What Is Sclerodactyly?

Sclerosis is an inflammation and demyelination of the central nervous system and the immune system. In fact, nerve fibers, neurons and oligospinous cells are also damaged.

Sclerosis is an inflammation and demyelination of the central nervous system and the immune system. In fact, nerve fibers, neurons and oligospinous cells are also damaged.

Causes of sclerosis

The cause of sclerosis is still unknown. Presumably there will be some evoking factors that cause seizures. Among them, viral infections are the most well known; there are also reports of bacteria-related infections. In addition, three aspects of production, immunization and stress should be considered.

Sclerosis is not a genetic disease, but genes still play an important role in the severity of the disease. In the assessment of risk factors, the incidence of first-degree relatives of patients with sclerosis is 3 to 5%; 3 to 5% of fraternal twins; 25% of those with identical twins; compared to 0.2% of the entire population The incidence is still increasing. In any case, the exact genes affected are still inconclusive.

Increased activated lymphocytes in the system; destruction of BBB; increased migration of lymphocytes in the central nervous system; local immune response to the central nervous system; subsequent activation of stellate cells and endothelial cells; plaques after central nervous system damage .

Clinical manifestations of sclerosis

Because sclerosis invades the myelin sheath of nerve fibers, the clinical symptoms depend on the damaged nerve fibers. In general, the effects of movement, sensation, and vision are common; symptoms of nerve fibers in the brainstem and cerebellum are damaged, diplopia, difficulty swallowing, instability, and dizziness. If the spinal cord becomes inflamed, in addition to exercise and sensory function, the excretory function will also be impaired. A small number of patients will also have epilepsy and mental retardation.

Sclerosis is characterized by a variety of symptoms and signs of central nervous system dysfunction, combined with repeated relief and recurrence. The most common symptoms are paresthesia, which occurs on one or more limbs, the trunk or one side of the face; weakness or clumsiness in the legs or hands; or visual impairments, such as partial blindness in one eye and pain during eye movement, Objects are fuzzy or dark. Other common early symptoms include diplopia due to ophthalmoplegia, transient weakness in one or more limbs, minor gait disturbances, mild stiffness and abnormal fatigue in one limb, difficulty in controlling the bladder, dizziness, and mildness All of these symptoms reflect disseminated lesions within the central nervous system and can often occur months or years before the disease is diagnosed. Increased body temperature can worsen symptoms and signs.

Sclerosis test

Feasible MRI of the brain and spinal cord.

Diagnosis of sclerosis

When a diagnosis of sclerosis is suspected, laboratory methods are needed to assist the diagnosis. At present, the following methods are generally adopted:

Blood test

In order to rule out other possible diagnoses, however, there are many blood draw items that cannot be listed one by one; nor can all tests be performed. Therefore, careful medical history is very important.

2. Brain and Spinal MRI

It is best to have a contrast agent showing new lesions. In patients with myelitis, after the completion of spinal magnetic resonance MRI, the opinion on whether to add brain MRI remains inconclusive. However, if the attack is localized in the brain, even in the first attack, 50 to 65% of the patient's MRI has been abnormal. Usually, the T2-weighted image is a basic examination. A new T1-weighted lesion is indicated after the application of the contrast agent, indicating an acute attack.

3.Cerebrospinal fluid

The main inspection is the presence or absence of immunoglobulins and OCB; the IgG index calculated by combining serum albumin and immunoglobulin is also calculated: if it is higher than 0.7, it indicates that immunoglobulin is produced in the spinal cord. In fact, although the inflammatory cells and protein of cerebrospinal fluid are elevated, if the inflammatory cells are greater than 50 / mm3 and the protein is greater than 100 mg / dl, other differential diagnosis must be considered.

4. Evoked potential wave

Somatosensory evoked potentials (SSEP) to examine cervical spinal cord lesions; visual evoked potential waves (VEP) to examine optic nerve inflammation; auditory brainstem evoked potential waves (ABEP) to examine brain stem lesions.

Sclerosis treatment

1. Change the course of the disease (DMT)

Currently the most common is interferon. In the study, it will reduce the frequency of seizures, prolong the period of recurrence, reduce the lesions on MRI images, and then improve the quality of life of patients. However, before using it, patients must understand that the administration of DMT is not a cure but a reduction of seizures; and DMT also has its side effects, such as interferon with cold-like symptoms and abnormalities of white blood cells and liver, so preventive antipyretics .

2. Treatment of acute attacks

The anti-inflammatory and anti-edema effects of steroids are used as first-line treatment in acute episodes. If the condition persists, transfusion therapy or immunoglobulin (IVIG) injections are also considered an alternative.