What are the current guidelines for the management and supportive care of patients diagnosed with prion diseases?

Management and supportive care of patients with prion diseases primarily focus on symptomatic relief and multidisciplinary supportive interventions, as there is currently no curative treatment available ¹. The UK guidelines emphasize comprehensive assessment and management of neurological symptoms, including cognitive decline, motor dysfunction, and psychiatric manifestations, with tailored pharmacological and non-pharmacological strategies to improve quality of life ¹. Supportive care involves coordination between neurology, palliative care, nutrition, and psychological services to address complex needs.

Nutritional management is a critical component of supportive care due to progressive dysphagia and weight loss in prion diseases. Recent literature highlights the importance of early and individualized nutritional interventions, comparing East Asian and Western approaches, which suggest that proactive enteral feeding strategies may help maintain nutritional status and potentially improve patient comfort (Perna et al., 2024). This complements UK practice by reinforcing the need for early dietetic involvement and consideration of feeding tube placement when oral intake becomes insufficient ¹.

Symptom control includes managing spasticity, myoclonus, and pain with appropriate medications, alongside psychological support for anxiety and depression ¹. Given the rapid progression of prion diseases, advance care planning and end-of-life care discussions are essential components of management, ensuring patient and family preferences are respected ¹.

In summary, current guidelines recommend a holistic, multidisciplinary approach focusing on symptom management, nutritional support, and palliative care, with recent evidence underscoring the value of early nutritional interventions to optimize supportive care outcomes (Perna et al., 2024) ¹.