RESUMO
Gallbladder paraganglioma is a very rare condition, and only a few cases have been reported in the literature. Herein, we present and discuss the clinical and radiological findings of a 72-year-old woman who complained of heaviness, pain, and on and off watery discharge from the left ear for 1 year and reduced hearing in both ears. She was thoroughly investigated radiologically where multiple paragangliomas were found with the discovery of the very rare gallbladder paraganglioma. There is a need to realize the importance of thorough radiological evaluation in cases of head and neck paraganglioma to look for more such lesions in the body for early identification of patients with familial paraganglioma syndromes. With this practice, gene mutation carriers can be identified early in the course of their disease and brought to surgical attention before their disease becomes extensive and potentially life-threatening.
RESUMO
CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a recently described, rare neuroinflammatory disorder diagnosed by clinical symptoms involving the brain stem with a distinct pattern on neuroimaging and a perivascular T-lymphocyte infiltrate on brain biopsy. It is a condition usually described in adults in the fourth to fifth decade. We report a case of 13-year-old Indian boy who presented with recurrent episodes of ataxia and diplopia with onset at 7 years of age. He was investigated extensively to rule out infective, neoplastic, autoimmune, and demyelinating conditions over a span of 6 years. The diagnosis of CLIPPERS was entertained on the basis of clinico-radio-pathological correlation. Treatment with steroids and steroid-sparing agents, particularly methotrexate, seems to provide a promising outcome. With very few cases in literature so far, reporting of a larger case series with pediatric onset may expand it to CLIPPERS spectrum disorder.