Introduction == Cogan’s syndrome (CS) is named after the ophthalmologist Cogan, who was the first to report non-syphilitic interstitial keratitis and audiovestibular symptoms [1]

Introduction == Cogan’s syndrome (CS) is named after the ophthalmologist Cogan, who was the first to report non-syphilitic interstitial keratitis and audiovestibular symptoms [1]. 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. == 1 . Introduction == Cogan’s syndrome (CS) is named after the ophthalmologist Cogan, who was the first to report non-syphilitic interstitial keratitis and audiovestibular symptoms [1]. The disease was probably present long before this formal identification, with Ludwig von Beethoven perhaps its most prominent sufferer [2]. CS is a rare autoimmune disease characterized by ocular inflammation and sensorineural hearing loss, with a number of possible related findings and a pathogenetic mechanism of vasculitis. Possible combined manifestations are mostly neurological, rheumatological, cardiovascular, and gastrointestinal; the latter two may be very severe, due to aortic insufficiency or mesenteric arteritis [38]. In 1980, Haynes et al. proposed a distinction between typical CS, as originally defined, and atypical CS (ACS), in which the ocular involvement does not affect the cornea but is responsible for chronic or recurrent conjunctivitis, scleritis, uveitis, optic disc edema, and retinal vasculitis [8]. It is more common for patients with ACS than for those with typical CS to have systemic manifestations [3]. A third disorder, Amisulpride hydrochloride which may have an overlapping pathogenesis and manifestations, is autoimmune inner ear disease (AIED), defined as primary when the pathology is restricted to the ear and secondary when it occurs in the context of a systemic autoimmune disease [9, 10]. All three syndromes (CS, ACS, and AIED) include neurological disorders, mostly as unexpected manifestations of acute vascular accidents and/or transient ischemic events. In this study, we prospectively investigated the alterations in brain glucose metabolism that may have been Rabbit Polyclonal to TEAD2 induced by mild, long-standing cerebral vasculitis in patients presenting with the aforementioned syndromes but no prior neurological symptoms and normal conventional neuroimaging findings. Functional mapping of the cerebral glucose consumption was obtained by quantitative molecular imaging with 2-[18F]fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET), combined with computed tomography (CT). detecting in vivo cerebral glucose utilization and changes in brain metabolism. == 2 . Patients and Amisulpride hydrochloride Methods == The study was conducted in its entirety at the University Hospital of Parma (Italy) with the participation of the following hospital units: Inflammatory and Infectious Ocular Diseases; Otorhinolaryngology; Nuclear Medicine; Medical Physics; and Neurology. The protocol was approved by the Institutional Ethics Committee, and informed consent was obtained from all patients according to the tenets of the Declaration of Helsinki. The patients included in the study were divided into three groups: CS (i. e., typical disease, with interstitial keratitis) ACS (autoimmune ocular inflammation other than interstitial keratitis) AIED (primary and secondary) The unmatched control group (CG) consisted of a cohort of individuals with a negative history of neuropsychiatric and neurological disorders who strictly required FDG-PET/CT for a pathology not involving the head and neck (mostly primary abdominal neoplasm) and who agreed to have the scan extended to the cerebral region (in which case the conventional written consent form was amended accordingly). The enrollment procedure targeted patients diagnosed with unilateral or bilateral sensorineural hearing loss who had been referred to an ophthalmologist for the assessment of possible concomitant ocular inflammation. A complete medical history was obtained from each patient. Blood samples were collected and tested for autoimmunity inducers. CS or ACS was diagnosed by the patient’s ophthalmologist and/or otolaryngologist based on the clinical features, general investigations to narrow the differential diagnosis, and results of serology for specific markers [11, 12]. AIED was Amisulpride hydrochloride finally diagnosed by an otolaryngologist after syphilis, noise and head trauma, drug toxicity, and hereditary hearing impairment were excluded. All patients also underwent brain magnetic resonance imaging (MRI) with gadolinium to exclude acoustic neurinoma, metastatic disease, lymphoma, and multiple sclerosis. After a patient was assigned to one of the three study groups.