ABSTRACT
Introduction: The association between primary brain tumors, such as glioneuronal tumors, with autosomal-dominant polycystic kidney disease (ADPKD) remains poorly understood, with only two cases reported excluding this one. This case of an ADPKD patient diagnosed with a rosette-forming glioneuronal tumor highlights an exceptionally rare potential association warranting further investigation. Case presentation: A 28-year-old male with ADPKD presented with progressive ataxia, dizziness, and headache. MRI revealed a cerebellar mass and obstructive hydrocephalus. Surgical resection and histopathological examination confirmed the diagnosis of a rosette-forming glioneuronal tumor. Postoperatively, the patient showed significant symptom improvement. Discussion: The interplay between genetics and glioneuronal development is complex and underexplored. While most glioneuronal arise sporadically, rare genetic syndromes may predispose individuals to these tumors. Additionally, although more than 70 cases of ADPKD with concurrent tumors were reported, the literature on this specific association remains limited. Conclusion: This case underscores the need for heightened awareness of potential associations between ADPKD and tumors such as glioneuronal tumors. With limited literature on this subject, further research is imperative to understand the underlying mechanisms and clinical implications. Enhancing our knowledge in this area can improve patient outcomes and management strategies.
ABSTRACT
Palindromic rheumatism (PR) is a unique syndrome considered a prelude to rheumatoid arthritis (RA). It is characterized by recurrent, unpredictable episodes of joint inflammation and distinct clinical features. Unlike RA, PR episodes are brief and reversible, involving sudden-onset joint pain, swelling, and erythema. The exact etiology and diagnostic criteria of PR remain elusive, but it often shares autoantibodies with RA, leading patients to transition from PR to RA. The management of PR is multifaceted and empirical, involving various treatment modalities such as non-steroidal anti-inflammatory drugs (NSAIDs), colchicine, and disease-modifying anti-rheumatic drugs (DMARDs). However, the relationship between obesity and PR remains underexplored. This case presents a 52-year-old woman, who presented to our hospital with recurrent and debilitating arthritis episodes since 2016. Initially affecting her wrists and later extending to her knees, shoulders, and back, these episodes severely impaired her daily activities. Despite a diagnosis of RA in 2019, supported by a positive rheumatoid factor (RF) test, and subsequent DMARD treatment, her symptoms persisted. In 2022, during her examinations at our hospital, the distinctive pattern of intermittent symptoms accompanied by damage-free joints was unveiled, suggesting a potential diagnosis of palindromic rheumatism. Notably, this case highlights the potential association between obesity and PR, as the patient's decision to undergo bariatric surgery in 2022 led to substantial weight loss of over 36 kg. This weight reduction yielded remarkable improvements in her condition, resulting in reduced frequency and severity of PR attacks. As a consequence, her medication regimen was simplified, emphasizing the therapeutic role of weight management in PR. This case paves the way for further research into the relationship between obesity, PR, and non-pharmacological interventions in PR management.
