Abstract
Abstract: :
Purpose: To support SPECT utility in early diagnosis of Cogan’s syndrome (CS). Methods: 13 patients affected by CS were studied , 8 males and 5 females, the age ranged from 9 to 65 years. 6 patients were affected by typical form of CS, 7 by the atypical form. The regional cerebral perfusion was studied with Single Photon Emission Computed Tomography (SPECT), using a dual detector camera with high resolution collimator. The examination started 20’ after the injection of 740 MBq of Bicisate and reduction of the perfusion was identified by visual interpretation and by a region cerebellum scale. To investigate a possible cerebral damage due to vascular involvement MRI and SPECT were performed in all patients. None of the patients studied had neurological symptoms, i.e. neuro-psychiatric involvement, such as cognitive disorders, headache, altered consciousness, organic brain syndrome. Results: MRI was negative in 12 patients and positive in one. SPECT demonstrated in 12 out of 13 patients one or more areas of cerebral perfusion defect. SPECT imaging showed focal defect pattern in the temporal lobe in 7 patients (58.3%), particularly in temporal inferior gyrus, focal defect pattern in frontal lobe in 3 patients (25%), defect pattern in occipital lobe in 1 patient (8.3%); in 1 patient the cerebral perfusion defect was diffuse in the temporal and frontal lobes (8.3%). Conclusions: CS is a rare vasculitis of autoimmune origin. CS characterised by interstitial keratitis accompanied by Menière-like symptoms and sensorineural hearing loss is called "typical CS". The same auditory symptoms together with other types of ocular inflammation, i.e. uveitis, conjunctivitis, papillitis are called "atypical CS". Systemic vasculitis in CS affects about 10% of patients ; therefore it is important, also for the ophthalmologists, to evaluate the systemic involvement and start an immunosuppressive treatment to avoid severe complications and deafness. An interdisciplinary approach, such as it has been used in this study, migth not only improve the diagnosis, but also be helpful to clarify the etiology and the pathogenesis of this rare disorder. SPECT analysis detects functional disorders related to actual cerebral blood flow. This examination could be useful in early diagnosis of Cogan’s syndrome, to allow an early treatment of this rare and severe vasculitis.
Keywords: autoimmune disease • imaging methods (CT, FA, ICG, MRI, OCT, RTA, S • imaging/image analysis: clinical