A case of Neuro-Behçet’s disease presenting with cognitive dysfunction： a case report

A case of Neuro-Behçet’s disease presenting with cognitive dysfunction： a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report A case of Neuro-Behçet’s disease presenting with cognitive dysfunction： a case report Yuelin Zhou, Lili Tang, Yi Dong, Qing Zhang This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8186304/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Neuro-Behçet’s disease(NBD) is a multisystemic disease characterized by a wide array of clinical manifestations. The diagnostic process is inherently complex and necessitates meticulous differentiation from other diseases. In this case report, we detail the meticulous diagnostic process of a young male patient who initially presented with cognitive impairment, ultimately leading to a diagnosis of Neuro-Behçet’s disease. It bears noting that the postive outcome of the patient’s needle prick test has rendered an invaluable contribution to our diagnostic process. Subsequently, the patient encountered complications during the treatment course. Through a retrospective analysis of this case, we have distilled the salient diagnostic and differential diagnostic criteria for Neuro-Behçet’s disease, as well as potential pitfalls to be vigilant about in the therapeutic course. It is our hope that this case report will offer valuable insights and serve as a useful reference for clinicians in their practice. Neuro-Behçet’s disease NBD case Report cognitive dysfunction Figures Figure 1 Background Behçet’s disease is a multisystem vasculitis of unknown etiology, typically manifesting as ocular inflammatory lesions, genital aphthous ulcers, oral aphthous ulcers, cutaneous manifestations, and vascular involvement. Additionally, it may involve the heart, lungs, nervous system, and gastrointestinal tract. Neurological involvement, known as Neuro-Behçet’s disease (NBD), with reported incidence rates ranging from 2.3% to 44% and an estimated 10-year mortality rate of approximately 10%[1], is one of the leading causes of long-term morbidity and mortality in Behçet’s syndrome. Despite its rarity, recognizing and differentiating NBD from other inflammatory, infectious, and demyelinating central nervous system disorders is crucial[2]. Herein, we present a case of NBD diagnosed following a careful differential diagnosis process. Case Presentation A 21-year-old male presented with dizziness and memory impairment persisting for nine days. His medical history included an upper respiratory tract infection characterized by rhinorrhea, preceding the onset of dizziness, weakness, and cognitive decline. Upon admission, a bedside neurological assessment revealed deficits in both recent and remote memory, as evidenced by the patient’s inability to perform basic arithmetic calculations or accurately recall his academic status. An initial cranial MRI conducted locally showed “scattered abnormal signals within the pons, bilateral thalami, left basal ganglia, and periventricular regions, suggestive of ischemic foci or demyelinating changes.” Dermatological examination revealed papulopustular eruptions on the face, back, and chest, with some lesions exhibiting central pustulation surrounded by erythematous halos (Fig. 1 , a, b). Neurological assessment indicated a positive palmomental reflex, with normal muscle strength and tone in all limbs, intact deep and superficial sensations as well as composite sensations, no abnormalities detected in cranial nerve examination, symmetrical tendon reflexes, normal coordination movements, and absence of other pathological signs, with negative meningeal irritation signs. The patient had no history of significant prior illnesses, denied a history of smoking or alcohol consumption, and reported no family history of autoimmune diseases. Following admission, we formulated a provisional diagnosis of intracranial pathology, with differential considerations including infectious etiologies and demyelinating disorders. A comprehensive diagnostic workup was initiated, including serological assays, lumbar puncture for cerebrospinal fluid analysis, and cognitive function evaluation. Laboratory blood tests indicated a WBC count of 8.7 × 10^9/L, with an absolute neutrophil count of 5.76 × 10^9/L, representing a proportion of 66.1%. The absolute lymphocyte count was 2.02 × 10^9/L, constituting 23.3% of the total. CRP was 11.7 mg/L, ESR was 13 mm/h, and IL-6 concentration was 308 ng/L. Liver function tests showed AST at 13 U/L and ALT at 9 U/L, with GGT at 9 U/L. Albumin was 43.8 g/L, and TBIL was 10.4 µmol/L, with DBIL at 2.1 µmol/L and IBIL at 8.3 µmol/L. Scr was 75.2 µmol/L, GFR was 124.51 mL/min. Immunoglobulins IgA, IgM, IgG, IgG4, and RF showed no significant abnormalities, and C3 at 1.24 g/L, C4 at 538 mg/L. Blood tests showed inflammatory changes. A follow-up enhanced MRI of the brain at our institution revealed “patchy long T1 and long T2 signal lesions in the brainstem and bilateral basal ganglia, with slightly restricted diffusion on DWI and nodular enhancement observed after contrast administration. This suggested the possibility of infectious granulomatous changes or demyelinating alterations” (Fig. 1 , C, D). Lumbar puncture revealed a colorless and clear CSF opening pressure of 115 mmH 2 O, with a nucleated cell count of 15×10^6/L, neutrophils accounting for 27%, lymphocytes accounting for 73%, protein content at 0.318 g/L, glucose level at 2.94 mmol/L, chloride ion level at 122 mmol/L, and the acid-fast bacillus smear and bacterial smear were negative. CSF data revealed signs of inflammation. Cognitive function-related scales showed an MMSE score of 25 and an MOCA score of 24 (the patient was a third-year undergraduate student). The patient’s clinical presentation and radiological findings led to a consideration of demyelinating disorders, particularly Multiple Sclerosis (MS). The distribution of lesions localized to the brainstem and bilateral basal ganglia, combined with the absence of significant abnormalities on cervical spine MRI. Based on the 2017 McDonald Criteria for MS diagnosis[ 3 ] and the MAGNIMS consensus guidelines[ 4 ], the typical lesion locations (juxtacortical, periventricular, infratentorial, spinal cord, and optic nerve) were insufficient in number. The patient’s disease course did not meet the criteria for dissemination in time and space, and CSF analysis showed negative results for the CNS demyelinating panel, including anti-MOG antibodies, making MS unlikely. Although the patient had not undergone histological biopsy, the cranial MRI findings indicated no involvement of the leptomeninges, pituitary gland, or hypothalamic region. The CSF protein, glucose, and lymphocyte counts were within the normal range, thus neurosarcoidosis (NS) was ruled out[ 5 ]. Given the patient's infection history and accompanying dizziness, CNS infections were considered. However, CSF analysis showed clear, colorless fluid with normal glucose and protein levels, negative bacterial and mycobacterial smears, and no fever at admission, ruling out CNS infections, including mycobacterial infections[ 2 ]. The patient exhibited a decline in cognitive abilities, along with pustular and papular dermatitis on the face and torso. However, the absence of recurrent episodes of meningoencephalitis and the lack of fever throughout the disease course, coupled with the presence of destructive vasculitic features in the skin lesions, militated against the diagnosis of Neuro-Sweet’s Disease (NSD)[ 6 ]. Upon further historical inquiry, the patient reported recurrent oral ulcers occurring more than three times per year (Fig. 1 , E, F). A pathergy test was positive, and ophthalmological examination showed no uveitis. Cognitive assessment with MMSE and MoCA suggested mild cognitive impairment. According to the 2014 ICBD criteria[ 7 ], the patient exhibited the following characteristics: recurrent oral ulcers (2 points), cutaneous lesions (1 point), neurological involvement (1 point), and positive pathergy reaction (1 point), totaling 5 points, which met the diagnostic criteria for Behçet’s disease. With symptoms of dizziness and cognitive decline, MRI findings involving the brainstem and basal ganglia, and CSF analysis showing increased cells and 27% neutrophils as indicators of inflammatory changes [ 8 ], a diagnosis of NBD was considered. Prior to establishing a definitive diagnosis, we considered the possibility of autoimmune diseases that can cause primary or secondary central nervous system vasculitis, such as Systemic Lupus Erythematosus (SLE) and Primary Sjögren’s Syndrome (pSS). In accordance with the 2019 EULAR/ACR criteria[ 9 ], the patient presented with oral ulcers (2 points) and memory impairment (2 points). However, the patient’s skin lesions did not align with the characteristics of subacute cutaneous lupus erythematosus or discoid lupus. Furthermore, the patient had not exhibited fever, hematological abnormalities, psychiatric symptoms, or epileptic seizures during the course of the disease. There was no evidence of non-scarring alopecia, lupus-like skin lesions, serosal involvement, musculoskeletal involvement, or immune-related antibody positivity, and no renal involvement. In summary, the total score was 4 points, which was less than the 10-point threshold, thus we provisionally ruled out SLE. The patient denied symptoms such as dry eyes and dry mouth, which did not meet the inclusion criteria for pSS[ 10 ], hence pSS was excluded. Following the meticulous differential diagnostic evaluation, we cautiously diagnosed this patient with NBD. The patient was treated with intravenous methylprednisolone 500 mg/day, which was administered for five consecutive days, followed by a gradual tapering. The methylprednisolone was gradually reduced to oral prednisone 60 mg/day, with a planned weekly reduction of 5 mg until a maintenance dose of 40 mg/day was reached. Considering the patient’s neurological involvement, thalidomide 50 mg/day was administered to suppress the production of TNF-α. Due to incomplete resolution of symptoms, cyclophosphamide 0.8 g/m² was added once a month. During the treatment, the patient developed a herpes zoster infection complicated by pneumonia and thrombocytopenia. Acyclovir was administered intravenously for antiviral therapy, and meropenem was combined with SMZ and moxifloxacin for anti-infection treatment. Thrombopoietin was used to elevate platelet counts. Subsequently, cyclophosphamide was discontinued, and adalimumab 40 mg was initiated as a subcutaneous injection every two weeks, along with enhanced infection surveillance during the use of immunosuppressants. The patient’s symptoms improved, and he is now regularly followed up in the rheumatology outpatient clinic at our hospital. Discussion and Conclusions The patient in this case is a 21-year-old male, primarily presenting with dizziness and cognitive dysfunction, which persisted for 9 days. It is not common for NBD to present with cognitive impairment as the main symptom. Additionally, the patient lacked the typical ocular involvement and genital ulcers, making the diagnosis more challenging. Ocular symptoms, which are frequent in NBD, are predominantly bilateral, with posterior and anterior uveitis being the most common in adults[ 11 ]. However, patients with NBD may not exhibit ocular symptoms, necessitating a comprehensive assessment based on other systemic manifestations. The neurological manifestations of NBD lack specificity, which can lead to confusion with a range of other conditions such as MS and NSD and so on. The absence of specific laboratory markers further complicates the diagnostic process. In this case, the patient’s MRI findings and clinical symptoms overlapped with diseases such as MS, NS and CNS infections, increasing the complexity of differential diagnosis. The patient’s cerebrospinal fluid examination and imaging results were nonspecific, necessitating an exhaustive process of exclusion to establish a definitive diagnosis. It is important to emphasize in the differential diagnosis that both NSD and NBD present with cutaneous lesions and neurological manifestations. However, NSD is often associated with recurrent episodes of encephalitis or meningitis, fevers above 38°C, predominant painful erythema, and a significant elevation of neutrophils, characteristics that were not present in this case. A positive pathergy test is a hallmark clinical sign of Behçet’s disease, yet it is not universally present in all affected individuals. This patient’s positive test outcome served as substantial diagnostic corroboration. Reflecting on the diagnostic trajectory, the patient experienced multiple episodes of oral aphthous ulcers and “acneiform” cutaneous lesions, which regrettably did not receive the requisite attention. This underscores the importance of maintaining a high index of suspicion for systemic pathologies in the presence of dermatological and mucocutaneous manifestations. The patient’s subsequent development of a herpes zoster infection during cyclophosphamide therapy underscores the inherent risks of immunosuppressive regimens, mandating vigilant surveillance and meticulous management to mitigate potential complications. Clinical literature documents instances of NBD misdiagnosed as demyelinating disorders, encephalitis, cerebral infarction, and Sweet syndrome, which can lead to inappropriate treatment regimens, exacerbating disease progression and prolonging morbidity. The consequences of misdiagnosis can significantly impact physical functionality, psychological well-being, and quality of life. This case underscores the importance of considering the possibility of NBD in young male patients presenting with cognitive dysfunction and cutaneous lesions. Additionally, early diagnosis and intervention can significantly improve prognosis. Abbreviations NBD Neuro—Behçet’s disease MS Multiple Sclerosis NS neurosarcoidosis NSD Neuro—Sweet’s Disease SLE Systemic Lupus Erythematosus pSS Primary Sjögren’s Syndrome Declarations Ethics approval and consent to participate This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of Kangci Hospital of Jiaxing, Jiaxing (Date 2025.02.14/No.2025(C-004)). Consent for publication The patient gave his informed consent prior to his inclusion in the study. Informed consent was obtained from the individual participant included in the study. Funding The authors did not receive support from any organization for the submitted work. Author Contribution All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by YZ and LT. The first draft of the manuscript was written by YZ and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript. Acknowledgements Not applicable. Clinical trial number: not applicable. Data Availability The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request. References Zheng W, Zhang N, Zhu X, et al. Guidelines for the Diagnosis and Treatment of Behçet’s Syndrome. Chin J Intern Med. 2021;60(10):860–7. 10.3760/cma.j.cn112138-20210604-00398 . Moretti R. Focus on neuro-Behçet’s disease: A review. Neurol India. 2018;66(6):1619. 10.4103/0028-3886.246252 . Internationalstudygroupforbehc. Criteria for diagnosis of Behcet’s disease. Lancet. 1990;335(8697). 10.1016/0140-6736(90)92643-V . Filippi M, Rocca MA, Ciccarelli O, et al. MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. Lancet Neurol. 2016;15(3):292–303. 10.1016/S1474-4422(15)00393-2 . Stern BJ, Royal W 3rd, Gelfand JM, et al. Definitionand ConsensusDiagnosticCriteriafor Neurosarcoidosis: From the NeurosarcoidosisConsortiumConsensusGroup. JAMA Neurol. 2018;75(12):1546–53. Jin X, Tian L, Teng W. Advances in the Diagnosis and Treatment of Neuro-Sweet’s Disease. Chin J Neurol. 2022;21(3):312–5. 10.3760/cma.j.cn115354-20211103-00709 . International Team for the Revision of the International Criteria for Behcet′s Disease. The International Criteria for Behcet′s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria[J]. J Eur Acad Dermatol Venereol. 2014;28(3):338–47. 10.1111/jdv.12107 . Kalra S, Silman A, Akman-Demir G, et al. Diagnosis and management of Neuro-Behçet’s disease: international consensus recommendations. J Neurol. 2014;261(9):1662–76. 10.1007/s00415-013-7209-3 . Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019;71(9):1400–12. 10.1002/art.40930 . Shiboski CH, Shiboski SC, Seror R, et al. Arthritis Rheumatol. 2017;69(1):35–45. 10.1002/art.39859 . 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Turk MA, Hayworth JL, Nevskaya T, et al. Ocular manifestations of Behçet’s disease in children and adults: a systematic review and meta-analysis. Clin Exp Rheumatol. 2021;39(5):94–101. 10.55563/clinexprheumatol/pt60bc . Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-8186304","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":561344848,"identity":"d465faf2-fbb6-4c3c-8131-86475168064c","order_by":0,"name":"Yuelin Zhou","email":"","orcid":"","institution":"Kangci Hospital of Jiaxing","correspondingAuthor":false,"prefix":"","firstName":"Yuelin","middleName":"","lastName":"Zhou","suffix":""},{"id":561344849,"identity":"71622fe3-6b9b-41e3-b475-fee5595a962e","order_by":1,"name":"Lili Tang","email":"","orcid":"","institution":"Zhejiang 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1","display":"","copyAsset":false,"role":"figure","size":632624,"visible":true,"origin":"","legend":"\u003cp\u003eThe patient experienced multiple cutaneous lesions (a, b). Contrast-enhanced cranial MRI revealed that the lesions were predominantly localized to the brainstem and basal ganglia regions (c, d). The patient had recurrent oral ulcers (e, f).\u003c/p\u003e","description":"","filename":"floatimage1.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8186304/v1/4b4da9b651199ede4ef11e0d.jpeg"},{"id":100610118,"identity":"c5eb2d0c-ddba-4eba-8f2a-f0765c060a58","added_by":"auto","created_at":"2026-01-19 16:30:06","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":960942,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8186304/v1/c5d9cb66-9c1c-4a74-82f3-f57799623c31.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"A case of Neuro-Behçet’s disease presenting with cognitive dysfunction： a case report","fulltext":[{"header":"Background","content":"\u003cp\u003eBehçet’s disease is a multisystem vasculitis of unknown etiology, typically manifesting as ocular inflammatory lesions, genital aphthous ulcers, oral aphthous ulcers, cutaneous manifestations, and vascular involvement. Additionally, it may involve the heart, lungs, nervous system, and gastrointestinal tract. Neurological involvement, known as Neuro-Behçet’s disease (NBD), with reported incidence rates ranging from 2.3% to 44% and an estimated 10-year mortality rate of approximately 10%[1], is one of the leading causes of long-term morbidity and mortality in Behçet’s syndrome. Despite its rarity, recognizing and differentiating NBD from other inflammatory, infectious, and demyelinating central nervous system disorders is crucial[2]. Herein, we present a case of NBD diagnosed following a careful differential diagnosis process.\n\n\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 21-year-old male presented with dizziness and memory impairment persisting for nine days. His medical history included an upper respiratory tract infection characterized by rhinorrhea, preceding the onset of dizziness, weakness, and cognitive decline. Upon admission, a bedside neurological assessment revealed deficits in both recent and remote memory, as evidenced by the patient\u0026rsquo;s inability to perform basic arithmetic calculations or accurately recall his academic status. An initial cranial MRI conducted locally showed \u0026ldquo;scattered abnormal signals within the pons, bilateral thalami, left basal ganglia, and periventricular regions, suggestive of ischemic foci or demyelinating changes.\u0026rdquo; Dermatological examination revealed papulopustular eruptions on the face, back, and chest, with some lesions exhibiting central pustulation surrounded by erythematous halos (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e, a, b). Neurological assessment indicated a positive palmomental reflex, with normal muscle strength and tone in all limbs, intact deep and superficial sensations as well as composite sensations, no abnormalities detected in cranial nerve examination, symmetrical tendon reflexes, normal coordination movements, and absence of other pathological signs, with negative meningeal irritation signs. The patient had no history of significant prior illnesses, denied a history of smoking or alcohol consumption, and reported no family history of autoimmune diseases.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eFollowing admission, we formulated a provisional diagnosis of intracranial pathology, with differential considerations including infectious etiologies and demyelinating disorders. A comprehensive diagnostic workup was initiated, including serological assays, lumbar puncture for cerebrospinal fluid analysis, and cognitive function evaluation. Laboratory blood tests indicated a WBC count of 8.7 \u0026times; 10^9/L, with an absolute neutrophil count of 5.76 \u0026times; 10^9/L, representing a proportion of 66.1%. The absolute lymphocyte count was 2.02 \u0026times; 10^9/L, constituting 23.3% of the total. CRP was 11.7 mg/L, ESR was 13 mm/h, and IL-6 concentration was 308 ng/L. Liver function tests showed AST at 13 U/L and ALT at 9 U/L, with GGT at 9 U/L. Albumin was 43.8 g/L, and TBIL was 10.4 \u0026micro;mol/L, with DBIL at 2.1 \u0026micro;mol/L and IBIL at 8.3 \u0026micro;mol/L. Scr was 75.2 \u0026micro;mol/L, GFR was 124.51 mL/min. Immunoglobulins IgA, IgM, IgG, IgG4, and RF showed no significant abnormalities, and C3 at 1.24 g/L, C4 at 538 mg/L. Blood tests showed inflammatory changes. A follow-up enhanced MRI of the brain at our institution revealed \u0026ldquo;patchy long T1 and long T2 signal lesions in the brainstem and bilateral basal ganglia, with slightly restricted diffusion on DWI and nodular enhancement observed after contrast administration. This suggested the possibility of infectious granulomatous changes or demyelinating alterations\u0026rdquo; (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e, C, D). Lumbar puncture revealed a colorless and clear CSF opening pressure of 115 mmH\u003csub\u003e2\u003c/sub\u003eO, with a nucleated cell count of 15\u0026times;10^6/L, neutrophils accounting for 27%, lymphocytes accounting for 73%, protein content at 0.318 g/L, glucose level at 2.94 mmol/L, chloride ion level at 122 mmol/L, and the acid-fast bacillus smear and bacterial smear were negative. CSF data revealed signs of inflammation. Cognitive function-related scales showed an MMSE score of 25 and an MOCA score of 24 (the patient was a third-year undergraduate student).\u003c/p\u003e \u003cp\u003eThe patient\u0026rsquo;s clinical presentation and radiological findings led to a consideration of demyelinating disorders, particularly Multiple Sclerosis (MS). The distribution of lesions localized to the brainstem and bilateral basal ganglia, combined with the absence of significant abnormalities on cervical spine MRI. Based on the 2017 McDonald Criteria for MS diagnosis[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] and the MAGNIMS consensus guidelines[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e], the typical lesion locations (juxtacortical, periventricular, infratentorial, spinal cord, and optic nerve) were insufficient in number. The patient\u0026rsquo;s disease course did not meet the criteria for dissemination in time and space, and CSF analysis showed negative results for the CNS demyelinating panel, including anti-MOG antibodies, making MS unlikely. Although the patient had not undergone histological biopsy, the cranial MRI findings indicated no involvement of the leptomeninges, pituitary gland, or hypothalamic region. The CSF protein, glucose, and lymphocyte counts were within the normal range, thus neurosarcoidosis (NS) was ruled out[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Given the patient's infection history and accompanying dizziness, CNS infections were considered. However, CSF analysis showed clear, colorless fluid with normal glucose and protein levels, negative bacterial and mycobacterial smears, and no fever at admission, ruling out CNS infections, including mycobacterial infections[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. The patient exhibited a decline in cognitive abilities, along with pustular and papular dermatitis on the face and torso. However, the absence of recurrent episodes of meningoencephalitis and the lack of fever throughout the disease course, coupled with the presence of destructive vasculitic features in the skin lesions, militated against the diagnosis of Neuro-Sweet\u0026rsquo;s Disease (NSD)[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eUpon further historical inquiry, the patient reported recurrent oral ulcers occurring more than three times per year (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e, E, F).\u003c/p\u003e \u003cp\u003eA pathergy test was positive, and ophthalmological examination showed no uveitis. Cognitive assessment with MMSE and MoCA suggested mild cognitive impairment. According to the 2014 ICBD criteria[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e], the patient exhibited the following characteristics: recurrent oral ulcers (2 points), cutaneous lesions (1 point), neurological involvement (1 point), and positive pathergy reaction (1 point), totaling 5 points, which met the diagnostic criteria for Beh\u0026ccedil;et\u0026rsquo;s disease. With symptoms of dizziness and cognitive decline, MRI findings involving the brainstem and basal ganglia, and CSF analysis showing increased cells and 27% neutrophils as indicators of inflammatory changes [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e], a diagnosis of NBD was considered.\u003c/p\u003e \u003cp\u003ePrior to establishing a definitive diagnosis, we considered the possibility of autoimmune diseases that can cause primary or secondary central nervous system vasculitis, such as Systemic Lupus Erythematosus (SLE) and Primary Sj\u0026ouml;gren\u0026rsquo;s Syndrome (pSS). In accordance with the 2019 EULAR/ACR criteria[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e], the patient presented with oral ulcers (2 points) and memory impairment (2 points). However, the patient\u0026rsquo;s skin lesions did not align with the characteristics of subacute cutaneous lupus erythematosus or discoid lupus. Furthermore, the patient had not exhibited fever, hematological abnormalities, psychiatric symptoms, or epileptic seizures during the course of the disease. There was no evidence of non-scarring alopecia, lupus-like skin lesions, serosal involvement, musculoskeletal involvement, or immune-related antibody positivity, and no renal involvement. In summary, the total score was 4 points, which was less than the 10-point threshold, thus we provisionally ruled out SLE. The patient denied symptoms such as dry eyes and dry mouth, which did not meet the inclusion criteria for pSS[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e], hence pSS was excluded.\u003c/p\u003e \u003cp\u003eFollowing the meticulous differential diagnostic evaluation, we cautiously diagnosed this patient with NBD.\u003c/p\u003e \u003cp\u003eThe patient was treated with intravenous methylprednisolone 500 mg/day, which was administered for five consecutive days, followed by a gradual tapering. The methylprednisolone was gradually reduced to oral prednisone 60 mg/day, with a planned weekly reduction of 5 mg until a maintenance dose of 40 mg/day was reached. Considering the patient\u0026rsquo;s neurological involvement, thalidomide 50 mg/day was administered to suppress the production of TNF-α. Due to incomplete resolution of symptoms, cyclophosphamide 0.8 g/m\u0026sup2; was added once a month. During the treatment, the patient developed a herpes zoster infection complicated by pneumonia and thrombocytopenia. Acyclovir was administered intravenously for antiviral therapy, and meropenem was combined with SMZ and moxifloxacin for anti-infection treatment. Thrombopoietin was used to elevate platelet counts. Subsequently, cyclophosphamide was discontinued, and adalimumab 40 mg was initiated as a subcutaneous injection every two weeks, along with enhanced infection surveillance during the use of immunosuppressants. The patient\u0026rsquo;s symptoms improved, and he is now regularly followed up in the rheumatology outpatient clinic at our hospital.\u003c/p\u003e"},{"header":"Discussion and Conclusions","content":"\u003cp\u003eThe patient in this case is a 21-year-old male, primarily presenting with dizziness and cognitive dysfunction, which persisted for 9 days. It is not common for NBD to present with cognitive impairment as the main symptom. Additionally, the patient lacked the typical ocular involvement and genital ulcers, making the diagnosis more challenging. Ocular symptoms, which are frequent in NBD, are predominantly bilateral, with posterior and anterior uveitis being the most common in adults[\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. However, patients with NBD may not exhibit ocular symptoms, necessitating a comprehensive assessment based on other systemic manifestations.\u003c/p\u003e \u003cp\u003eThe neurological manifestations of NBD lack specificity, which can lead to confusion with a range of other conditions such as MS and NSD and so on. The absence of specific laboratory markers further complicates the diagnostic process. In this case, the patient\u0026rsquo;s MRI findings and clinical symptoms overlapped with diseases such as MS, NS and CNS infections, increasing the complexity of differential diagnosis. The patient\u0026rsquo;s cerebrospinal fluid examination and imaging results were nonspecific, necessitating an exhaustive process of exclusion to establish a definitive diagnosis. It is important to emphasize in the differential diagnosis that both NSD and NBD present with cutaneous lesions and neurological manifestations. However, NSD is often associated with recurrent episodes of encephalitis or meningitis, fevers above 38\u0026deg;C, predominant painful erythema, and a significant elevation of neutrophils, characteristics that were not present in this case. A positive pathergy test is a hallmark clinical sign of Beh\u0026ccedil;et\u0026rsquo;s disease, yet it is not universally present in all affected individuals. This patient\u0026rsquo;s positive test outcome served as substantial diagnostic corroboration.\u003c/p\u003e \u003cp\u003eReflecting on the diagnostic trajectory, the patient experienced multiple episodes of oral aphthous ulcers and \u0026ldquo;acneiform\u0026rdquo; cutaneous lesions, which regrettably did not receive the requisite attention. This underscores the importance of maintaining a high index of suspicion for systemic pathologies in the presence of dermatological and mucocutaneous manifestations.\u003c/p\u003e \u003cp\u003eThe patient\u0026rsquo;s subsequent development of a herpes zoster infection during cyclophosphamide therapy underscores the inherent risks of immunosuppressive regimens, mandating vigilant surveillance and meticulous management to mitigate potential complications.\u003c/p\u003e \u003cp\u003eClinical literature documents instances of NBD misdiagnosed as demyelinating disorders, encephalitis, cerebral infarction, and Sweet syndrome, which can lead to inappropriate treatment regimens, exacerbating disease progression and prolonging morbidity. The consequences of misdiagnosis can significantly impact physical functionality, psychological well-being, and quality of life.\u003c/p\u003e \u003cp\u003eThis case underscores the importance of considering the possibility of NBD in young male patients presenting with cognitive dysfunction and cutaneous lesions. Additionally, early diagnosis and intervention can significantly improve prognosis.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eNBD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eNeuro\u0026mdash;Beh\u0026ccedil;et\u0026rsquo;s disease\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eMS\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eMultiple Sclerosis\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eNS\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eneurosarcoidosis\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eNSD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eNeuro\u0026mdash;Sweet\u0026rsquo;s Disease\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eSLE\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eSystemic Lupus Erythematosus\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003epSS\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003ePrimary Sj\u0026ouml;gren\u0026rsquo;s Syndrome\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e \u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e \u003cp\u003eThis study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of Kangci Hospital of Jiaxing, Jiaxing (Date 2025.02.14/No.2025(C-004)).\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eConsent for publication\u003c/strong\u003e \u003cp\u003eThe patient gave his informed consent prior to his inclusion in the study.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eInformed consent\u003c/strong\u003e \u003cp\u003ewas obtained from the individual participant included in the study.\u003c/p\u003e \u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e \u003cp\u003eThe authors did not receive support from any organization for the submitted work.\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eAll authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by YZ and LT. The first draft of the manuscript was written by YZ and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.\u003c/p\u003e\u003ch2\u003eAcknowledgements\u003c/h2\u003e \u003cp\u003eNot applicable.\u003c/p\u003e \u003cp\u003e \u003cem\u003eClinical trial number: not applicable.\u003c/em\u003e \u003c/p\u003e\u003ch2\u003eData Availability\u003c/h2\u003e\u003cp\u003eThe datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eZheng W, Zhang N, Zhu X, et al. Guidelines for the Diagnosis and Treatment of Beh\u0026ccedil;et\u0026rsquo;s Syndrome. 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Clin Exp Rheumatol. 2021;39(5):94\u0026ndash;101. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.55563/clinexprheumatol/pt60bc\u003c/span\u003e\u003cspan address=\"10.55563/clinexprheumatol/pt60bc\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Neuro-Behçet’s disease, NBD, case Report, cognitive dysfunction","lastPublishedDoi":"10.21203/rs.3.rs-8186304/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8186304/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eNeuro-Behçet’s disease(NBD) is a multisystemic disease characterized by a wide array of clinical manifestations. The diagnostic process is inherently complex and necessitates meticulous differentiation from other diseases. In this case report, we detail the meticulous diagnostic process of a young male patient who initially presented with cognitive impairment, ultimately leading to a diagnosis of Neuro-Behçet’s disease. It bears noting that the postive outcome of the patient’s needle prick test has rendered an invaluable contribution to our diagnostic process. Subsequently, the patient encountered complications during the treatment course. Through a retrospective analysis of this case, we have distilled the salient diagnostic and differential diagnostic criteria for Neuro-Behçet’s disease, as well as potential pitfalls to be vigilant about in the therapeutic course. It is our hope that this case report will offer valuable insights and serve as a useful reference for clinicians in their practice.\u003c/p\u003e","manuscriptTitle":"A case of Neuro-Behçet’s disease presenting with cognitive dysfunction： a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-12-22 09:38:57","doi":"10.21203/rs.3.rs-8186304/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"b90fcb16-e1a9-494a-b6d2-7fd4afefa905","owner":[],"postedDate":"December 22nd, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-01-19T15:29:33+00:00","versionOfRecord":[],"versionCreatedAt":"2025-12-22 09:38:57","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8186304","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8186304","identity":"rs-8186304","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}