connective tissue disease combined with peripheral neuropathy from the professional perspective of neurology: First, it is necessary to evaluate the degree, activity and relationship of connective tissue disease and peripheral neuropathy; the treatment of connective tissue disease itself ; Symptomatic treatment of neurology: treatment of nutritional nerves, neuropathic pain, treatment for autonomic neuropathy; immunotherapy: Guillain-Barré syndrome (gamma bulb or plasma exchange, generally without hormones); CIDP (hormone or gamma bulb preferred, Second-line immunosuppressant, rituximab).
When rheumatism encounters peripheral neuropathy, there are many sparks.
The key is to follow the clues to find out who is arson.
At the Rheumatology and Immunity Conference, I listened to a neurology doctor talking about peripheral neuropathy.
.
.
20 March 20-21, 2021.
The 3rd Peking University Rheumatism and Immunity Hot Forum was successfully held.
In the conference, many speakers shared some cases of peripheral neuropathy.
What is the connection between connective tissue disease and peripheral neuropathy? Let's listen to Dr.
Meng Lingchao from the Department of Neurology of Peking University First Hospital tell the story of "When the Department of Rheumatology and Immunology encountered peripheral neuropathy".
Figure 1: Screenshot of the meeting conducted by Dr.
Meng Lingchao.
Dr.
Meng Lingchao said at the beginning, "Peripheral neuropathy actually seems to be quite simple.
"
Because of the peripheral nerves, the motor nerve, sensory nerve and autonomic nerve fibers are from large to small, and their respective functions can be ascertained from their names.
The structure of all nerves is very uniform, composed of cell bodies, axons, and myelin sheaths.
If you want to name the peripheral neuropathy, you can easily solve it with the time dimension (acute, subacute, chronic) plus the spatial dimension (upper extremity, lower extremity, distal end, proximal end, symmetry, and asymmetry).
However, it is really difficult to put it clinically.
.
.
Because the peripheral nerves are easily damaged, their causes are diverse.
However, peripheral neuropathy lacks examinations with the diagnostic significance of the gold standard.
Therefore, Dr.
Meng Lingchao emphasized that it is necessary to be cautious to establish any relationship between the cause of the disease and peripheral neuropathy.
In this case, how do we understand the relationship between connective tissue disease and peripheral neuropathy? Dr.
Meng Lingchao listed the following 5 possibilities: Peripheral neuropathy is part of the systemic damage of connective tissue disease; Peripheral neuropathy and connective tissue disease are both immune-mediated but relatively independent; Peripheral neuropathy is a certain systemic damage of connective tissue disease Secondary disease; Peripheral neuropathy is an adverse reaction to the treatment of connective tissue disease; it just happens to happen, it doesn't matter. In order to visualize these possibilities, Dr.
Meng Lingchao enumerated the relationship between several common connective tissue diseases and peripheral neuropathy.
The first is vasculitic peripheral neuropathy that is easiest to think of.
Vasculitis peripheral neuropathy, usually acute or subacute onset, is painful sensory or sensorimotor neuropathy.
It often begins with pain and weakness in a single nerve area, and gradually progresses to multiple mononeuropathy, or it is symmetrical, with less cranial nerve involvement.
In the auxiliary examination of vasculitic peripheral neuropathy: EMG shows axonal neuropathy, asymmetry, sensory or sensory + motor nerve involvement; laboratory tests (ANCA, etc.
); sural nerve biopsy: vasculitis, active axonal disease , Heterogeneity; nerve + muscle combined biopsy increases the positive rate.
The pathological manifestations of vasculitic peripheral neuropathy are mostly typical vasculitis.
However, if vasculitis is not cut on the cut surface, it can also be diagnosed as vasculitic peripheral neuropathy based on the pathological changes of nerves + clinical manifestations.
Figure 2: Sural nerve biopsy of vasculitis peripheral neuropathy.
Primary Sjogren’s syndrome (pSS) is also closely related to peripheral neuropathy.
Studies have shown that the proportion of pSS combined with peripheral neuropathy can be as high as 60%.
And the types of peripheral neuropathy associated with pSS are diverse, including distal sensory polyneuropathy, sensory neuron neuropathy, axonal sensorimotor polyneuropathy, mononeuropathy, chronic inflammatory demyelinating polyneuropathy , Guillain Barre syndrome, simple cranial neuropathy, small fiber neuropathy.
Among them, sensory neuron neuropathy is located in the cell body of sensory neuron; it is manifested as sensory neuropathy, especially deep sensory disorders and sensory ataxia.
When sensory neuron neuropathy occurs, in addition to considering pSS, we must also be vigilant about whether it is a paraneoplastic syndrome.
The electromyography of sensory neuron neuropathy checks that the motor nerve conduction is normal, and the sensory nerve conduction generally cannot be induced by the action potential wave amplitude.
The pathological examination of sensory neuron neuropathy revealed fiber loss, active axon, and lack of regeneration.
Figure 3: Pathological examination of sensory neuron neuropathy The pathological feature of chronic inflammatory demyelinating multiple radiculopathy (CIPD) is chronic demyelination, which is mainly manifested by numbness and weakness of the limbs; electromyography indicates sensory motor neuropathy.
Mainly demyelination; lumbar puncture has separation of protein cells; pathological examination is mainly demyelination (onion skin-like changes), and there may be uneven distribution of lesions.
Figure 4: Pathological features of CIPD.
Under the electron microscope, macrophages can be seen attacking myelin sheath.
Figure 5: Electron micrograph of CIPD.
Imaging shows thickening of nerve roots.
Figure 6: Imaging pictures of CIPD (CIPD patients on the left and normal people on the right) B-ultrasound can show segmental thickening of nerves.
Figure 7: B-ultrasound image of CIPD.
Small fiber neuropathy, mainly involving small pain-temperature sensory fibers and autonomic nerve fibers.
Often manifested as pain, numbness and autonomic neuropathy (perspiration, cardiovascular, gastrointestinal, genitourinary problems).
Routine electromyography may be normal, and skin biopsy can be used to evaluate small fibers.
Figure 8: PGP 9.
5 staining of skin biopsy of small fiber neuropathy.
Sweat test, upright tilt test, urine flow rate, urodynamics, esophageal pressure measurement, and gastric emptying radionuclide imaging can also help diagnose small fiber neuropathy.
Finally, Dr.
Meng Lingchao made a summary of the treatment of connective tissue disease combined with peripheral neuropathy from the professional perspective of neurology: First, it is necessary to evaluate the degree, activity and relationship of connective tissue disease and peripheral neuropathy; the treatment of connective tissue disease itself ; Symptomatic treatment of neurology: treatment of nutritional nerves, neuropathic pain, treatment for autonomic neuropathy; immunotherapy: Guillain-Barré syndrome (gamma bulb or plasma exchange, generally without hormones); CIDP (hormone or gamma bulb preferred, Second-line immunosuppressant, rituximab).
When rheumatism encounters peripheral neuropathy, there are many sparks.
The key is to follow the clues to find out who is arson.
