Ormond's disease is a systemic autoimmune disease with serious complications. We present our retrospective analysis of 83 patients diagnosed with and treated for idiopathic retroperitoneal fibrosis (Ormond's disease) in our department from 1997 to 2023. In this retrospective study, we analysed the diagnostic approaches, the clinical history and surgical and immunosuppressive therapies, and their subsequent effects on our patients. Patients with established disease activity were given immunosuppressive treatment, using corticosteroids alone or in combination with azathioprine, in patients with exacerbation of the disease mycophenolate mofetil. Three patients with Ormond's disease and systemic complications (IgG4-related disease) were treated with rituximab. In the entire cohort, 83 patients received immunosuppressive therapy; the next 5 patients did not receive this treatment because they did not present inflammatory activity from the disease. In these 83 patients, computed tomography showed that immunosuppressive treatment resulted in partial or complete regression of the inflammatory infiltrate. Out of the 83 patients, 10 patients experienced disease exacerbation 7 and 24 months after the immunosuppressive treatment was discontinued. The follow-up ranged from 24 months to 26 years.
- Klíčová slova
- Hydronephrosis, Immunosuppression, Ormond’s disease, Periaortitis,
- MeSH
- dospělí MeSH
- imunosupresiva * terapeutické užití MeSH
- lidé středního věku MeSH
- lidé MeSH
- retroperitoneální fibróza * diagnóza farmakoterapie MeSH
- retrospektivní studie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- imunosupresiva * MeSH
PURPOSE: Stereotactic radiofrequency amygdalohippocampectomy (SAHE) has been modified recently in our center for the therapy of mesial temporal epilepsy (MTLE). It has promising clinical results comparable with microsurgical amygdalohippocampectomy despite smaller volume reduction of the hippocampus. We hypothesized that the extent of perirhinal and entorhinal cortex (PRC, EC) reduction could explain the clinical outcome. Therefore, we performed, retrospectively, volumetric analysis of PRC and EC and compared it with the seizure control. METHODS: Twenty-six consecutive patients with MTLE treated by SAHE were included. PRC and EC volumes were measured from magnetic resonance imaging (MRI) records obtained before and 1 year after SAHE. The clinical outcome was assessed each year after SAHE using Engel's classification. KEY FINDINGS: Twenty-six patients were analyzed. The volume of PRC decreased by 46 ± 17% (p < 10(-12) ); EC volume decreased by 56 ± 20% (p < 10(-10) ). Two years after the procedure, 73% of patients were classified as Engel's I, 19% as Engel's II; in 2 (8%) the treatment failed (were reoperated). Eighteen patients finished 3 years follow-up; 72% of them were classified as Engel's I, 17% as Engel's II, and in 2 (11%) above-mentioned patients the treatment failed. Thirteen patients finished 4 years of follow-up, 11 of them as Engel's I. There was no significant correlation of the clinical outcome to PRC and EC volume reductions. SIGNIFICANCE: The clinical effect of SAHE is not clearly explained by the volume reductions of PRC and EC (nor of the hippocampus and the amygdala). It promotes opinion that the extent of resection/destruction is not important for seizure outcomes.
- MeSH
- amygdala patologie chirurgie MeSH
- cortex entorhinalis patologie chirurgie MeSH
- dospělí MeSH
- elektrokoagulace metody MeSH
- epilepsie temporálního laloku patologie chirurgie MeSH
- hipokampus patologie chirurgie MeSH
- lidé středního věku MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- mapování mozku MeSH
- mladiství MeSH
- radiochirurgie přístrojové vybavení metody MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- srovnávací studie MeSH
