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Jaccoud's arthropathy (JA) is a chronic deforming arthropathy, initially linked to rheumatic fever, now more commonly associated with systemic lupus erythematosus (SLE). We report a case of a 27-year-old male presenting with a four-month history of joint pain in the bilateral hands and feet, accompanied by stiffness but no swelling, erythema, or fever. Physical examination revealed flexion deformities, ulnar deviation at the metacarpophalangeal joints, and hyperextension at the proximal interphalangeal joints, without tenderness. Laboratory findings showed elevated anti-double stranded DNA (anti-dsDNA) antibodies and positive antinuclear antibodies (ANA), and imaging confirmed non-erosive arthropathy. Diagnosed with SLE-associated JA, the patient was treated with prednisone, diclofenac, and hydroxychloroquine, leading to significant symptom improvement and decreased anti-dsDNA antibody levels. Even though non-erosive and non-deforming arthropathy is more commonly seen in SLE, timely identification of JA as a non-erosive but deforming arthritis is crucial in differentiating SLE from rheumatoid arthritis. This case underscores the need for comprehensive evaluation and tailored therapy in complex autoimmune conditions to prevent long-term joint damage and improve patient outcomes., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Sanchez et al.)

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an uncommon genetic disorder that affects small blood vessels in the brain. It leads to neurological symptoms, disability-adjusted life years, and difficult emotional and physical situations for patients and their families. As unusual brain symptoms appear, it becomes important to understand the different clinical manifestations of CADASIL. Our case report and review examine several cases to demonstrate different presentations and management strategies of CADASIL. A 52-year-old male with a family history of strokes at a young age from his father and paternal grandfather presented to a neurology clinic for left facial droop and drooling. Brain magnetic resonance imaging showed extensive periventricular and subcortical white matter disease, including the external capsule and subcortical white matter of the temporal lobe. Findings were suggestive of small vessel vasculopathy. A cerebral angiogram showed that all large extra- and intracranial vessels were patent without evidence of aneurysm formation. There was no obvious evidence of beading of the distal intracranial vessels. Cerebrospinal fluid studies were normal. The NOTCH3 mutation was sent to test for CADASIL, which came back positive. The patient was started on aspirin (81 mg) and atorvastatin (20 mg) daily. The patient was counseled on the possibility of having an ischemic or hemorrhagic stroke. Aspirin and atorvastatin were continued, a neuropsychological evaluation was ordered, and CADASIL genetic counseling and testing were offered to him and his children. Over several years, patients developed several strokes and seizures due to infarcts. He also developed intraparenchymal hemorrhage complicated by dysphagia, requiring a feeding tube. Due to his severe physical debility, he was discharged to a nursing home for rehabilitation, where he did not improve with therapy and remained bedbound. He was discharged and sent home with his family. CADASIL can present as a diagnostic challenge due to its common presentation with migraines, transient ischemic attacks, and strokes, with or without risk factors. This unique presentation of CADASIL with facial palsy highlights the importance of emerging atypical presentations and the need for a detailed history of neuroimaging, family history, and personal history of neurovascular events. By accurately diagnosing the condition, patients and families can be counseled on the disease course and genetics. Management requires a multidisciplinary approach with neurology, genetic counseling, physical therapy, psychology, and psychiatry if depression or anxiety is present, with the aim of improving the patient's quality of life., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Jain et al.)