scholarly journals White and Gray Matter Abnormalities in Manifest Huntington's Disease: Cross‐Sectional and Longitudinal Analysis

2020 ◽  
Vol 30 (3) ◽  
pp. 351-358 ◽  
Author(s):  
Wafaa Sweidan ◽  
Fen Bao ◽  
Navid‐Seraji Bozorgzad ◽  
Edwin George
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Gray Matter ◽  
Longitudinal Analysis ◽  
Cross Sectional

scholarly journals Differential Diagnosis of Chorea—HIV Infection Delays Diagnosis of Huntington’s Disease by Years

2021 ◽  
Vol 11 (6) ◽  
pp. 710
Author(s):  
Jannis Achenbach ◽  
Simon Faissner ◽  
Carsten Saft
Keyword(s):  
Hiv Infection ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Onset ◽  
Diagnostic Delay ◽  
Depression Scale ◽  
Cag Repeat ◽  
Psychiatric Disease ◽  
Disease Manifestation ◽  
Cross Sectional

Background: There is a broad range of potential differential diagnoses for chorea. Besides rare, inherited neurodegenerative diseases such as Huntington’s disease (HD) chorea can accompany basal ganglia disorders due to vasculitis or infections, e.g., with the human immunodeficiency virus (HIV). The clinical picture is complicated by the rare occurrence of HIV infection and HD. Methods: First, we present a case suffering simultaneously from HIV and HD (HIV/HD) focusing on clinical manifestation and disease onset. We investigated cross-sectional data regarding molecular genetic, motoric, cognitive, functional, and psychiatric disease manifestation of HIV/HD in comparison to motor-manifest HD patients without HIV infection (nonHIV/HD) in the largest cohort of HD patients worldwide using the registry study ENROLL-HD. Data were analyzed using ANCOVA analyses controlling for covariates of age and CAG repeat length between groups in IBM SPSS Statistics V.25. Results: The HD diagnosis in our case report was delayed by approximately nine years due to the false assumption that the HIV infection might have been the cause of chorea. Out of n = 21,116 participants in ENROLL-HD, we identified n = 10,125 motor-manifest HD patients. n = 23 male participants were classified as suffering from HIV infection as a comorbidity, compared to n = 4898 male non-HIV/HD patients. Except for age, with HIV/HD being significantly younger (p < 0.050), we observed no group differences regarding sociodemographic, genetic, educational, motoric, functional, and cognitive parameters. Male HIV/HD patients reported about a 5.3-year-earlier onset of HD symptoms noticed by themselves compared to non-HIV/HD (p < 0.050). Moreover, patients in the HIV/HD group had a longer diagnostic delay of 1.8 years between onset of symptoms and HD diagnosis and a longer time regarding assessment of first symptoms by the rater and judgement of the patient (all p < 0.050). Unexpectedly, HIV/HD patients showed less irritability in the Hospital Anxiety and Depression Scale (all p < 0.05). Conclusions: The HD diagnosis in HIV-infected male patients is secured with a diagnostic delay between first symptoms noticed by the patient and final diagnosis. Treating physicians therefore should be sensitized to think of potential alternative diagnoses in HIV-infected patients also afflicted by movement disorders, especially if there is evidence of subcortical atrophy and a history of hyperkinesia, even without a clear HD-family history. Those patients should be transferred for early genetic testing to avoid further unnecessary diagnostics and improve sociomedical care.

Gray Matter Vulnerabilities Predict Longitudinal Development of Apathy in Huntington's Disease

2021 ◽  
Author(s):  
Audrey E. De Paepe ◽  
Alberto Ara ◽  
Clara Garcia‐Gorro ◽  
Saül Martinez‐Horta ◽  
Jesus Perez‐Perez ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Gray Matter ◽  
Longitudinal Development

Longitudinal atrophy characterization of cortical and subcortical gray matter in Huntington’s disease patients

2019 ◽  
Vol 51 (8) ◽  
pp. 1827-1843 ◽  
Author(s):  
Gabriel Ramirez‐Garcia ◽  
Víctor Galvez ◽  
Rosalinda Diaz ◽  
Leo Bayliss ◽  
Juan Fernandez‐Ruiz ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Gray Matter

Evaluation of cerebellar gray matter damage in huntington's disease: a voxel based morphometry study

2015 ◽  
Vol 357 ◽  
pp. e216-e217
Author(s):  
P.C. Azevedo ◽  
R.P. Guimarães ◽  
C. Piccinin ◽  
B. Campos ◽  
L.G. Piovesana ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Gray Matter ◽  
Voxel Based Morphometry ◽  
Cerebellar Gray Matter

scholarly journals Epidemiology and health care utilization of patients suffering from Huntington’s disease in Germany: real world evidence based on German claims data

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Christoph Ohlmeier ◽  
Kai-Uwe Saum ◽  
Wolfgang Galetzka ◽  
Dominik Beier ◽  
Holger Gothe
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Claims Data ◽  
Routine Care ◽  
Care Utilization ◽  
Research Database ◽  
Cross Sectional ◽  
Case Identification ◽  
Starting Point ◽  
Icd 10

Abstract Background Huntington’s disease (HD) is a rare, genetic, neurodegenerative and ultimately fatal disease with no cure or progression-delaying treatment currently available. HD is characterized by a triad of cognitive, behavioural and motor symptoms. Evidence on epidemiology and management of HD is limited, especially for Germany. This study aims to estimate the incidence and prevalence of HD and analyze the current routine care based on German claims data. Methods The source of data was a sample of the Institute for Applied Health Research Berlin (InGef) Research Database, comprising data of approximately four million insured persons from approximately 70 German statutory health insurances. The study was conducted in a retrospective cross-sectional design using 2015 and 2016 as a two-year observation period. At least two outpatient or inpatient ICD-10 codes for HD (ICD-10: G10) during the study period were required for case identification. Patients were considered incident if no HD diagnoses in the 4 years prior to the year of case identification were documented. Information on outpatient drug dispensations, medical aids and remedies were considered to describe the current treatment situation of HD patients. Results A 2-year incidence of 1.8 per 100,000 persons (95%-Confidence interval (CI): 1.4–2.4) and a 2-year period prevalence of 9.3 per 100,000 persons (95%-CI: 8.3–10.4) was observed. The prevalence of HD increased with advancing age, peaking at 60–69 years (16.8 per 100,000 persons; 95%-CI: 13.4–21.0) and decreasing afterwards. The most frequently observed comorbidities and disease-associated symptoms in HD patients were depression (42.9%), dementia (37.7%), urinary incontinence (32.5%), extrapyramidal and movement disorders (30.5%), dysphagia (28.6%) and disorders of the lipoprotein metabolism (28.2%). The most common medications in HD patients were antipsychotics (66.9%), followed by antidepressants (45.1%). Anticonvulsants (16.6%), opioids (14.6%) and hypnotics (9.7%) were observed less frequently. Physical therapy was the most often used medical aid in HD patients (46.4%). Nursing services and speech therapy were used by 27.9 and 22.7% of HD patients, respectively, whereas use of psychotherapy was rare (3.2%). Conclusions Based on a representative sample, this study provides new insights into the epidemiology and routine care of HD patients in Germany, and thus, may serve as a starting point for further research.

scholarly journals Cross-sectional and longitudinal voxel-based grey matter asymmetries in Huntington's disease

2018 ◽  
Vol 17 ◽  
pp. 312-324 ◽  
Author(s):  
Lora Minkova ◽  
Sarah Gregory ◽  
Rachael I. Scahill ◽  
Ahmed Abdulkadir ◽  
Christoph P. Kaller ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Grey Matter ◽  
Cross Sectional
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