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Title: Relapsing encephalo polychondritis. Reports of two cases [Encéfalo-policondritis recidivante. Reporte de dos casos]
Author: Andrade-Ramos, M.A.
Avila-Carrillo, A.
Navarro-Bonnet, J.
Alvarez-Palazuelos, L.E.
Gonzalez-Gonzalez, M.
Padilla-Martinez, J.J.
Morales-Ruvalcaba, E.
Velasco-Gutierrez, A.
García-Renteria, J.
Chiquete, E.
Gonzalez-Cornejo, S.
Ruiz-Sandoval, J.L.
Issue Date: 2009
Abstract: Introduction: Relapsing polychondritis (PR) is an autoimmune disease of unknown etiology characterized by a progressive and recurrent inflammatory process that affects mainly cartilaginous tissue and can also affect other systems. The affection of the central nervous system in PR has been named relapsing encephalo polychondritis (REP). Case report: We report two patients. The first case is a 78-year-old man with diagnosis of REP and a clinical presentation of rapidly progressive dementia, parkinsonism and myoclonias. MRI showed severe atrophy and hyperintense lesions on T2-weighted and FLAIR sequences. The second case is a 35-year-old man with endocranial hypertension syndrome. An MRI showed subcortical hyperintense lesions on T2-weighted and FLAIR sequences and leptomeningeal enhancement with gadolinium on T1-weighted images. Both patients were treated with immunosuppressive therapy. The first patient died of sepsis and the second patient showed improvement. Conclusions: RP is a rare disease easily to diagnose by rheumatologist and dermatologist that eventually can be associated with neurological manifestations, complicating its diagnosis. The radiological-pathological correlation of EPR is actually poorly understood. Both the EPR and PR have an insidious course with relapses and poor prognosis in a short or middle-term, therefore the acute treatment with steroids and chronic treatment with typical immunosuppressive therapy and new therapies are a constant in these patients.
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