This protocol applies to patients diagnosed with xeroderma pigmentosum who also present with abnormalities of the central and/or peripheral nervous systems — including those meeting criteria for XP/Cockayne syndrome complex (XP/CS complex).
Neurological complications are a recognised feature of xeroderma pigmentosum. Psychomotor retardation and related central and peripheral nervous system abnormalities significantly influence the progression and prognosis of the disease. When the XP/CS complex is present, specific neurological disease guidelines apply.
Management in this setting addresses nutritional status, as poor oral intake is a recognised complication. A nutritional support intervention may be considered to manage undernutrition — the complete structured regimen specifies the approach and indications.
Full protocol details, sequencing, and criteria are available via the link below.
In the majority of Japanese patients, XP is complicated with abnormalities in the central and peripheral nervous systems, such as psychomotor retardations of which progression and severity greatly influence the prognosis.
If the patient is diagnosed with XP/CS complex, conform to the guidelines for XP neurological disease.
A percutaneous endoscopic gastrostomy is often constructed for improvement of undernutrition caused by poor oral intake.