BMC Neurology最新文献

Background: Sjögren's disease (SjD) is a chronic autoimmune disease featured by lymphocytic infiltration of exocrine glands and numerous organ involvements, but central nervous system involvement is relatively rare. Due to the variety of symptoms, diagnosis is particularly difficult under certain conditions, which may delay the optimal timing of treatment. Increased clinical awareness and effective pharmacotherapies are urgently needed.

Case presentation: A case of a 39-year-old female was initially considered to have an intracranial occupancy based upon rapidly progressing nervous symptoms including intermittent fever, parieto-occipital headache, numbness and weakness in the hand, blurred vision, memory impairment and imaging findings, but it was ultimately clarified that she had SjD involving nervous system, with immunological results showing positive antinuclear antibodies (1:640) and anti-SSA/SSB antibodies (+++) in serum and pathological findings suggesting vasculitis of brain. Finally, she was successfully treated with rituximab.

Conclusions: The onset of SjD may even only manifest as neurological symptoms, which could be easily confused with other systemic or neurological diseases, thus demanding careful differential diagnosis through a detailed medical history and immunological, even pathological examinations to clarify the etiology. Rituximab is an effective treatment option for SjD involving nervous systems, which is an option not commonly featured in standard treatment protocols.

Background: Cerebral venous sinus thrombosis (CVST) is an uncommon cerebrovascular condition, constituting less than 1% of all stroke cases, particularly affecting young adults. CVST typically presents with nonspecific symptoms, complicating timely diagnosis. Although anticoagulation remains the primary treatment, complicated cases may necessitate endovascular intervention. Reports of CVST presenting with multifaceted neurological and orthopedic complications in young patients without thrombophilic disorders are uncommon, underscoring the importance and educational value of documenting such cases.

Case presentation: We report the case of an 18-year-old female without prior medical or migraine history who presented to the emergency department with a six-day history of persistent, progressive headache accompanied by nausea. Initial clinical evaluation revealed no neurological deficits, but symptoms and positional exacerbation warranted further investigation. Neuroimaging via contrast-enhanced CT demonstrated extensive thrombosis involving the superior sagittal sinus and bilateral cortical veins, accompanied by minimal parietal hemorrhage and edema. Endovascular thrombectomy was performed due to progressive neurological deterioration, achieving significant clot reduction and continuous venous recanalization. Despite treatment, the patient experienced persistent left-sided hemianopsia and subsequent focal seizures, managed effectively with anti-epileptic therapy. She also sustained an orthopedic injury; anteroinferior left shoulder dislocation, secondary to nocturnal seizure activity, requiring orthopedic intervention. Extensive thrombophilia screening was negative, with no identifiable hereditary factors. Follow-up imaging showed marked thrombus resolution, clinical symptoms improved significantly, and hemianopsia resolved completely after seven months.

Conclusions: This case illustrates the potential severity and complexity of CVST in healthy young patients. It underscores the importance of prompt diagnosis and multidisciplinary management, including consideration of endovascular thrombectomy in refractory or complicated cases. Further research is needed to elucidate the underlying mechanisms and optimize treatment strategies, particularly in young patients without identifiable thrombophilic disorders.