Unraveling Neuro-Behcet's Disease: Insights from a Case of Acute Brainstem Syndrome and the Clinical Value of the Cascade Sign

Dr. Priyanka Jangam, Dr. N. V. Sundarachary, Dr. G. Bindu Narmada, Dr. M. Bhargavi Devi, Dr. Kamalakar P

Abstract: Introduction: Behcet's disease (BD) is characterized by recurrent oral and genital ulcers, and uveitis. It is also known to affect various organs; however, central nervous system involvement is rare. Association of HLA-B5/B*51 has been recognized as the strongest genetic susceptibility factor for BD. NBD manifestations can be categorized into central nervous system (CNS) and peripheral nervous system (PNS) presentations. CNS manifestations fall into parenchymal and non-parenchymal subtypes. The parenchymal type is more prevalent and presents with brainstem, hemispheric, spinal, and meningoencephalitic syndromes. Case Report: A 35-year-old man presented with a 1-week history of progressive brainstem symptoms including slurred speech, dysphagia, diplopia, and facial weakness. This was followed 2 days later by acute-onset, symmetrical, distal-predominant quadriparesis. He had past history of quadriparesis 5 years prior, with residual foot weakness. On examination patient is Conscious and oriented. Tachypnea and tachycardia were present. Cranial nerve examination revealed palsies of CN III, IV, VI, VII, IX, and X. Motor examination showed symmetrical UMN-type quadriparesis (power 2/5 in all limbs). Materials and Methods: CBC, RFT, LFT, and CSF analysis were normal. ESR and CRP were elevated. Serum NMO, MOG, ANA, and CSF OCB were negative. MRI Brain showed T2/FLAIR hyperintensities in the brainstem, thalami, and basal ganglia with a "cascade sign." MRA suggested vasculitis. HLA-B*51 was positive. Final Diagnosis: Neuro-Behcet's Disease; Discussion: Behcet's Disease (BD) is a systemic vasculitis characterized by oral/genital ulcers and uveitis. Neurological involvement (Neuro-Behcet's) occurs in about 9% of cases and can sometimes be the presenting feature. Parenchymal brainstem syndrome is the most common neurological presentation. The "cascade sign" on MRI (lesions extending from thalamus to midbrain) is a typical radiological finding. HLA-B*51 is the strongest genetic susceptibility factor. Conclusion: Neuro-Behcet's should be considered in cases of acute brainstem involvement with upward extension into thalami, especially with a relapsing-remitting course and systemic features. Aggressive immunosuppression with steroids and IVIg can be life-saving and improve outcomes.

Keywords: Behcet disease, Neuro-Behcet's, CNS- Central nervous system, PNS- Peripheral nervous system, HLA-B*51, cascade sign, vasculitis MCA- middle cerebral artery, NMO- Neuromyelitis optica, MOG- myelin oligodendrocyte antibody

How to Cite?: Dr. Priyanka Jangam, Dr. N. V. Sundarachary, Dr. G. Bindu Narmada, Dr. M. Bhargavi Devi, Dr. Kamalakar P, "Unraveling Neuro-Behcet's Disease: Insights from a Case of Acute Brainstem Syndrome and the Clinical Value of the Cascade Sign", Volume 14 Issue 11, November 2025, International Journal of Science and Research (IJSR), Pages: 1409-1411, https://www.ijsr.net/getabstract.php?paperid=SR251118135744, DOI: https://dx.doi.org/10.21275/SR251118135744