Neuropsychological characteristics of encephalopathy in Susac's Syndrome – Case report

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 neuropsycho...

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Bibliographic Details
Published in:Neurologia i neurochirurgia polska 2017-03, Vol.51 (2), p.174-179
Main Authors: Roessler-Górecka, Magdalena, Mendel, Tadeusz, Wiśniowska, Justyna, Seniów, Joanna
Format: Article
Language:English
Subjects:
Adult
Brain Diseases - diagnosis
Case reports
Cognitive-behavioral dysfunction
Corpus Callosum - pathology
Diagnosis, Differential
Encephalopathy
Female
Follow-Up Studies
Humans
Internal Capsule - pathology
Magnetic Resonance Imaging
Neurologic Examination
Neuropsychological Tests - statistics & numerical data
Neuropsychology
Psychometrics - statistics & numerical data
Susac Syndrome - diagnosis
Susac's Syndrome
Vasculopathy
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Summary: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.
ISSN:0028-3843
1897-4260
DOI:10.1016/j.pjnns.2017.01.001