Susac Syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, hearing loss and retinal arterial occlusions, with prevalent structural changes identified on brain magnetic resonanc...Susac Syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, hearing loss and retinal arterial occlusions, with prevalent structural changes identified on brain magnetic resonance imaging (white matter, corpus callosum, basal ganglia region and the thalamic region extending to the midbrain) in the majority of cases, which lead to cognitive manifestations of which there is a paucity of descriptions in the literature. The objective of this case study is to compare to post-rehabilitation neurocognitive profile of a 29-year-old woman with SS presenting with compromised intellectual and motor skills and cognitive functions, together with neuropsychiatric symptoms. Better performance was found in the neuropsychological assessment, with changes in the structural cerebral network evidenced on Diffusion Tensor Imaging (DTI) performed following the therapeutic and pharmacological intervention.展开更多
Background:Susac syndrome is a rare disease of unknown pathogenesis.It consists of retinal arterial occlusion,hearing loss and encephalopathy(triad).Patients:The features of two cases of this syndrome are presented.Co...Background:Susac syndrome is a rare disease of unknown pathogenesis.It consists of retinal arterial occlusion,hearing loss and encephalopathy(triad).Patients:The features of two cases of this syndrome are presented.Conclusion:A high percentage of patients do not have the clinical triad at the time of onset of symptoms and,therefore,this disease may be under-diagnosed.Any patient with unexplained encephalopathy should thus be examined by an ophthalmologist and have an audiogram performed.展开更多
Introduction. Susac’s syndrome consists of the clinical triad of cerebral microangiopathy, branch retinal artery occlusions, and hearing loss. The pathogenesis of the disease remains unknown. The severity of retinal ...Introduction. Susac’s syndrome consists of the clinical triad of cerebral microangiopathy, branch retinal artery occlusions, and hearing loss. The pathogenesis of the disease remains unknown. The severity of retinal vasculopathy remains variable. Case report. A 57-year-old Caucasian woman presented in 1999 for the diagnostic and therapeutic management of a severe and complete form of the disease. Fluorescein angiography and cerebral MRI were contributive in this case and confirmed the diagnosis. A severe unilateral visual loss occurred one month after the initiation of systemic corticosteroids. Funduscopy disclosed a temporal retinal artery occlusion with major ischemia. Therefore, cyclophosphamide and anticoagulants were added to corticosteroids and total visual recovery was achieved within 2 weeks without any relapse after a follow-up period of 54 months. Conclusion. Therapeutic management of Susac’s syndrome is still controversial. Major immunosuppressive regimens are mandatory in the face of severe visual loss associated with central or branch retinal artery occlusions. The final prognosis of the disease seems favorable in the absence of prolonged and symptomatic retinal ischemia.展开更多
文摘Susac Syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, hearing loss and retinal arterial occlusions, with prevalent structural changes identified on brain magnetic resonance imaging (white matter, corpus callosum, basal ganglia region and the thalamic region extending to the midbrain) in the majority of cases, which lead to cognitive manifestations of which there is a paucity of descriptions in the literature. The objective of this case study is to compare to post-rehabilitation neurocognitive profile of a 29-year-old woman with SS presenting with compromised intellectual and motor skills and cognitive functions, together with neuropsychiatric symptoms. Better performance was found in the neuropsychological assessment, with changes in the structural cerebral network evidenced on Diffusion Tensor Imaging (DTI) performed following the therapeutic and pharmacological intervention.
文摘Background:Susac syndrome is a rare disease of unknown pathogenesis.It consists of retinal arterial occlusion,hearing loss and encephalopathy(triad).Patients:The features of two cases of this syndrome are presented.Conclusion:A high percentage of patients do not have the clinical triad at the time of onset of symptoms and,therefore,this disease may be under-diagnosed.Any patient with unexplained encephalopathy should thus be examined by an ophthalmologist and have an audiogram performed.
文摘Introduction. Susac’s syndrome consists of the clinical triad of cerebral microangiopathy, branch retinal artery occlusions, and hearing loss. The pathogenesis of the disease remains unknown. The severity of retinal vasculopathy remains variable. Case report. A 57-year-old Caucasian woman presented in 1999 for the diagnostic and therapeutic management of a severe and complete form of the disease. Fluorescein angiography and cerebral MRI were contributive in this case and confirmed the diagnosis. A severe unilateral visual loss occurred one month after the initiation of systemic corticosteroids. Funduscopy disclosed a temporal retinal artery occlusion with major ischemia. Therefore, cyclophosphamide and anticoagulants were added to corticosteroids and total visual recovery was achieved within 2 weeks without any relapse after a follow-up period of 54 months. Conclusion. Therapeutic management of Susac’s syndrome is still controversial. Major immunosuppressive regimens are mandatory in the face of severe visual loss associated with central or branch retinal artery occlusions. The final prognosis of the disease seems favorable in the absence of prolonged and symptomatic retinal ischemia.
