Treatment of BPAN is symptomatic. Long-term follow-up in children is usually coordinated by a paediatric neurologist or developmental paediatrician. Adults will often need a referral to a movement disorders neurologist once they enter a period of regression with parkinsonism.
Following the initial diagnosis, the following are recommended:
• Complete neurologic exam and developmental assessment with attention to motor function, possible seizures, speech, swallowing / feeding and sleep
• Ophthalmologic evaluation
• Consultation with a clinical geneticist and/or genetic counsellor
Treatment for manifestations may include the following:
• Management of seizures should be tailored to each individual
• Developmental delay / intellectual disability should be managed with standard interventions such as formal early intervention programs, speech therapy, physical therapy, occupational therapy and development of an individualized education plan. Consider referral to specialist in augmentative and alternative communication.
• Motor dysfunction should be addressed as appropriate with physical therapy, occupational therapy, and durable medical equipment as needed. For common muscle tone abnormalities, consider involving appropriate specialists to manage use of baclofen, Botox, anti-parkinsonian medications, or orthopaedic procedures.
• Once parkinsonism emerges, referral to a movement disorders specialist may be helpful
• Disordered sleep can be extremely disruptive to the entire family and may benefit in some cases from pharmacologic treatment with melatonin, chloral hydrate, or benzodiazepines
• Eye findings are treated in a routine manner
• A gastric feeding tube may eventually be indicated to help maintain adequate weight and reduce the risk of aspiration pneumonia. GE reflux and constipation and also common and may be treated in a routine manner.
WDR45