The Secret Link to Mysterious Diseases: Why does Susac Syndrome cause lymphocytosis?

Lymphocytosis, i.e., an abnormal increase in lymphocytes in the myeloid cerebrospinal fluid, is often seen as a sign of infection or inflammation within the nervous system.This phenomenon can be observed in a variety of neurological diseases, such as pseudomigra, Susac syndrome and encephalitis.Lymphocytes are one of the main components of human white blood cells. However, under normal conditions, the number of lymphocytes in the medullary cerebrospinal fluid is generally less than five per microliter.When the number of lymphocytes exceeds one thousand per microliter, it indicates that an inflammatory response may occur and may be accompanied by an increase in protein concentration in the cerebrospinal fluid.

Symptoms and Signs

The specific symptoms of lymphocytosis vary with each disease.Milder lymphocytosis is usually considered to be onset within the range of 10 to 100 lymphocytes per cubic millimeter, and the medullary cerebrospinal fluid in healthy individuals usually contains only 0 to 5 white blood cells.In patients studying pseudomigra, lymphocyte concentrations have been shown to range from 10 to 760 per cubic millimeter, while the mean concentration is 199 ± 174 per cubic millimeter.When the number of white blood cells rises to more than 500 per cubic millimeter, the medullary cerebrospinal fluid may appear cloudy during testing.This increase in concentration often coincides with the inflammatory immune response caused by typical viral infections.Despite the diversity of these diseases, various lymphocytosis diseases are usually the most obvious symptoms of headache and neurological deficiencies.

causes

The lymphocytosis in the medullary cerebrospinal fluid is usually an immune response to neurovascular inflammation.The root cause of many cases is viral infection, which triggers the immune system to produce antibodies against neurons and vascular antigens.Therefore, this phenomenon may be related to viral meningitis and Mollaret disease.Certain nonviral infections, such as Lyme disease, are also considered as potential causes.In some diseases, infection triggers an autoimmune response, further leading to increased lymphocyte levels.

Diagnosis

The presence of lymphocytosis is usually detected by lumbar puncture and subsequent clinical cerebrospinal fluid analysis.In combination with the appearance, pressure analysis, glucose and protein content measurement of medullary cerebrospinal fluid, leukocyte counts can be used to detect or diagnose a variety of diseases, including subarachnoid hemorrhage, multiple sclerosis, and various types of meningitis.Although lumbar puncture may return to the normal range of 0-5 cells per microliter, this does not rule out the possibility of disease.

Disease Relevance

Study found that lymphocytosis is related to the following diseases:

Viral encephalitis

The occurrence of encephalitis causes brain inflammation to lead to loss of neurological function, and patients may show symptoms such as fever, confusion, forgetfulness, personality changes, paralysis, epilepsy and language disorders.Viral infection may directly lead to encephalitis, or it may trigger subsequent autoimmune responses, which ultimately lead to an increase in lymphocyte concentration in the medullary cerebrospinal fluid.Among patients with herpes simplex virus, more than 90% of patients were found to have different degrees of lymphocytosis.Intravenous acyclovir can prevent viral replication, and high doses of acyclovir can also be used in the case of persistent lymphocytosis.Studies have shown that this treatment combines vancomycin to fight HSV-1 and ultimately restores lymphocyte counts to normal levels.

Pseudomigra

Several studies have been conducted on the correlation between pseudomigra and lymphocytosis.Pseudomigra is characterized by moderate or severe bilateral pulsating headaches, accompanied by transient neurological symptoms and lymphocytosis.These migraine attacks are repeated and self-limiting.In these studies, individuals aged 15-40 were examined, with most of the subjects being tested as males.After each attack, the patient recovered asymptomatic.After EEG, CT and MRI examinations, both CT and MRI scans were normal; however, if abnormal EEG scans were found in 42 out of 30 patients.EEG in 26 patients showed unilateral hyperslowing, while in 4 patients, bilateral slowing.

Susac syndrome

In Susac syndrome, autoimmune response damages blood vessels in the brain, retina and cochlea, resulting in loss of neurological function.Patients with this syndrome have hearing and vision loss, and found that their lymphocyte and protein concentrations in the medullary cerebrospinal fluid are high.Treatment with such immunosuppressive drugs such as steroids and Azathioprine was found to have significant effects on patients, and some patients can gradually recover their lost function within a few weeks.

Treatment

The best way to restore lymphocyte count to normal levels is to treat diseases that cause elevation.In cases where the root cause is viral or bacterial infection, drugs against these pathogens have been proven to be effective.Because herpesvirus infection often leads to lymphocyte growth, acyclovir and vancomycin are common prescription drugs; when lymphocytosis is caused by an autoimmune response, immunosuppressive drugs such as steroids can be used to alleviate symptoms.

History

As the development of lumbar puncture surgery, the diagnosis of lymphocytosis has become possible.Early lumbar punctures were performed in the late 19th century, while tests that revealed lymphocytosis in the medullary cerebrospinal fluid occurred later.Modern lumbar puncture techniques are often used to diagnose or rule out certain diseases, such as meningitis, and to evaluate the presence of infection in the medullary cerebrospinal fluid.

How will understanding lymphocytosis affect our medical decisions in the face of such a diverse range of neurological diseases?

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