Neurocognitive Profile of a Woman with Susac’s Syndrome: Further Evidence of Cognitive Variability

LeMonda, Brittany C.; Peck, Caleb P.; Giles, Katherine J.; Bowers, Dawn

doi:10.1080/13854046.2015.1076891

Cited by 11 publications

(5 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…We present a case study of 29-year-old woman who developed full SS and underwent a detailed neuropsychological assessment and rehabilitation, so it can contribute to the existing literature on this rare syndrome. In the presented case, the cognitive-behavioral disturbances only partly resemble these descripted by Le Monda et al [9], which also suggests the possible individual differences in the neuropsychological profile of encephalopathy in SS.…”

Section: Introductionsupporting

confidence: 77%

“…Cognitive impairments and their severity probably vary in individual patients: some studies have described mainly psycho-motor slowness, while others indicated more specific symptoms in domain of memory, attention, and executive functioning. Recently, comprehensive neuropsychological assessment was provided by Le Monda et al [9]. In this case report a patient examined two years after the SS's onset, presented some cognitive inefficiencies, including visuoconstructive disability, difficulty of encoding of a wordlist and limited set-shifting.…”

Section: Introductionmentioning

confidence: 94%

“…It occurs in 60-70% of SS patients and can manifest itself as major deficits in various cognitive domains, at the beginning of the disease cooccurring with confusion and/or personality changes, bizarre behavior, mood disturbances, and even psychotic symptoms; features of abulia can also be present and may progress to a stage where verbal and nonverbal communication is no longer possible [8]. However, until today, only five articles have described in detail neuropsychological dysfunctions in SS [9]. Cognitive impairments and their severity probably vary in individual patients: some studies have described mainly psycho-motor slowness, while others indicated more specific symptoms in domain of memory, attention, and executive functioning.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Roessler-Górecka

Mendel

Wiśniowska

et al. 2017

Neurologia i Neurochirurgia Polska

View full text Add to dashboard Cite

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.

show abstract

Section: Introductionsupporting

confidence: 77%

Section: Introductionmentioning

confidence: 94%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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

Roessler-Górecka

Mendel

Wiśniowska

et al. 2017

Neurologia i Neurochirurgia Polska

View full text Add to dashboard Cite

show abstract

“…92 Numerous other chronic inflammatory brain diseases may present with cognitive impairment, with or without evidence of neurodegeneration, and may be included in the differential diagnosis of AiD-ND. These include MS, [104][105][106] sarcoidosis, [107][108][109] primary angiitis of the CNS, [110][111][112] amyloid β-related angiitis, 113 Susac's syndrome, 114,115 Rasmussen's encephalitis and other inflammatory epilepsies, 116,117 and systemic rheumatologic conditions. [118][119][120][121][122][123][124] Diagnosing Autoimmune Dementia…”

Section: Aid-ae In Patients With Other (Atypical) Causes Of Aementioning

confidence: 99%

Autoimmune Dementia

Long

Day

2018

Semin Neurol

View full text Add to dashboard Cite

Dementia refers to an acquired syndrome of intraindividual cognitive decline that ultimately interferes with an individual's ability to manage their usual duties at work or home. As experience with the diagnosis and management of patients with autoimmune and paraneoplastic encephalitis (AE) has expanded, it has become increasingly apparent that dementia may arise as a subacute or chronic complication of immune-mediated injury to the central nervous system. Progressive memory and thinking problems may represent the first (or only) sign of an underlying autoimmune or paraneoplastic disease. Accordingly, there is a need to routinely consider the diagnosis of AE in patients with dementia, and to evaluate patients recovering from AE for clinically meaningful cognitive impairment. We review and summarize the available evidence concerning the diagnosis and care of AE patients with associated cognitive impairment, herein referred to as autoimmune dementia (AiD). Relevant information is used to propose a novel diagnostic framework that may be applied to improve recognition, and facilitate the expedited evaluation and treatment of patients with AiD.

show abstract

“…Although cognitive and behavioral changes are often the main complaint of patients, these have not been thoroughly described [13]. Neuropsychological functioning in SuS is little researched and only few articles have described it in detail, mostly in the form of case reports [14][15][16][17][18]. These show a variability in type and severity in cognitive deficits over individuals, ranging from psycho-motor slowness to deficits in memory, attention and executive functioning [15].…”

Section: Introductionmentioning

confidence: 99%

Cognitive outcomes in Susac syndrome: A 2‐year neuropsychological follow‐up study

Van Vrekhem,

Miatton,

Hemelsoet

et al. 2024

Euro J of Neurology

View full text Add to dashboard Cite

Background and purposeSusac syndrome (SuS) is a rare, autoimmune, neurological disease characterized by a clinical triad of branch retinal artery occlusion, sensorineural hearing loss and encephalopathy. Neuropsychological functioning in SuS is little researched and the prevalence, nature, and evolution over time of cognitive deficits in SuS remain unclear. This study aimed to better understand the long‐term neuropsychological outcomes of patients with SuS.MethodsThirteen patients with SuS (mean [SD] age 39.5 [11.1] years) were enrolled at the Ghent University Hospital by their treating neurologist. The cognitive functioning and emotional well‐being of each patient was evaluated by means of a thorough neuropsychological test battery at baseline and after 2 years. Follow‐up testing after 2 years was performed in 11 patients (mean [SD] age 42.2 [11.5] years).ResultsPatients showed normal neuropsychological test results at a group level, both at baseline and follow‐up testing. Significant improvements over time were found for information processing speed, verbal recognition, and semantic and phonological fluency. Individual test results showed interindividual variability at baseline, with most impairments being in attention, executive functioning and language, which improved after a 2‐year period. In addition, patients reported significantly lower mental and physical well‐being, both at baseline and follow‐up testing.ConclusionsOur results suggest that neuropsychological dysfunction in SuS is limited at a group level and improves over time. Nonetheless, individual test results reveal interindividual variability, making cognitive screening essential. Furthermore, a high psycho‐emotional burden of the disease was reported, for which screening and follow‐up are necessary.

show abstract

Neurocognitive Profile of a Woman with Susac’s Syndrome: Further Evidence of Cognitive Variability

Abstract: Our findings provide further evidence of cognitive interindividual variability in a confirmed case of Susac's syndrome.

Cited by 11 publications

References 20 publications

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

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

Autoimmune Dementia

Cognitive outcomes in Susac syndrome: A 2‐year neuropsychological follow‐up study

Contact Info

Product

Resources

About