Table 1

Clinical requirements indicating advanced disease

General indicators of deterioration in neurological diseases
  • Progressive deterioration in physical or cognitive function despite optimal therapy

  • Complex symptoms

  • Swallowing problems leading to respiratory complications

  • Speech problems: dysarthria and progressive dysphasia

Specific clinical indicators for the disease groups
ALS/MND
  • Evidence of disturbed sleep related to respiratory muscle weakness in addition to signs of dyspnoea at rest

  • Increased cognitive difficulties/barely intelligible speech

  • Difficulty swallowing poor nutritional status/weight loss

  • Needing assistance with ADLs

  • Medical complications, eg, pneumonia, sepsis

  • A low vital capacity (below 50% of predicted, hypercapnia)

Multiple sclerosis
  1. EDSS ≥8, 5

  2. Presence of at least one of the following conditions:

    • Significant complex symptoms and medical complications

    • Dysphagia (swallowing difficulties) admissions with sepsis and poor nutritional status

    • Communication difficulties, eg, dysarthria+fatigue

    • Cognitive impairment

    • Breathlessness

Movement disorders
  1. Hoehn and Yahr stage ≥4

  2. No indication for neurosurgical procedures

  3. The presence of 2 or more of the following criteria:

    • Drug treatment is less effective or an increasingly complex regime of drug treatments

    • Reduced independence, need for help with daily living

    • Recognition that the condition has become less controlled and less predictable with “off” periods

    • Dyskinesias, mobility problems and falls

    • Swallowing problems

    • Psychiatric signs (depression, anxiety, hallucinations, psychosis)

  • Adapted from the Gold Standards Framework prognostic indicator guidance.

  • ADL, activity of daily living; ALS/MND, amyotrophic lateral sclerosis motor neurone disease.