Williams-Beuren syndrome (WBS) involves a unique mental profile, characterized by overly friendly, empathetic personalities, but also significant challenges like ADHD, severe anxiety, and specific phobias, alongside mild intellectual disability with strong verbal skills but poor visuospatial abilities, affecting concentration and learning. Key mental symptoms include social disinhibition, high anxiety, phobias, attention deficits, and difficulties with abstract thinking, despite strong language and rote memory.
People with WS were delayed in comprehending basic emotions of anger and surprise. Deviant replacement patterns of basic emotions in people with WS were observed. Deficient emotion knowledge may be a cause of atypical social cognition.
What are the symptoms of Williams syndrome?
Changes with Age in Williams Syndrome
The profile of cognitive strengths and weakness reported in Williams syndrome also remains relatively stable with age, although some improvements with age are reported, particularly for performance IQ. Reading and spelling may improve but only minimally.
Individuals with Williams syndrome have also been strongly associated with high anxiety levels, as well as phobia development,17,18 hyperacusis,12 attention-deficit/hyperactivity disorder (ADHD; 70% of individuals with Williams syndrome have been diagnosed with ADHD),7,14,15 and related psychological symptoms such as ...
The majority of behavioural difficulties in people with Williams syndrome tend to relate to problems in attention and impulsivity. There is little evidence for self-injury in Williams syndrome.
The mental age of participants was assessed using the Woodcock-Johnson Test of Cognitive Ability-Revised (WJ-COG-R; Woodcock & Johnson, 1989–1990). The mean mental age of the entire sample was 6 years 3 months (range: 2.16–10.58 years), typical of the WS population.
Affected individuals have outgoing, engaging personalities and tend to take an extreme interest in other people. Attention deficit disorder (ADD), problems with anxiety, and phobias are common among people with this disorder.
Most individuals with Williams syndrome have a degree of intellectual disability, ranging from mild to severe. Research studies have suggested that IQ scores for children with Williams syndrome range from 40 to 112, with a mean IQ score of 68 which equates to a mild intellectual disability (average IQ is 100).
Treatment with gonadotropin-releasing hormone (GnRH) agonists may be considered. Although data are limited, buspirone has been shown to be effective in the treatment of generalized anxiety disorder in patients with Williams syndrome.
Most older children and adults with WS speak fluently and use good grammar. More than 50% of children with WS have attention deficit disorders (ADD or ADHD), and about 50% have specific phobias, such as a fear of loud noises. The majority of individuals with WS worry excessively.
MRI studies of WS have demonstrated a series of brain abnormalities, including decreased brain size, with a relatively greater decrease in the volume of the cerebral white matter volume as compared to the cerebral gray matter.
Additional manifestations can include sleep problems, ocular issues, hearing loss, dental problems, gastrointestinal difficulties, urinary tract abnormalities, and musculoskeletal issues.
distinctive facial features – including a long upper lip, small chin, generous lips, chubby face, upturned nose and flattened nose bridge. Some people with Williams syndrome may have a squint. weight and growth problems – a newborn baby with William syndrome may have a low birth weight and gain weight slowly.
This is thought to be due to a combination of genetic and neurological factors that affect the development of social and emotional skills in people with Williams syndrome. This trait is thought to be partly due to the deletion of several genes involved in the development of anxiety and fear.
Common issues include: reflux, abdominal pain, constipation, diarrhea, irritable bowel syndrome, and dysmotility. Less common but significant issues include diverticulitis (at much younger age than is common in the general population)celiac disease, and gluten intolerance.
Individuals with WS typically have an intellectual disability, learning difficulties and impairments in more specific areas of cognitive functioning, such as visual-spatial construction, attention and executive functions (Cherniske et al., 2004; Thomaidis, Amenta, Youroukos, & Fryssira, 2004; Wilska & Kaski, 2001).
Children with WS CAN learn to read. Proficiency with reading is DIRECTLY tied to instructional methods. Children need systematic phonics instruction that includes phoneme manipulation. Listening comprehension is crucial to overall understanding.
(7/15) The Myth: People with Williams syndrome can't/won't drive. Mythbuster: A small but growing number of individuals are obtaining licenses and drive themselves to work and around town daily – in cars or even a few on motorcycles.
People with Williams syndrome can develop physical and mental health problems later in life, including anxiety and depression. People with Williams syndrome are very talkative and can be excessively friendly.
Residents with Williams syndrome who desire employment are often enrolled in Job Skills Training. They learn the importance of staying on task, arriving at work on time, taking directions and adopting appropriate behaviors in an employment setting.
No single dental finding was pathognomonic of WS, however patterns of oral findings may be characteristic of the syndrome. The most common constellation of findings -- microdontia, anterior crossbite, tongue thrusting, and excessive interdental spacing-- occurred in approximately one-third of all patients.
Developmental disabilities should be addressed by early intervention programs, special education programs, & vocational training. Recommended therapies include speech-language therapy, PT, & OT. Consider hippotherapy (use of equine movement during speech-language therapy, PT, &/or OT).
Individuals born with Williams syndrome have a normal life expectancy but may experience medical, cognitive and behavioral challenges of varying severity.
Infants often have delayed development and can develop physical and mental health problems, including anxiety and depression in later life. WS people tend to be talkative and excessively friendly towards adults.