This special type of rheumatism, misdiagnosed as a tumor will be fatal!

*Only for medical professionals to read and refer to the brain burn cases! Have you encountered a situation in which the superficial phenomenon and the actual cause are difficult to associate with each other in clinical practice? Today, the editor brought a case like this to burn your brain! Without further ado, let's go to the case! Case Award - Superficial Tumor? 39 years old, male, native of Morocco
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 The patient presented with left-sided hemiparesis, fever, weight loss, stomatitis aphtha, elbow arthritis, and systemic inflammatory response for 3 weeks
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No complaints of vision discomfort
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He was healthy in the past, with no special medical history, and no family history of similar diseases and genetic diseases
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 This case report [1] provides several relevant findings: slit-lamp examination and macular optical coherence tomography: normal; fundus imaging: showing bilateral vitreitis and retinal vasculitis; retinal angiography: showing venous vasculitis; brain External magnetic resonance imaging (MRI): showed an infiltrative lesion from the outer capsule to the diencephalon with heterogeneity restriction and gadolinium enhancement (Fig.
1A-G); fluorodeoxyglucose-positron tomography (PET-FDG): suggestive Tumor progression (basal ganglia hypermetabolism) (Fig.
1H); CSF examination: 126 leukocytes, 80% lymphocytes, HLA-B51 positive
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Figure 1: The patient's MRI and 18FDG-PET imaging results After reading the content of this case report, whether it is clinical symptoms or brain imaging results, do you feel that it is very consistent with the diagnosis of brain parenchyma? The actual cause - what is invisible is the diagnosis? We might as well classify the patient's current information according to whether it is in line with the diagnosis of the tumor: those consistent with brain parenchymal tumors: left hemiplegia, fever, weight loss, systemic inflammatory response; MRI showing infiltrative lesions from the outer capsule to the diencephalon; PET -FDG suggests tumor progression; not consistent with brain parenchymal tumor manifestations: stomatitis aphtha, elbow arthritis and systemic inflammatory response for 3 weeks; fundus imaging shows bilateral vitreous inflammation and retinal vasculitis; retinal angiography shows venous vasculitis; In cerebrospinal fluid examination, 80% of lymphocytes were positive for HLA-B51
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After this classification, it was found that, in addition to tumor characteristics, most of them seemed to be related to inflammation, especially vasculitis
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Oral ulcers are more common in rheumatic immune diseases, coupled with HLA-B51 positive in cerebrospinal fluid.
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Good guy, this feels a bit like Behçet's disease (BD)! BD, also known as Behçet's disease and oral-eye-genital triad, is a chronic, recurrent, multisystem vascular inflammatory disease triggered by a combination of infection and environmental factors in genetically susceptible individuals
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It is clinically characterized by recurrent oral ulcers, genital ulcers, and ophthalmia, and multiple organs such as the digestive system, cardiovascular system, and nervous system may be involved at the same time [2]
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 So is this patient a brain parenchymal tumor + BD? Or follow "monism" and try to explain all symptoms with one disease? Is it possible that this patient's appearance of a parenchymal tumor is also caused by BD? The editor reviewed the neurological symptoms and manifestations that BD can involve, and actually found something! Neurological lesions in BD patients are called neuro-Behcet syndrome (NBS).
The onset is non-specific and can be manifested as high fever, headache, mental apathy, inattention, and hemiplegia on one side of the limb.
bundle sign
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Physical examination showed positive meningeal irritation sign or positive pyramidal tract sign
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Neuroimaging examinations often show intracranial lesions with low signal on T1-weighted images and high signal on T2-weighted images
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 Isn't this the characteristic of this patient's "brain parenchymal tumor"? It turns out that this "invisible" vasculitis is the culprit of the "tumor-like" symptoms! Reminds of pain - solemnly studying NBS Why does the editor want to talk about pain and remorse here? Because NBS patients who look like tumors, if you follow the treatment of brain parenchyma tumors, the patients are very likely to die in a short period of time.
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But! If BD is correctly diagnosed in time, only hormones and immunosuppressants are needed, and the prognosis can be excellent~ Therefore, let's calm down now, solemnly learn about NBS, and see how to differentiate it from tumors
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 The current clinical diagnostic criteria for NBS are mostly [2]: (1) in line with the 1990 International Study Group BD diagnostic criteria (Table 1); (2) neurological syndrome caused by BD with objective neurological signs and neurological symptoms Imaging and/or cerebrospinal fluid findings support; (3) no other better explanation for neurological manifestations
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 Table 1: 1990 International Study Group Diagnostic Criteria for BD According to clinical and neuroimaging examinations, NBS is generally classified into 3 types [3]: Parenchymal NBS (p-NBS), manifesting as inflammatory parenchymal lesions of the central system, p- According to the clinical course, NBS is further subdivided into: acute p-NBS and chronic progressive p-NBS; non-substantial NBS (np-NBS), manifesting as intracranial vascular disease mainly due to cerebral venous thrombosis; mixed type, The above two conditions exist at the same time, and this type is clinically rare
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The key to distinguish NBS from brain parenchymal tumors lies in the following three auxiliary examinations [3]: ①MRI: MRI is the gold standard for imaging diagnosis of NBS
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On MRI, p-NBS lesions usually involve the telencephalon/diencephalon connectome and the brainstem.
The brainstem lesions are usually large and indistinct, and the lesions may extend into the diencephalon
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In the acute phase of p-NBS, abnormal lesions in the brain parenchyma can be observed on MRI, which are isointense or hypointense on T1-weighted imaging, and hyperintense on T2
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Brainstem atrophy is a characteristic feature of the chronic phase
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 ② Cerebrospinal fluid examination: CSF examination has no obvious specificity in the diagnosis of NBS.
Generally, in the acute stage of p-NBS, the typical cerebrospinal fluid is infiltrated by neutrophils.
After that, it can be transformed into mainly lymphocytes, and the protein level of cerebrospinal fluid can be slightly higher.
15% to 20% of patients may have positive oligoclonal bands
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 ③Nerve biopsy: Pathological findings are not specific, acute or subacute parenchymal involvement manifests as perivascular inflammation, characterized by perivascular infiltration of lymphocytes, neutrophils, and eosinophils (in rare cases), with Or not accompanied by necrosis, and the late inflammatory infiltration is not obvious, mainly axonal loss and gliosis
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 After studying this case, I really sighed that life is full of chicken soup.
Sometimes don't just focus on what you see, what you see is not necessarily true
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 Reference [1] Ion IM, Villesot M, Goulabchand R, Cagnazzo F, Thouvenot E.
Tumefactive lesion revealing neuro-Behçet disease.
Rheumatology (Oxford).
2022 Mar 25:keac194.
doi: 10.
1093/rheumatology/keac194.
Epub ahead of print.
PMID: 35333305.
https://pubmed.
ncbi.
nlm.
nih.
gov/35333305/[2] Guidelines for the diagnosis and treatment of Behcet's disease [J].
Chinese Journal of Rheumatology, 2011(05):345-347 .
https://kns.
cnki.
net/kcms/detail/detail.
aspx?dbcode=CJFD&dbname=CJFDZHYX&filename=FSBZ201105020&v=MjcxMzdJUjhlWDFMdXhZUzdEaDFUM3FUcldNMUZyQ1VSN2lmWk9WdUZDcmdXcnJQSXo3SmRMRzRIOURNcW85SFo=[3] Lu Chui, water Yang Zhou, Zhao Yingchun.
diagnosis and treatment of neurological Behcet's syndrome [J] The medical review, 2020,26 (19):.
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