Beethoven's illness—A likely diagnosis

ArticleinProceedings of the Royal Society of Medicine 64(5):493-6 · June 1971with 182 Reads
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    Purpose . Prospective, controlled cohort study to investigate possible alterations in brain glucose metabolism (CMRglc) in patients with Cogan’s syndrome (CS). Patients and Methods . Functional mapping of the CMRglc was obtained by quantitative molecular imaging positron emission tomography, combined with computed tomography (FDG-PET/CT). The patients were divided into three clinical groups: typical CS; atypical CS (ACS); autoimmune inner ear disease (AIED). The unmatched control group (CG) consisted of subjects requiring FDG-PET/CT for an extracranial pathology. Statistical mapping searched areas of significant glucose hypometabolism in all the affected patients (DG) and in each clinical subgroup. The results were compared with those of the CG. Results . 44 patients were enrolled (DG) and assigned to the three study groups: 8 patients to the CS group; 21 patients to the ACS group; and 15 to the AIED group. Sixteen subjects formed the CG group. Areas of significant brain glucose hypometabolism were identified in all the study groups, with the largest number and extension in the DG and CS. Conclusions . This study revealed areas of significantly altered CMRglc in patients with CS (any subform) without neurologic complains and normal conventional neuroimaging. Our results suggest that FDG-PET/CT may represent a very useful tool for the global assessment of patients with Cogan’s syndrome.
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    Cogan’ syndrome is a multisystemic disorder characterized by the almost unfailing combination of inflammatory ocular disease and sensorineural hearing loss. In addition to constitutional symptoms, clinical features may also include cardiovascular, gastrointestinal, and neurological manifestations with variable frequency. Skin and mucous membranes, kidney and urogenital apparatus are rarely involved. The etiology remains undefined whereas an autoimmune pathogenesis is sustained by experimental and clinical observations. Given its rarity and clinical heterogeneity, the diagnosis is often delayed with possible progression to impairment of visual acuity and auditory dysfunction up to deafness. Therapy is based on the administration of glucocorticoids and immunosuppressive agents. Tumor necrosis factor-alpha blockers, the B-cell depleting monoclonal antibody rituximab, the anti-interleukin-6 receptor monoclonal antibody tocilizumab, and the immunomodulatory leflunomide have also been employed in single patients. Cochlear implantation can be used for hearing rehabilitation. Finally, stent-graft insertion and prosthetic aortic replacement can be extremely useful to correct the consequences of aortitis and large vessel aneurysms.
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    Background: Cogan's syndrome (CS) is a rare autoimmune vasculitis characterized by ocular inflammation and sensorineural hearing loss. CS is divided into a "typical" form with nonsyphilitic interstitial keratitis and audiovestibular symptoms, and an "atypical" form with ocular involvement affecting structures other than the cornea. Anti-Hsp70 antibodies were found at variable levels in patients presenting with various forms of autoimmune sensorineural hearing loss (ASNHL). Objectives: To assess the correlation between anti-Hsp70 antibodies and specific ASNHL subgroups. Methods: We divided 112 subjects into four groups: 14 subjects with typical CS, 24 with atypical CS, 55 with ASNHL, and 19 control subjects (healthy subjects and patients with systemic autoimmune diseases but no sensorineural hearing or audiovestibular alterations). Patients were tested for serological autoimmunity markers including anti-Hsp70. Results: Positivity of the anti-Hsp70 antibody test was highest in the typical CS group (92.9%) and lowest in the control group (5.2%). The test was positive in 52.7% of patients in the ASNHL group and 16.6% in the atypical CS group. The paired comparison analysis between groups showed that sensitivity of anti-Hsp70 in the typical CS group was significantly higher, as compared to the other three study groups. Conclusions: Anti-Hsp70 antibodies can be considered a serological marker of "typical" CS. "Atypical" CS is conceivably a sort of "melting pot" of different forms of autoimmune diseases still characterized by ocular inflammation and sensorineural hearing loss but whose antigenic characteristics need to be further defined.
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    Ludwig van Beethoven suffered from various constitutional disorders, most publicly known his deafness. He died in 1827 from a decompensated liver cirrhosis. On his deathbed, admirers cut some ringlets from his head that can nowadays be used for scientific analyses. Hair analyses demonstrated that Beethoven was increasedly exposed to lead in his last 110 living days that may have contributed to the hepatic decompensation. The reason for the lead exposition was probably consumption of wines mixed with lead sugar, but also iatrogenic by lead-containing preparations administered for pneumonia in winter 1826/1827 as well as wound closure after puncturing for ascites four times.
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