TABLE 4.

Implications for Occupational and Physical Therapists

Recommend medical genetic evaluation if they recognize a phenotypic pattern consistent with XXY or XXYY syndromes:

▪ Features such as tall stature, clinodactyly (curved 5th finger), hypotonia, flat feet, joint hyperextensibility, radioulnar synostosis, and/or testosterone deficiency in a child or adolescent with minimal or no facial dysmorphic features.

▪ In early development, features such as hypotonia or delayed motor milestones.

▪ Intention tremor in a child or adolescent.

▪ Motor coordination and manual dexterity deficits, or global motor deficits, with relative strengths in visual perceptual skills.

▪ The presence of comorbid conditions such as learning disabilities, language delays, sensory sensitivities, anxiety, or autism symptoms.

In individuals with an established diagnosis of XXY or XXYY, consider features of the syndrome and the profile of strengths and weaknesses when setting therapy goals and planning interventions:

▪ Utilize areas of relative strengths with this population when planning intervention strategies, including strengths in visual perceptual and visual memory skills.

▪ Individuals with XXY and XXYY with lower visual motor integration abilities have lower adaptive functioning and daily living skills, therefore therapies targeting motor skills may lead to improvement in adaptive outcomes.

▪ When developing a treatment plan for XXY or XXYY, consider therapy approaches to address impairments in manual dexterity, motor praxis, and bilateral integration

▪ Offer parent education and materials for implementation of home-based activities using visual strategies that capitalize on the strengths in nonverbal, visual-spatial skills

▪ Testosterone deficiency often develops during adolescence in XXY and XXYY, and some deficits in motor strength and stamina may respond to testosterone therapy by an endocrinologist.

▪ Difficulties with completing self-care demands and learning new motor or adaptive skills may be exacerbated by neuropsychological weaknesses associated with XXY or XXYY such as learning disabilities, attention deficits, slow processing speed, language disorders, executive dysfunction or anxiety/emotional symptoms. These must also be evaluated and considered when developing an intervention program.

▪ We recommend using the International Classification of Functioning, Disability, and Health (ICF) model (WHO 2001) along with the Occupational Therapy Practice Framework (OTPF), (American Occupational Therapy Association, 2014) and American Physical Therapy Association’s guide to Physical Therapist Practice (2001) to guide evaluation and intervention goals.

▪ Provide support and direction to families to promote healthy lifestyles and successful motor activities during recreation and leisure time. Children and adolescents with XXY and XXYY often do well in and enjoy individual sports such as swimming, cycling, martial arts, dance, golf, or skiing. Other activities based on interest and skill should not be excluded.

▪ Address difficulties with self-regulation, coping skills, sensory sensitivities, and provide other behavioral strategies to address anxiety and adaptive skills.

▪ Provide consultation to families and school teams that includes specific examples of how the impairments associated with XXY or XXYY present in academic, home, and community settings.