Table 4.

Neurodevelopment: Recommendations for individuals with PROS with brain involvement

Neuropsychological assessment	All individuals should have a formal neuropsychological assessment. Upon initial diagnosis, individuals should undergo a comprehensive assessment of cognitive, communication, socialization, behavioural, and motor needs. This assessment would identify baseline neurodevelopmental deficits and strategize a plan for ongoing monitoring and treatment
Treatment plans	These should target each individual neurodevelopmental disorder (e.g., ID, ASD, ADHD, anxiety, etc.) and should be based on standard of care (e.g., applied behavioural analysis therapy for autism). For affected infants and toddlers, some locales provide early intervention programs which evaluate for developmental delay and provide developmental services pertaining to different developmental domains
For school age children	Parents/caretakers should consider requesting an individualized education plan (IEP) or an Education and Healthcare Plan (EHCP) in order to optimize learning and development. Depending on the individual’s developmental profile, possible developmental services that may be warranted include physical therapy, occupational therapy, and speech and language therapy. In individuals who are nonverbal, augmentative and alternative communication may be helpful
Transition	Assessments should be offered around normal transition periods (i.e., puberty/move to high school and transition to adult care). These often entail a change in behaviour or routines that need be taken in consideration along with the individual’s (a) range of cognitive abilities, (b) coping/social strategies in view of possible disfiguring lesions, (c) possible co-existing autistic traits and (d) size/overgrowth
Neurological decline/developmental regression	The range of developmental levels is wide, and individuals can be expected to progress along their own learning curve. Loss of developmental milestones should prompt evaluation for treatable causes of developmental regression (e.g., shunt malfunction or tethered cord). A neurological decline may be caused by worsening of a Chiari malformation with the associated risk of developing a syrinx or hydrocephaly, and primary or repeat MRI imaging should be considered. Specific complaints to consider are (paroxysmal) neck pain, limb weakness, swallowing problems, and sleep apnoea