Atypical social cognitive processing in premotor Huntington’s disease: a single case study

2015 ◽  
Vol 34 (1) ◽  
pp. 53-58
Author(s):  
T. Burke ◽  
D. Healy ◽  
P. Casey ◽  
O. Hardiman ◽  
N. Pender
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Processing ◽  
Social Cognitive ◽  
Single Case ◽  
Neuropsychological Testing ◽  
Behavioural Change ◽  
Cognitive Changes ◽  
Affective Deficits ◽  
Specific Manifestation

BackgroundWe report the case of a 52-year-old male with pre-motor Huntington’s disease (HD) who has undergone detailed clinical and neuropsychological examination. This patient’s negative symptomatology and behavioural change are having a detrimental impact on his social, occupational and interpersonal life, in the absence of motor symptoms.MethodsThe patient has undergone repeat neuropsychological testing (T1 aged 50; T2 aged 52) with particular focus on executive function and social cognition on repeat testing.ResultsThis case details a specific manifestation of HD relating to behavioural, psychiatric and social affective deficits.ConclusionsThis case illustrates how social cognitive changes can occur in HD, months and even years prior to the onset of motor features and how such unrecognized deficits can have a deleterious impact on an individual’s functional ability and lifestyle, before the disease is traditionally considered to have become manifest.

Assessing Impairment of Executive Function and Psychomotor Speed in Premanifest and Manifest Huntington’s Disease Gene-expansion Carriers

2015 ◽  
Vol 21 (3) ◽  
pp. 193-202 ◽  
Author(s):  
Ida Unmack Larsen ◽  
Tua Vinther-Jensen ◽  
Anders Gade ◽  
Jørgen Erik Nielsen ◽  
Asmus Vogel
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuropsychological Testing ◽  
Distinct Group ◽  
Healthy Controls ◽  
Psychomotor Speed ◽  
Gene Expansion ◽  
Individual Scores ◽  
Hd Gene ◽  
Group Comparisons

AbstractExecutive functions (EF) and psychomotor speed (PMS) has been widely studied in Huntington’s disease (HD). Most studies have focused on finding markers of disease progression by comparing group means at different disease stages. Our aim was to investigate performances on nine measures of EF and PMS in a group of premanifest and manifest HD-gene expansion carriers and to investigate which measures were most sensitive for assessment of individual patients by analyzing frequencies of impaired performances relative to healthy controls. We recruited HD gene-expansion carriers, 48 manifest and 50 premanifest and as controls 39 healthy gene-expansion negative individuals. All participants underwent neurological examination and neuropsychological testing with nine cognitive measures. The frequency of impairment was investigated using cutoff scores. In group comparisons the manifest HD gene-expansion carriers scored significantly worse than controls on all tests and in classification of individual scores the majority of scores were classified as probably impaired (10th percentile) or impaired (5th percentile) with Symbol Digit Modalities Test (SDMT) being the most frequently impaired. Group comparisons of premanifest HD gene-expansion carriers and healthy controls showed significant differences on SDMT and Alternating fluency tests. Nevertheless the frequencies of probably impaired and impaired scores on individual tests were markedly higher for Alternating and Lexical fluency tests than for SDMT. We found distinct group differences in frequency of impairment on measures of EF and PMS in manifest and premanifest HD gene-expansion carriers. Our results indicate to what degree these measures can be expected to be clinically impaired. (JINS, 2015, 21, 1–10)

A single case of huntington's disease simultaneously occurring with obstructive hydrocephalus

1991 ◽  
Vol 35 (2) ◽  
pp. 136-138 ◽  
Author(s):  
Nicholas Y. Lorenzo ◽  
Lyal G. Leibrock ◽  
Agapito S. Lorenzo
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Single Case ◽  
Obstructive Hydrocephalus

scholarly journals Do Changes in Synaptic Autophagy Underlie the Cognitive Impairments in Huntington’s Disease?

2021 ◽  
pp. 1-12
Author(s):  
Hilary Grosso Jasutkar ◽  
Ai Yamamoto
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Impairments ◽  
Critical Role ◽  
Pathological Changes ◽  
Cognitive Changes ◽  
Underlying Pathophysiology ◽  
Insight Into ◽  
Early Manifestation ◽  
Made In

Although Huntington’s disease (HD) is classically considered from the perspective of the motor syndrome, the cognitive changes in HD are prominent and often an early manifestation of disease. As such, investigating the underlying pathophysiology of cognitive changes may give insight into important and early neurodegenerative events. In this review, we first discuss evidence from both HD patients and animal models that cognitive changes correlate with early pathological changes at the synapse, an observation that is similarly made in other neurodegenerative conditions that primarily affect cognition. We then describe how autophagy plays a critical role supporting synaptic maintenance in the healthy brain, and how autophagy dysfunction in HD may thereby lead to impaired synaptic maintenance and thus early manifestations of disease.

Neuropsychological, Neurological, and Mri Correlates of Dementia in Advanced Huntington's Disease: A Single Case Study

1991 ◽  
Vol 72 (3_suppl) ◽  
pp. 1363-1374 ◽  
Author(s):  
Alan E. Brooker ◽  
David S. Dougherty ◽  
Karl F. Love ◽  
William M. Kelly
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Single Case ◽  
Single Case Study

Huntington's Disease: Update and Review of Neuropsychiatric Aspects

1994 ◽  
Vol 24 (3) ◽  
pp. 189-208 ◽  
Author(s):  
Mario F. Mendez
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Deficits ◽  
Neuropsychological Testing ◽  
Genetically Engineered ◽  
Caudate Nuclei ◽  
Neuropsychological Profile ◽  
Personality Changes ◽  
Testing Management ◽  
Brain Behavior

Objective: This article presents a general update on Huntington's disease (HD) and reviews the psychiatric and cognitive features of this disorder. Method: HD is discussed in five sections: an introduction and update, the psychiatric aspects, the cognitive aspects, brain-behavior relationships, and the differential diagnosis and management. Results: Recent advancements in HD include the identification of presymptomatic testing methods and HD gene defect, structural and metabolic neuroimaging findings, and a neuropsychological profile. HD is associated with mood disorders, personality changes, irritable and explosive behavior, a schizophrenia-like illness, suicidal behavior, sexuality changes, and specific cognitive deficits. Conclusions: HD results in organic mental disorders from dysfunction of prefrontal-subcortical circuits coursing through the caudate nuclei. The diagnosis of HD is aided by genetic testing, neuroimaging, and neuropsychological testing. Management involves education, genetic counseling, and psychotropic medications. Finally, the future of HD holds promise for the development of rational, neurobiologically-based treatments and genetically engineered therapies.

scholarly journals Dual Task Performance May be a Better Measure of Cognitive Processing in Huntington’s Disease than Traditional Attention Tests

2015 ◽  
Vol 4 (2) ◽  
pp. 119-130 ◽  
Author(s):  
Eleftheria Vaportzis ◽  
Nellie Georgiou-Karistianis ◽  
Andrew Churchyard ◽  
Julie C. Stout
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Task Performance ◽  
Cognitive Processing ◽  
Dual Task ◽  
Dual Task Performance

scholarly journals Visuospatial Processing Deficits Linked to Posterior Brain Regions in Premanifest and Early Stage Huntington’s Disease

2016 ◽  
Vol 22 (6) ◽  
pp. 595-608 ◽  
Author(s):  
Izelle Labuschagne ◽  
Amy Mulick Cassidy ◽  
Rachael I. Scahill ◽  
Eileanoir B. Johnson ◽  
Elin Rees ◽  
...  
Keyword(s):  
Visual Search ◽  
Huntington's Disease ◽  
Mental Rotation ◽  
Huntington’S Disease ◽  
Functional Capacity ◽  
Disease Onset ◽  
Motor Symptoms ◽  
Cognitive Changes ◽  
Visuospatial Processing ◽  
Processing Deficits

AbstractObjectives: Visuospatial processing deficits have been reported in Huntington’s disease (HD). To date, no study has examined associations between visuospatial cognition and posterior brain findings in HD. Methods: We compared 119 premanifest (55> and 64<10.8 years to expected disease onset) and 104 early symptomatic (59 stage-1 and 45 stage-2) gene carriers, with 110 controls on visual search and mental rotation performance at baseline and 12 months. In the disease groups, we also examined associations between task performance and disease severity, functional capacity and structural brain measures. Results: Cross-sectionally, there were strong differences between all disease groups and controls on visual search, and between diagnosed groups and controls on mental rotation accuracy. Only the premanifest participants close to onset took longer than controls to respond correctly to mental rotation. Visual search negatively correlated with disease burden and motor symptoms in diagnosed individuals, and positively correlated with functional capacity. Mental rotation (“same”) was negatively correlated with motor symptoms in stage-2 individuals, and positively correlated with functional capacity. Visual search and mental rotation were associated with parieto-occipital (pre-/cuneus, calcarine, lingual) and temporal (posterior fusiform) volume and cortical thickness. Longitudinally, visual search deteriorated over 12 months in stage-2 individuals, with no evidence of declines in mental rotation. Conclusions: Our findings provide evidence linking early visuospatial deficits to functioning and posterior cortical dysfunction in HD. The findings are important since large research efforts have focused on fronto-striatal mediated cognitive changes, with little attention given to aspects of cognition outside of these areas. (JINS, 2016, 22, 595–608)

scholarly journals Are cognitive changes the first symptoms of Huntington's disease? A study of gene carriers

1998 ◽  
Vol 64 (2) ◽  
pp. 172-177 ◽  
Author(s):  
V Hahn-Barma ◽  
B Deweer ◽  
A Durr ◽  
C Dode ◽  
J Feingold ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Changes ◽  
Gene Carriers
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