open access

Vol 51, No 2 (2017)
Case reports
Submitted: 2016-09-05
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Neuropsychological characteristics of encephalopathy in Susac's Syndrome – Case report

Magdalena Roessler-Górecka, Tadeusz Mendel, Justyna Wiśniowska, Joanna Seniów
DOI: 10.1016/j.pjnns.2017.01.001
·
Neurol Neurochir Pol 2017;51(2):174-179.

open access

Vol 51, No 2 (2017)
Case reports
Submitted: 2016-09-05

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.

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Keywords

Susac' s Syndrome, Vasculopathy, Encephalopathy, Cognitive-behavioral dysfunction, Magnetic resonance imaging

About this article
Title

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

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 51, No 2 (2017)

Pages

174-179

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

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