BACKGROUND AND PURPOSE Validation of the Boston criteria for the in vivo diagnosis of cerebral amyloid angiopathy (CAA) is challenging, because noninvasive diagnostic tests do not exist. Hereditary cerebral hemorrhage with amyloidosis-Dutch type is an accepted monogenetic model of CAA and diagnosis can be made with certainty based on DNA analysis. The aim of this study was to analyze and refine...

Deferiprone Superficial siderosis (SS) is a neurodegenerative condition caused by hemosiderin deposition on the surface of the brain, cranial nerves, and spinal cord. SS is an exceptionally rare condition characterized by a triad of hearing loss, ataxia, and myelopathy. The most common etiologies that lead to SS are trauma, previous surgical procedures, dural tears, and tumors of the central ne...

Neurology Service, State University of Rio de Janeiro (UERJ), Rio de Janeiro RJ, Brazil; Laboratory of Panic & Respiration, Institute of Psychiatry, Federal University of Rio de Janeiro (UFRJ), INCT Translational Medicine, Rio de Janeiro RJ, Brazil. Superficial siderosis (SS) of the central nervous system (CNS) is a rare disorder characterized by a classic triad of symptoms consisting of sensor...

A 44-year-old man with a past medical history unremarkable presented with a 4-month evolution of progressive cognitive deterioration , deafness and unsteady gait. Initial neurological examination revealed mild cognitive impairment, dysarthria, cerebellar ataxia with wide-based unsteady gait and bilateral sensorineural hypoacusia. Laboratory serum analysis was unre-markable, including vitamin B1...