Headache for August, weakness of the right limb with speech failure for 10 days, what do you think about it?

*Only for medical professionals to read and refer to the main features, diagnostic procedures, and medications of 8 infectious central nervous system vasculitis.
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The 24th Chinese Medical Association held on September 24-26, 2021 At the National Conference on Neurology, Professor Zhang Xinghu from the Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, brought us a wonderful case, and through the case, the diagnosis and treatment of infectious central nervous system (CNS) vasculitis In-depth explanation, "Medical Neurology Channel" organizes the lecture content as follows
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Picture 1: Professor Zhang Xinghu’s report of the case review Let’s take a look at a case together.
The patient is a 22-year-old woman who was admitted to the hospital for “headache for 8 months, weakness of the right limb with speech failure for 10 days”
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▌ Onset process: 8 months before admission: headache and intermittent fever
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5 months before admission: amenorrhea
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20 days before admission: The patient developed high fever (39°C), polyuria (6000-7000ml/d) and decreased right vision
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10 days before admission: The patient developed weakness on the right side of the body, unable to speak, but was able to understand other people's words
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▌ Physical examination on admission: complete motor aphasia, decreased acupuncture sensation on the right side of the face, shallow nasolabial folds on the right, grade 4 muscle strength on the right side, positive Pap sign on the right side, and decreased acupuncture sensation on the right side
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▌ blood tests: blood, biochemical, infectious diseases, coagulation and pituitary gonadal hormones, like procalcitonin no exception
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Rheumatic immune antibody spectrum, anti-neutrophil antibody (ANCA), cardiolipin antibody, AQP-4 antibody, autoimmune encephalitis antibody, tuberculosis antibody: negative
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Thyroid function and antibodies: no abnormalities
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The erythrocyte sedimentation rate is 23mm/h
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TB-SPOT: positive
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▌ Cerebrospinal fluid examination: Figure 2: Cerebrospinal fluid examination results for fungus, tuberculosis, bacterial staining and culture: negative
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AQP-4 antibody, autoimmune encephalitis antibody, virus antibody, tuberculosis antibody, TB-SPOT: negative
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Pathogen next-generation sequencing (NGS): negative
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▌ Imaging examination: MRI of the brain: Long T1 and long T2 signal shadows can be seen in the left frontotemporal cortex, and DWI manifests as limited diffusion
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Figure 3: Cranial MRI (T1, T2) Figure 4: Cranial MRI (DWI, ADC, SWI) Figure 5: High-resolution MRI of the brain: Thickening of the wall of the M1 section of the left middle cerebral artery (MCA), lumen Stenosis, enhanced visible tube wall enhancement Figure 6: MRI of sellar area: features of inflammatory changes in sellar area▌ Systemic blood vessel screening: ultrasound of aortic arch, ultrasound of both renal arteries, and arteriovenous ultrasound of extremities: no abnormalities
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▌ Other examinations: chest radiograph, urinary system ultrasound, abdominal ultrasound, echocardiogram: no abnormalities
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Combined with the patient's headache, fever, and rapid erythrocyte sedimentation rate in experimental examinations, TB-SPOT is positive, and imaging shows that the blood vessel wall is thickened and the lumen is narrow.
It is considered that the patient is central vasculitis caused by tuberculosis infection
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The patient has amenorrhea and polyuria.
MRI showed inflammatory lesions in the sellar area.
Consider the possibility of tuberculous granuloma in the sellar area
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Give empirical treatment, including quadruple anti-tuberculosis (isoniazid, rifapentine, pyrazinamide, levofloxacin), hormones (start with methylprednisolone 500mg, gradually reduce the dose and change to oral prednisone), mannitol , Desmopressin acetate tablets
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Three months after the patient was discharged from the hospital, the headache symptoms improved significantly, and no fever appeared.
After reviewing the imaging, the frontotemporal lobe lesions were better than before, and most of the lesions were softened
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Further confirm the diagnosis
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Final diagnosis: infectious CNS vasculitis (tuberculous granuloma in sellar area)
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Infectious CNS vasculitis is not common clinically.
It is an inflammatory change of the cerebral vascular wall caused by various infectious microorganisms.
It often involves the brain parenchyma and the small and medium blood vessels of the meninges.
Large blood vessels can also be affected
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The disease causes ischemia, thrombosis, vasospasm and hemorrhagic lesions in the corresponding blood supply area
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The incidence rate accounts for about 0.
5% of the causes of stroke, and about 32% of non-atherosclerotic large-medium vascular cerebrovascular diseases are caused by infection
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The clinical manifestations of infectious CNS vasculitis (infectious CNS vasculitis) are complex and changeable, and it is easy to be missed or misdiagnosed in the clinic
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▌ pathogenesis direct reasons: direct damage to the vascular wall of infectious agents, including Staphylococcus, Streptococcus, Treponema, cytomegalovirus (CMV), herpes simplex virus (HSV) -1 and Type 2 and the like
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Indirect reasons: reaction vessel wall damage by stimulating the immune, including tuberculosis, hepatitis B virus (HBV), human immunodeficiency virus (HIV) and so on
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These microorganisms have common epitopes with the host or can modify self-antigens to cause cross-over immune responses
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Professor Zhang Xinghu introduced that, in fact, it is difficult to distinguish the two.
Most of the time, the two mechanisms coexist
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▌ Clinical type 1.
Streptococcus pneumoniae CNS vasculitis, old age, pneumonia, external ear infection, sinusitis, immunocompromised risk factors; more common in infective endocarditis, sepsis, about 25% secondary to meningitis, can be Occurs several weeks after bacterial infection; anterior circulation vasculitis is more common; aneurysm is prone to form, accounting for about 2.
5%-4.
5% of intracranial aneurysms, and death from aneurysm rupture reaches 40%-80%; subarachnoid hemorrhage may occur , Subdural hematoma
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Figure 7: Imaging manifestations of Streptococcus pneumoniae CNS vasculitis 2.
Tuberculous CNS vasculitis tuberculous exudate causes inflammation of MCA, arterioles, capillaries and veins, forming cerebral infarction, hemangioma and focal hemorrhage; often Involve the MCA and subsegmental area, pons, thalamus and perforating artery in the internal capsule area; 13-35% of the infarcts can be seen on CT, and up to 57% on MRI
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Figure 8: Imaging manifestations of tuberculous CNS vasculitis A.
Tuberculoma, ventriculitis; B.
Infarct in the left basal region; C.
Bilateral MCA, posterior cerebral artery, and pericardial artery stenosis
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3.
Spirochetal CNS vasculitis alone is rare (0.
3%-1%); often combined with cerebral (spinal) meningitis, radiculitis, large, medium and small blood vessels can be involved, and multiple focal Vasculitis; skin rashes are common, and 50% have a history of tick bites
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Figure 9: Imaging manifestations of burgdorferi's CNS vasculitis A B.
Left pontine cerebellar infarction; C.
Shows irregular stenosis of the upper third of the basilar artery
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4.
Syphilitic CNS vasculitis is mostly middle-aged and elderly.
Symptoms appear several months to several years after infection, subacute or chronic onset, and often coexists with HIV infection; meningeal vascular syphilis accounts for 40%-60% of all neurosyphilis and can be involved Any cerebrovascular, causing cerebrovascular stenosis and cerebral infarction; clinical manifestations such as headache, dizziness, seizures, hemiplegia, hemisensory disturbance, hemianopia, aphasia, cognitive decline, and changes in mood and behavior, etc.
; it is migratory, cerebrovascular Check for abnormal changes or not
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Figure 10: Imaging findings of syphilitic CNS vasculitis.
Left MRA: irregular stenosis of the basilar artery (long arrow) and undeveloped right vertebral artery (short arrow); right DSA shows stenosis of the left internal carotid artery
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5.
Fungal CNS vasculitis is mostly spread from blood sources (heart, lung, skin lesions); vasculitis causes cerebral infarction, subarachnoid space (aneurysm rupture), and aneurysms are more common in intracranial arteries far away End branch; MRI shows that the infarct is mainly located in the gray matter junction area, and the perforating arteries may also be involved; common CNS infectious fungi: Aspergillus is the most common, common in patients with AIDS, leukemia, tuberculosis, cancer or alcoholism, and the lesion is located in the thalamus, The basal section and the corpus callosum
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Cryptococcal meningitis combined with vasculitis, cerebral infarction is common in the basal segment, internal capsule and thalamus; Figure 11: Aspergillus infectious CNS hematitis: a shows gray matter junction disease; b, c show multiple occurrences Infarct focus
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Figure 12: Cryptococcal infectious CNS vasculitis: multiple infarcts (bottom segment area, left occipital lobe), DSA and CTA are normal
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6.
Herpes zoster CNS vasculitis is more common in the elderly; early herpes zoster infection may occur; arterial intimal thickening, vascular occlusion and ischemia can form aneurysms and hemorrhage; unilateral MCA branch vessel segmental stenosis ( M1 is more common), internal carotid artery (ICA) stenosis (rare), deep perforating branch may also be involved; cerebral infarction or TIA may occur
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Figure 13: Herpes zoster CNS vasculitis A: DSA shows severe stenosis at the end of the left internal carotid artery; B.
The stenosis is reduced after hormone and aprovir treatment; CDHR-MRI shows that the LICA tube wall has increased concentric circular tube wall Thick and strengthened
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7.
HIV CNS vasculitis can invade within 1 week after systemic infection; CNS can develop at any age; opportunistic infections, acquired immunodeficiency syndrome (AIDS) encephalopathy dominate (syphilis is the most common); HIV infection It can increase the risk of stroke; vasculitis leads to thinning of the middle layer of the artery, MCA occlusion with or without tumor-like vasodilation and vascular stenosis, and ICA occlusion is rare; it can lead to large-scale cerebral infarction
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Figure 14: HIV-induced CNS vasculitis A.
Lumbar death in the blood supply area of ​​the anterior cerebral artery; D.
LACA strengthening of the distal branch vessels; January 8 after GJ antiretroviral therapy.
Cerebral cysticercosis CNS vasculitis is reported in patients with active cysticercosis The incidence in the brain is 4%-12%; in patients with subarachnoid cysticercosis, the incidence of vasculitis is up to 20%, but there is often no cerebral infarction; vasculitis can cause segmental stenosis and occlusion of blood vessels, and brain Infarction; can also lead to aneurysm formation and bleeding; common parts of stroke: internal capsule hind limbs, radiation crown, brain stem; clinical manifestations: lacunar syndrome (such as simple migraine) is more common, bleeding is rare; CT or MRI can be seen Cerebral infarction or cerebral hemorrhage, MRA shows segmental vascular stenosis or occlusion
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Infectious CNS vasculitis diagnosis process Professor Zhang Xinghu introduced that for the diagnosis of infectious CNS vasculitis, two aspects should be considered in the clinic (Figure 15): First, the diagnosis of vasculitis, when repeated attacks or multiple cerebral infarctions are encountered in the clinic, Cerebral hemorrhage or microhemorrhage, multiple white matter lesions, headache, epilepsy, cognitive and memory decline, etc.
, should consider the possibility of vasculitis
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The second is the possibility of intracranial infection.
When the patient has headache, fever, increased white blood cells in the cerebrospinal fluid, and increased protein, it is not difficult to diagnose intracranial infection
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If both are present, then infectious CNS vasculitis should be highly suspected
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Figure 15: Infectious CNS vasculitis diagnosis process Infectious CNS vasculitis treatment Infectious CNS vasculitis treatment, including anti-infective therapy, hormone application and other treatments
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Anti-infective treatment: according to the classification of pathogenic bacteria, anti-infective treatment drugs and treatment courses are different, see Figure 16 for details
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Figure 16: Anti-infective treatment drugs and hormone use in the course of treatment 1.
Herpes virus infection: prednisone (1mg/kg/d, 5d) or methylprednisolone (500mg, iv qd 3d); 2.
Tuberculosis infection: use as appropriate; 3 Neuro Lyme disease: methylprednisolone (1g) for 5 days or prednisone (1mg/kg) for 2 weeks; 4.
Neurosyphilis: prednisone (60 mg/d) treatment with penicillin for 3 days; 5.
cerebral cysticercosis : Dexamethasone (10-20mg/d), use as appropriate
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Other treatments: including antiplatelet drug therapy, acute stroke thrombolysis, and endovascular or surgical treatment
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Summary: 1.
The clinical features of infectious CNS vasculitis are complex and changeable; 2.
When the patient has cerebral infarction, cerebral hemorrhage and infection symptoms such as fever, the epidemiology, laboratory examinations, and imaging findings should be combined with epidemiology, laboratory examination, and imaging.
The possibility of CNS vasculitis, and pathogen detection such as NGS, etc.
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For patients with complex disease, continuous disease progression or severe disease, brain biopsy can be confirmed if necessary; 4.
Different infections should be treated with different anti-infective treatment plans 5.
The prognosis of CNS vasculitis caused by different infections is different
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