Neurobehcet's disease
Introduction
Introduction to neurobehide disease Neurobeausis syndrome refers to Behcet's syndrome, also known as neurobehavioral disease, which has neurological damage. Its clinical features are in addition to the three characteristics of oral mucosa aphthous stomatitis, uveitis and genital pain ulcer. After a certain period of time (average 6.5 years), there are symptoms of nervous system damage such as sputum, meningeal irritation, and personality changes, which are autoimmune diseases after infection. basic knowledge The proportion of illness: 0.002%-0.005% Susceptible people: no special people Mode of infection: non-infectious complication:
Cause
Cause of neurobehavioral disease
(1) Causes of the disease
Neurobehide syndrome is caused by allergic vasculitis caused by a certain bacteria or virus as an antigen.
(two) pathogenesis
As one of the complications of Behcet's disease, the neurobehavioral syndrome is similar to Behcet's disease. The literature reports the following several theories:
1. Infection theory Behcét et al believe that the disease is related to viral infection. Many scholars have found that patients with NBD have a history of fever and a history of inflammation of the tonsils, and found evidence of viral infection in the serum of patients.
2. The mechanism of immune mechanism can find anti-oral mucosal antibody in the serum of patients in the early stage. Kansu et al found that lymphocytes in CSF increased, complement C3 and IgG increased, and Hamza found that the ratio of T4/T8 in serum was decreased and NK cells were found. Increased, while NK's vitality decreased, which may be related to the lack of interleukin-2 (IL-2) and cytokine IFN-r.
3. Genetic factors The disease has a regional morbidity, mainly found in Japan, China, Iran and some countries in the eastern Mediterranean. Some scholars believe that it may be associated with people with certain HLA antigens (especially HLA-B51 and HLA-DRW52). Relatedly, HLA-DR1 and HLA-DQW1 antigens were significantly reduced in the population with high incidence.
4. A few other scholars have suggested that the onset of BD is associated with the secretion of sex hormones and the lack of zinc.
(3) Pathological changes
The basic lesion of NBD is intracranial small vessel damage. The main pathological changes are intracranial diffuse, small vascular inflammatory changes, early inflammatory cell infiltration around small blood vessels, focal necrosis in late stage, nerve glue Proliferating cells, local demyelination and different degrees of hyperplasia and fibrosis of the meninges, brain parenchyma (brain feet, brainstem, basal ganglia, thalamus or cerebellum, etc.) may have medium, large and small infarct lesions, generally Brain tissue edema and brain stem atrophy can be seen.
Prevention
Neurobehide disease prevention
There are no good preventive measures, early diagnosis and early treatment may improve the prognosis of NBD.
Complication
Neurobehide complications Complication
In addition to the above manifestations of nervous system involvement, patients have recurrent and relieved oral ulcers, genital ulcers and uveitis; skin may have papules, papules or other nodules; Device.
Symptom
Symptoms of neurobehavioral disease Common symptoms Nausea and vomiting Cerebellar ataxia Personality changes Diplopia disturbance
The central nervous system (CNS) of NBD is more affected than the peripheral nervous system. According to statistics, peripheral nerve damage of BD accounts for only 1% of the total number of NBD. All parts of CNS can be involved, and white matter is more affected than gray matter. The clinical manifestations of NBD are The affected parts vary, and there is generally no typical clinical form. Currently, the affected parts are classified into the following types:
1. Meningoencephalitis type is mostly acute or subacute, mainly manifested as headache, fever, neck stiffness, nausea, vomiting, diplopia, disturbance of consciousness, personality change, memory loss, etc., after a few days, hemiplegia, aphasia, structure Sound and difficulty swallowing.
2. Brainstem type often manifested as brain stem lesion syndrome or called multiple sclerosis syndrome, which can be expressed as typical cross-sacral hernia, cerebellar ataxia symptoms.
3. Spinal cord type NBD combined with spinal cord damage is rare. The literature reports that there may be spinal cord lesions such as paraplegia or quadriplegia, dysfunction of the bowel and bladder.
4. Peripheral nerve type This type is rare, and may have single neuropathy and nerve root damage.
5. Cerebellar lesions often manifest as cerebellar ataxia.
6. Cranial nerve spasm The nerves and facial nerves are more common.
In addition, it can also be expressed as a benign intracranial hypertension type, and there have been reports of muscle involvement in recent years, but it is extremely rare. The disease has a long course, and the disease course often has remission and deterioration. Hormonal and immunosuppressive drugs can improve the condition, and there are often sequelae of nerve damage after multiple episodes. According to the patient's meningitis and (or) encephalitis, brain stem damage and other neurological symptoms, accompanied by oral, genital ulcers and eye disease history, the diagnosis of this disease is not difficult, therefore, when the clinical high degree of suspicion of this disease, should be repeated Ask about the medical history, combined with clinical examination, give serum immune index examination, brain MRI, lumbar puncture and cerebral angiography and other auxiliary examinations, if necessary, perform biopsy of skin mucosa.
Examine
Check for neurobehavioral disease
1. The blood is often high.
2. Cerebrospinal fluid examination It is reported that 80% of patients have abnormal CSF examination, and the number of CSF cells is slightly increased, mainly lymphocytes, the total number is often less than 60×106/L, and the protein content is moderately increased, mostly below 1.00g/ L, CSF increased gamma globulin, CSF bacterial culture was negative, CSF changes are often not parallel with clinical manifestations, and the CSF changes in each episode are different, if the patient's CSF sees antimyelin factor (AMSF), It is often suggested that the disease is in the active phase. The CSF, IgA, IgM, IgG and other immune indicators are also increased in the CSF of active patients, and the IL-6 level in CSF is higher than that in normal people, and interleukin-6 (IL-6) persists. Increased often indicates poor prognosis, which can be used as an indicator for clinical monitoring and treatment.
3. The patient's serum immune index is similar to cerebrospinal fluid, and the clinical significance is the same.
4. Brain CT diagnosis of NBD sensitivity is poor, only a small number of patients found low-density lesions in the brain stem, thalamus or cerebral hemisphere.
5. MRI has high sensitivity. In the acute phase, patients with high-density shadows can be seen on T2-weighted images, while on T1-weighted images, they are equal-density or low-density shadows, mostly round, linear, crescent or irregular. Shape, mainly distributed in the brainstem, especially around the cerebral peduncle and pons of the midbrain. In addition, similar abnormal signals can be found in the thalamus, basal ganglia, cerebral hemisphere, spinal cord and cerebellum. When the condition is serious, it can be seen. Edema zone and mass effect, recurrent episodes of chronic NBD patients, brain stem atrophy can be seen in the late stage.
6. Cerebral angiography can show extensive stenosis and thrombosis of large, medium and small blood vessels. Among them, the stenosis of the anterior and middle cerebral arteries is more common.
Diagnosis
Diagnosis and differentiation of neurobehide disease
Because NBD can present a course of remission and recurrence, and the same patient may have multiple involvement of the nervous system at different times, clinically should be differentiated from multiple sclerosis (MS), aseptic meningitis, pseudobulbar paralysis.
