Conduct disorder and affective disorder among the offspring of patients with Huntington's Disease

1983 ◽  
Vol 13 (1) ◽  
pp. 45-52 ◽  
Author(s):  
Susan E. Folstein ◽  
Mary Lousie Franz ◽  
Barbara A. Jensen ◽  
Gary A. Chase ◽  
Marshal F. Folstein
Keyword(s):  
Conduct Disorder ◽  
Psychiatric Disorder ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Affective Disorder ◽  
Genetic Counselling ◽  
Early Onset ◽  
Affective Illness ◽  
Hd Gene ◽  
Early Manifestation

SynopsisThe rate of occurrence of conduct disorder and affective illness was studied for a sample of 112 offspring of 34 Huntington's Disease (HD) patients. Psychiatric disorder in the offspring was assessed as a function of: (1) age of the parent at the onset of symptoms of HD; (2) family disorganization; and (3) psychiatric disorder in either parent. The findings indicated an increased frequency of conduct disorder in disrupted families, most especially in those where the HD parent had an early onset of symptoms and the non-HD parent showed psychiatric disorder. Affective disorder in the offspring was most strongly associated with the presence of similar symptoms in the HD parent. Affective disorder, but not conduct disorder, may be an early manifestation of the HD gene. The implication of these findings for genetic counselling is discussed.

Genetic counselling: a new diagnosis in the family

2020 ◽  
pp. 64-71
Author(s):  
Oliver Quarrell
Keyword(s):  
At Risk ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Genetic Counselling ◽  
Genetic Disorders ◽  
Medical Doctors ◽  
Genetic Tests ◽  
The Family ◽  
Close Relatives ◽  
New Diagnosis

This chapter describes the process of genetic counselling in general but with an emphasis on Huntington’s disease. The chapter discusses issues for a new diagnosis in the family and describes the challenges of telling children that they are at risk. Medical doctors often lead genetic counselling teams as they are specially trained to give information about genetic disorders and explain the implications of genetic tests. The doctor or counsellor has to understand your particular circumstances and support you in a way that allows you to make your own decisions. A diagnosis of HD has implications for you and all your close relatives.

Huntington’s disease-like 2: a phenocopy not to miss

2020 ◽  
Vol 20 (6) ◽  
pp. 479-481
Author(s):  
Daniel Sabino De Oliveira ◽  
Daniela Pereira Santos ◽  
Daniel Oliveira Araujo ◽  
Pedro José Tomaselli ◽  
WIlson Marques Júnior ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Genetic Counselling ◽  
Neurological Examination ◽  
Clinical Management ◽  
African Ancestry ◽  
Epileptic Seizures ◽  
Correct Identification ◽  
History Of ◽  
Slow Saccades

A 67-year-old Brazilian man of African ancestry and his 60-year-old sister both presented with choreiform movements, although in the man these were significantly overshadowed by additional parkinsonism. The man also had a history of four epileptic seizures. Neurological examination in each also found slow saccades and a dysexecutive syndrome. Genetic tests for Huntington’s disease were negative but were positive for Huntington’s disease-like 2. There are various genetic causes of chorea diseases, and their correct identification is important for appropriate clinical management and genetic counselling.

scholarly journals Early-onset sleep defects inDrosophilamodels of Huntington's disease reflect alterations of PKA/CREB signaling

2015 ◽  
Vol 25 (5) ◽  
pp. 837-852 ◽  
Author(s):  
Erin D. Gonzales ◽  
Anne K. Tanenhaus ◽  
Jiabin Zhang ◽  
Ryan P. Chaffee ◽  
Jerry C.P. Yin
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Early Onset

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)

The association of affective disorder with Huntington's Disease in a case series and in families

1983 ◽  
Vol 13 (3) ◽  
pp. 537-542 ◽  
Author(s):  
Susan E. Folstein ◽  
Margaret H. Abbott ◽  
Gary A. Chase ◽  
Barbara A. Jensen ◽  
Marshal F. Folstein
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Affective Disorder ◽  
Genetic Heterogeneity ◽  
Geographical Area ◽  
Case Series ◽  
Consecutive Case ◽  
Multiple Sources

SynopsisMajor affective disorder clinically similar to the disorder found in conditions other than Huntington's Disease (HD) was found in 41% of patients with HD in a consecutive case series ascertained through multiple sources in a defined geographical area. The association appears to be confined to certain families, and affective disorder may appear as long as 20 years before the onset of chorea and dementia. The association may represent genetic heterogeneity in HD.

Expression of the mutant allele of IT-15 (the HD gene) in striatum and cortex of Huntington's disease patients

1995 ◽  
Vol 4 (1) ◽  
pp. 15-18 ◽  
Author(s):  
O. C. Stine ◽  
S.-H. Li ◽  
N. Pleasant ◽  
M. V. Wagster ◽  
J. C. Hedreen ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Mutant Allele ◽  
Hd Gene
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