title: Neuropsychological characteristics of encephalopathy in Susac's Syndrome - Case report.
abstract: Susac's Syndrome (SS) is a rare, autoimmune angiopathy characterized by hearing loss, retinal artery occlusions and encephalopathy, which is usually expressed in multifocal neurological signs and symptoms, confusion state and cognitive impairment. There have been few descriptions of neuropsychological assessment of SS. We present a case study of 29-year-old woman who developed full SS. During the post-acute stage of disease, she was admitted to neurorehabilitation ward to improve her cognitive-behavioral and motor functioning. The initial assessment revealed attention, memory and executive dysfunctions, as well as behavioral changes including impulsivity, affective dysregulation and reduced self-awareness of disease deficits. After five weeks recovery process supported by rehabilitation program, improvement was observed, although some cognitive-behavioral deficits were still present in the follow-up assessment.
authors.affiliation: 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland. Electronic address: roessler@ipin.edu.pl.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Roessler-Górecka
authors.affiliation: 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland. Electronic address: mendel@ipin.edu.pl.
authors.forename: Tadeusz
authors.initials: T
authors.lastname: Mendel
authors.affiliation: 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland. Electronic address: justyna.wisniowska@psych.uw.edu.pl.
authors.forename: Justyna
authors.initials: J
authors.lastname: Wiśniowska
authors.affiliation: 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland. Electronic address: seniowj@ipin.edu.pl.
authors.forename: Joanna
authors.initials: J
authors.lastname: Seniów
mesh_terms: D000328:Adult; D001927:Brain Diseases / Q000175:diagnosis*; D003337:Corpus Callosum / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D020772:Internal Capsule / Q000473:pathology; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination; D009483:Neuropsychological Tests / Q000706:statistics & numerical data*; D011594:Psychometrics / Q000706:statistics & numerical data*; D055955:Susac Syndrome / Q000175:diagnosis*
keywords: Cognitive-behavioral dysfunction; Encephalopathy; Magnetic resonance imaging; Susac's Syndrome; Vasculopathy