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Neuropsychological characteristics of encephalopathy in Susac's Syndrome – Case report
Affiliations- 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland
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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.
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.
Keywords
Susac' s Syndrome, Vasculopathy, Encephalopathy, Cognitive-behavioral dysfunction, Magnetic resonance imaging
About this articleTitle
Neuropsychological characteristics of encephalopathy in Susac's Syndrome – Case report
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
174-179
Page views
400
Article views/downloads
709
DOI
10.1016/j.pjnns.2017.01.001
Bibliographic record
Neurol Neurochir Pol 2017;51(2):174-179.
Keywords
Susac's Syndrome
Vasculopathy
Encephalopathy
Cognitive-behavioral dysfunction
Magnetic resonance imaging
Authors
Magdalena Roessler-Górecka
Tadeusz Mendel
Justyna Wiśniowska
Joanna Seniów
