Which of the following is associated with a tall stature, decreased intelligence and has historically been linked with criminal deviancy?
Exam Question Oct 2014
XYY (aka Jacobs syndrome) is associated with tall stature and decreased intelligence. Despite previous claims, the evidence does not support a link with criminal deviancy (Re 2015).
Re L (2015) The 47,XYY syndrome, 50 years of certainties and doubts: A systematic review. Aggression and Violent Behavior 22, 9-17
Exam Question Oct 2014
XYY (aka Jacobs syndrome) is associated with tall stature and decreased intelligence. Despite previous claims, the evidence does not support a link with criminal deviancy (Re 2015).
Re L (2015) The 47,XYY syndrome, 50 years of certainties and doubts: A systematic review. Aggression and Violent Behavior 22, 9-17
Genetic conditions
| Condition | Chromosome | Features |
|---|---|---|
| Downs (trisomy 21) | 21 | Short stature, simian crease, almond shaped eyes due to epicanthic folds, upslanting palpebral fissures, low set ears, brushfield spots, and poor muscle tone. Most have mild (IQ 50-70) to moderate (IQ 35-50) mental retardation |
| Angelman syndrome (Happy puppet syndrome) | 15q11 maternal origin | Flapping hand movements (uplifted, flexed arms when walking), ataxia, pronounced verbal delay (compared to comprehension), serveve to profound learning disability, seizures and sleep problems |
| Prader-Willi | 15q11 paternal origin | Hyperphagia, excessive weight gain, short stature, frequent skin picking, mild learning disability, small gonads, and hypotonia |
| Cri du chat | 5p deletion | Characteristic cry like a meowing kitten, hypotonia, hypertelorism, a down-turned mouth, and microcephaly |
| Velocardiofacial syndrome (Di George syndrome) | 22q (deletion) | Cleft palate, cardiac problems, and learning disabilities. A higher rate of psychiatric disorders is also seen |
| Edwards's syndrome (trisomy 18) | 18 | Kidney malformations, upturned nose, webbing of second and third toes, and clubbed feet (rocker bottom) |
| Lesch-Nyhan syndrome | Xq26-27 | Self mutilation, dystonia and writhing movements |
| Smith-Magenis syndrome | 17p11 | Pronounced self injurious behaviour, self hugging, and a hoarse voice |
| Fragile X | X | Elongated face, large ears, large testicles, hand flapping, shyness, and little eye contact |
| Wolf Hirschhorn syndrome | 4p | Profound mental retardation, microcephaly, seizures, down turned fishlike mouth, Greek warrior helmet face, and cleft lip |
| Patau syndrome (trisomy 13) | 13 | Mental retardation, polydactyl, microcephaly, overlapping of fingers over thumb |
| Rett syndrome | Xq28 | Normal for the first 12 months. Regression and loss of skills from around 18 months onwards. Hand-wringing movements are the most common feature. Associated learning disability is profound. Affects girls almost exclusively |
| Tuberous sclerosis | Genetically heterogeneous, linkage to 9q and 16p | Hamartomatous tumours affect various organs including the brain. 80% suffer with epilepsy. Autism, ADHD, and sleep problems are common |
| Williams syndrome | 7q11 deletion | Elfin like features, social disinhibition, and abnormal friendliness towards strangers. Very sensitive hearing is also seen. Advanced verbal skills, speech is articulate but superficial (referred to as cocktail party speech) |
| Rubinstein-Taybi syndrome | Unclear, 16p deletions have been reported | Tend to be short with small heads. Associated with a friendly disposition and moderate learning disability |
| Klinefelter syndrome | Extra X chromosome in phenotypic males (47 XXY) | Tend to be tall with small testicles. Typically introverted with poor social and school performance |
| Jacob's syndrome | Extra Y chromosome (47 XYY) | Tend to be tall. Sexual development is normal. Tend to have lower mean intelligence. Early links with criminality have not been supported |
| Coffin-Lowry syndrome | Xp22 | Short stature, slanting eyes with short broad nose. Severe learning difficulty |
| Turner syndrome | 45 X0 | Short stature, webbed neck, widely spaced nipples |
| Niemann Pick disease (types A and B) | 11p15 | Abdominal swelling, cherry red spot, feeding difficulties |
