Pediatric Neurobehcet Syndrome
Introduction
Introduction to Pediatric Neurobehide Syndrome 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), symptoms of nervous system damage such as sputum, meningeal irritation, and personality changes occur. It is an autoimmune disease after infection. basic knowledge The proportion of the disease: the incidence rate is about 0.001% -0.002% Susceptible people: children Mode of infection: non-infectious Complications: optic atrophy dementia paraplegia
Cause
Pediatric neurobehide syndrome etiology
Infection theory (35%):
Behcét et al believe that the disease is associated with viral infections. Many scholars have found that patients with NBD have a history of fever and a history of inflammation of the tonsils, and evidence of viral infections in the serum of patients.
Immune mechanism theory (30%):
In the early stage, anti-oral mucosal cell antibodies were found in the serum of patients. Kansu et al found that lymphocytes increased in CSF, and complement C3 and IgG increased. Hamza found that the ratio of T4/T8 in serum decreased and NK cells increased, while NK Vitality declines, which may be related to the lack of interleukin-2 (IL-2) and cytokine IFN-r.
Genetic factors theory (25%):
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 related to people with certain HLA antigens (especially HLA-B51 and HLA-DRW52). In the population with high incidence, HLA-DR1 and HLA-DQW1 antigens were significantly reduced.
Other factors (10%):
A few scholars have suggested that the onset of BD is associated with the secretion of sex hormones and the lack of zinc.
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, and focal necrosis in the late stage. , glial cell hyperplasia, 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 infarcts In the lesion, brain tissue edema and brain stem atrophy are generally visible.
Prevention
Pediatric nerve white syndrome prevention
1. Actively prevent and treat various infectious diseases and do various vaccinations.
2. Keep the indoor air fresh and ventilate twice a day for 15-30 minutes each time.
3. Wash hands before and after touching the patient, do not string the door to prevent hospital infection.
Complication
Pediatric neurobehide syndrome complications Complications, optic atrophy, dementia, paraplegia
Often accompanied by anterior chamber empyema, optic disc and optic atrophy, mental decline, severe dementia, increased intracranial pressure, paraplegia, sensory disturbance, bladder, rectal sphincter dysfunction, cerebellar ataxia.
Symptom
Pediatric nerve white syndrome syndrome common symptoms oral ulcer nausea uveitis pustular ataxia sensory disorder sleepiness eyeball tremor vulvar ulcer blisters
1. Oral ulcer: Aphthous stomatitis, manifested in the lips, gums, tongue, etc. There are single or multiple painful ulcers, often self-healing in 3 to 7 days, but there may be multiple recurrences in a year, even the former The latter has been started, and it has been one after another.
2. Vulvar ulcer: In the penis, scrotum, labia, vagina and other genital mucosa, small pustules or small blisters appear first. After 1 to 2 days, the ulceration is round and painful, and it usually heals itself in 1 to 2 weeks.
3. Ocular uveitis: ocular symptoms with recurrent uveitis, especially iridocyclitis, most often with anterior chamber empyema, can also be expressed as conjunctiva, cornea, retinitis, optic disc and optic nerve Shrinking and so on.
4. Skin damage: can be expressed as nodular erythema, subcutaneous thrombophlebitis and allergic rash.
5. Neurological symptoms
(1) Brain stem symptoms: cross-elastic spasm, medullary paralysis, nystagmus, and co-direction skew often occur.
(2) meningeal irritation symptoms: fever, headache, nausea, vomiting, neck stiffness and so on.
(3) Psychiatric symptoms: apathy, depression or euphoria, excitement, lethargy, coma or hallucinations, delusions, mental retardation, severe dementia.
(4) increased intracranial pressure: manifested as headache, vomiting, optic disc edema, increased cerebrospinal fluid pressure.
(5) Spinal cord symptoms: symptoms of paraplegia or spinal cord damage, sensory disturbances below the level of damage, bladder and rectal sphincter dysfunction.
(6) Others: may have cerebellar ataxia, pseudobulbar palsy, quadriplegia, soft diaphragmatic hernia, bilateral median nerve and ulnar nerve damage.
6. Other manifestations: may have synovitis, colitis and other joint and gastrointestinal symptoms.
Oduffy's diagnostic criteria are: recurrent aphthous ulcers (oral or perineum), plus 2 of the following 3 can be diagnosed as Behcet's syndrome:
1 choroiditis (front or posterior).
2 synovitis.
3 cutaneous vasculitis, if there is aphthous stomatitis plus any of the above three diagnosed as incomplete Behcet's syndrome, the diagnosis of symptoms or signs of nervous system damage in the process of Behcet's syndrome is diagnosed For the neurobehide syndrome.
Examine
Pediatric nerve white syndrome
1. Blood test: serum anti-oral mucosal antibody and leukocyte cytoplasmic binding factor increased, the positive rate of red blood cell agglutination increased by heat-agglutinated human -globulin as antigen, T lymphocyte increased, B lymphocyte decreased, IgD increased Increased mucin, accelerated erythrocyte sedimentation rate, positive C-reactive protein, etc.
2. Cerebrospinal fluid examination: Cerebrospinal fluid pressure increased, white blood cell count increased, mostly below 60 × 106 / L (ie 60 / mm3), high can be greater than 5000 × 10 6 / L (ie 5000 / mm 3 ), white blood cell species with lymph Cells are predominant, and neutrophils are also reported. The protein is mild to moderately increased (50 mg% to 100 mg%), and the sugar and oxide changes are not obvious.
3. EEG examination: The abnormal rate of EEG is about 70%. The generalized -wave type, ie, the extensive monosyllabic alpha wave, can appear in the worsening or remission period. This waveform often indicates brain stem damage. Abnormalities such as slowing of basic rhythm, persistent or paroxysmal slow waves, and changes in brain waves are often parallel to central nervous system symptoms.
4. Brain CT scan: Most of the brain atrophy, ventricular enlargement, brain stem atrophy and low density areas and other abnormalities.
5. Evoked potential: There may be abnormalities in somatosensory evoked potentials and auditory evoked potentials.
6. Non-specific skin test: The formation of papules or pustules can be seen by general acupuncture on the skin, or subcutaneous injection with 0.1 ml of normal saline, and erythema and pustules with a diameter of 5-20 mm can be formed after 24 to 48 hours. The specific skin reaction is caused by tissue hemorrhage accompanied by acute invasive inflammation dominated by neutrophils.
Diagnosis
Diagnosis and differentiation of children with neurobehavioral syndrome
Laboratory tests are helpful for the diagnosis of this condition. Skin tests are simple and specific, and evoked potentials are also a useful test.
It must be differentiated from Mollaret meningitis, Harada syndrome, and neurosarcoma during the diagnosis.
