Longitudinal improvement in neuropsychological profile following treatment of severe encephalopathy in Susac syndrome

BACKGROUND: Susac syndrome (retino-cochleo-cerebral vasculopathy, SuS) is an autoimmune endotheliopathy characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. In contrast to data regarding auditory function, data measuring vestibular function is sparse and the cervical vestibular-evoked myogenic potentials (cVEMPs). OBJECTIVE: To determine whether the video head impulse test (vHIT) can serve as a confirmatory assessment of vestibulocochlear dysfunction in cases of suspected SuS. METHODS: Seven patients diagnosed with SuS underwent pure tone audiometry, a word recognition test, cVEMPs and the vHIT. RESULTS: Five patients were diagnosed with definite SuS, and two with probable SuS. Two patients were asymptomatic for hearing loss or tinnitus, and no sensorineural hearing loss was detected by audiograms. Four patients complained of tinnitus, and three patients reported experiencing vertigo. Three patients had abnormal cVEMPs results. All seven patients’ vHIT results were normal, except for patient #2, who was one of the three who complained of vertigo. The calculated gain of her left anterior semicircular canal was 0.5, without saccades. CONCLUSIONS: This is the first study to describe the results of the vHIT and cVEMPs among a group of patients with SuS. The results suggest that the vHIT should not be the only exam used to assess the function of the vestibular system of SuS patients.

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Brain MRI lesion load at diagnosis, severity at onset and outcomes in Susac syndrome: a prospective cohort study

European Journal of Neurology ◽

10.1111/ene.15062 ◽

2021 ◽

Author(s):

Carole Scheifer ◽

Marie‐Cécile Henry Feugeas ◽

Mélanie Roriz ◽

Fleur Cohen Aubart ◽

Serge Doan ◽

...

Keyword(s):

Cohort Study ◽

Prospective Cohort Study ◽

Prospective Cohort ◽

Brain Mri ◽

Lesion Load ◽

Susac Syndrome

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Poster 167: Susac Syndrome in a Young Female: A Unique Case and Rehabilitation Perspective

Archives of Physical Medicine and Rehabilitation ◽

10.1016/j.apmr.2007.06.420 ◽

2007 ◽

Vol 88 (9) ◽

pp. E56-E57

Author(s):

Peter Bailey ◽

Kenley D. Schmidt

Keyword(s):

Young Female ◽

Unique Case ◽

Susac Syndrome

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Neuropsychological profile in patients with early stage of Parkinson's disease in Taiwan

Parkinsonism & Related Disorders ◽

10.1016/j.parkreldis.2012.06.002 ◽

2012 ◽

Vol 18 (10) ◽

pp. 1067-1072 ◽

Cited By ~ 23

Author(s):

Rwei-Ling Yu ◽

Ruey-Meei Wu ◽

Chun-Hwei Tai ◽

Chin-Hsien Lin ◽

Ting-Wen Cheng ◽

...

Keyword(s):

Parkinson’S Disease ◽

Parkinson's Disease ◽

Early Stage ◽

Neuropsychological Profile

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Corpus Callosum Lesions are not Inextricably Linked to CNS Symptoms in Reported Cases of Susac Syndrome

Multiple Sclerosis and Related Disorders ◽

10.1016/j.msard.2021.102883 ◽

2021 ◽

pp. 102883

Author(s):

Gillian R Paton ◽

Claire Sheldon ◽

Talia Vertinsky ◽

Eduardo Navajas ◽

Anthony Traboulsee ◽

...

Keyword(s):

Corpus Callosum ◽

Susac Syndrome

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Susac syndrome and pregnancy: a review of published cases and considerations for patient management

Therapeutic Advances in Neurological Disorders ◽

10.1177/1756286420981352 ◽

2021 ◽

Vol 14 ◽

pp. 175628642098135

Author(s):

Barbara Willekens ◽

Ilka Kleffner

Keyword(s):

Hearing Loss ◽

Family Planning ◽

Postpartum Period ◽

Management Strategy ◽

Patient Management ◽

Treatment Options ◽

Young Females ◽

Susac Syndrome ◽

Retinal Artery

Susac syndrome (SuS) is a rare autoimmune endotheliopathy leading to hearing loss, branch retinal artery occlusions and encephalopathy. Young females are more frequently affected than males, making counselling for family planning an important issue. We reviewed published cases on SuS during pregnancy or in the postpartum period, and selected 27 reports describing the details of 33 patients with SuS. Treatment options and implications for pregnancy and breastfeeding are discussed. We propose new areas for research and suggest a management strategy.

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Neuropsychology and malnutrition: a study with 7 to 10 years-old children in a poor community

Revista Brasileira de Saúde Materno Infantil ◽

10.1590/s1519-38292007000100006 ◽

2007 ◽

Vol 7 (1) ◽

pp. 45-54 ◽

Cited By ~ 8

Author(s):

Mônica C. Miranda ◽

Fernando J. Nóbrega ◽

Kazue Sato ◽

Sabine Pompéia ◽

Elaine G. Sinnes ◽

...

Keyword(s):

Working Memory ◽

Executive Functions ◽

Cognitive Functions ◽

Maternal Mental Health ◽

Average Birth Weight ◽

Term Memory ◽

Malnourished Children ◽

Visual Spatial ◽

Neuropsychological Profile ◽

Prenatal Malnutrition

OBJECTIVES: to compare the neuropsychological profile of eutrophic, stunted and chronically malnourished children, and to analyse the role of socio-economic factors on the cognitive development. METHODS: seven to 10 year-old girls and boys from a poor community in São Paulo were evaluated: 27 eutrophyc, 31 stunted and 15 chronically malnourished. Neuropsychological evaluation involved cognitive functions not fully assessed in this population, such as working, declarative and non-declarative memories, attention and executive functions. Socio-economic indicators, maternal mental health and the children's behaviour at school were also evaluated. RESULTS: malnourished children had a lower score on the vocabulary test than the eutrophic and stunted groups (p s<0.05), performed worse in the visuospatial working memory task (p = 0.01), were more anxious than the stunted (p = 0.006), and despite having lower average birth weight than eutrophic children (p = 0.01), only two children as had prenatal malnutrition. Stunted children exhibited no impairment. No differences in socio-economic variables were found among groups. CONCLUSIONS: chronic malnutrition was associated to impairment of expressive speech, visual-spatial short-term memory and increased anxiety. No effects were observed in intellectual abilities, executive functions, verbal working memory, long-term memory, nor in visuoconstructive function. Preservation of the latter cognitive functions can be attributed to adequate environmental conditions and the lack of overall prenatal malnutrition.

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The neuropsychological profile of ADHD and relation to Wechsler intelligence scale performance

International Journal of Psychophysiology ◽

10.1016/j.ijpsycho.2012.06.013 ◽

2012 ◽

Vol 85 (3) ◽

pp. 293

Author(s):

E.E. Bakar ◽

A.Ş. Soysal ◽

Y.I. Taner ◽

S. Karakas

Keyword(s):

Intelligence Scale ◽

Neuropsychological Profile

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Neuropsychological Profile of Intellectually Gifted Children: A Systematic Review

Journal of the International Neuropsychological Society ◽

10.1017/s1355617721000515 ◽

2021 ◽

pp. 1-17

Author(s):

Aurélie Bucaille ◽

Christophe Jarry ◽

Justine Allard ◽

Sylvain Brochard ◽

Sylviane Peudenier ◽

...

Keyword(s):

Systematic Review ◽

Working Memory ◽

Cognitive Functions ◽

Empirical Studies ◽

Verbal Working Memory ◽

Social Problem Solving ◽

Gifted Children ◽

Neuropsychological Profile ◽

Intellectually Gifted ◽

Visuospatial Processing

Abstract Objective: The term intellectually gifted (IG) refers to children of high intelligence, which is classically measured by the intelligence quotient (IQ). Some researchers assume that the cognitive profiles of these children are characterized by both strengths and weaknesses, compared with those of their typically developing (TD) peers of average IQ. The aim of the present systematic review was to verify this assumption, by compiling data from empirical studies of cognitive functions (language, motor skills, visuospatial processing, memory, attention and executive functions, social and emotional cognition) and academic performances. Method: The literature search yielded 658 articles, 15 of which met the selection criteria taken from the Preferred Reporting Items for Systematic Reviews and Meta-Analyses model. We undertook a qualitative summary, to highlight any discrepancies between cognitive functions. Results: IG children exhibited better skills than TD children in a number of domains, including attention, language, mathematics, verbal working memory, shifting, and social problem solving. However, the two groups had comparable skills in visuospatial processing, memory, planning, inhibition, and visual working memory, or facial recognition. Conclusion: Although IG children may have some strengths, many studies have failed to find differences between this population and their TD peers on many other cognitive measures. Just like any other children, they can display learning disabilities, which can be responsible for academic underachievement. Further studies are needed to better understand this heterogeneity. The present review provides pointers for overcoming methodological problems and opens up new avenues for giftedness research.

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