What is the recommended approach for Amyotrophic Lateral Sclerosis (ALS) screening?

Recommended Approach for ALS Screening

Screening for dysphagia should be performed in all ALS patients at diagnosis and during follow-up as part of comprehensive neurological evaluation, with videofluoroscopy recommended as the primary instrumental assessment method. 1

Initial Screening Protocol

Clinical Evaluation (at diagnosis)

• Perform comprehensive dysphagia screening using:
  • Structured questionnaires: EAT-10 (86% sensitivity, 76% specificity) 1, 2
  • Volume-Viscosity Swallow Test (V-VST) (92% sensitivity, 80% specificity) 1, 2
  • Evaluation of voluntary cough airflow 1

Instrumental Assessment

• Videofluoroscopy (VFS): Recommended for all ALS patients at diagnosis, even those without bulbar symptoms 1, 2

  • Evaluates both oral and pharyngeal phases of swallowing
  • Can identify silent aspirations and evaluate effect of compensatory postures
  • Protocol should include different consistencies and volumes of contrast bolus

• Fiberoptic Endoscopic Evaluation of Swallowing (FEES): Alternative or complementary to VFS 1

  • Identifies impaired chewing, tongue muscle deficit, velo-pharyngeal closure
  • Can detect pharyngeal residues
  • Useful as a bedside evaluation tool

Follow-up Screening Protocol

• Conduct follow-up screening every 3 months 1
• More frequent evaluation may be needed based on:
  • Presence of bulbar symptoms
  • Rate of disease progression
  • Nutritional status changes (weight loss, BMI changes)

Parameters to Monitor During Follow-up

• Lip closure and evidence of saliva pooling
• Tongue strength, mobility, and tone
• Chewing capacity
• Palatal movement in response to tactile stimulation
• Quality and strength of cough
• Phoniatric function
• Weight and BMI changes (malnutrition risk assessment)

Clinical Significance and Rationale

Dysphagia is found in 6.2-85.7% of ALS patients (48.1-85.7% in bulbar form and 41.1-71.4% in spinal form) 1. Early detection is critical as:

1. Dysphagia leads to malnutrition (present in 0-21% at diagnosis and 7.5-53% during follow-up) 1
2. Aspiration pneumonia occurs in approximately 15% of patients 1
3. Early intervention can improve quality of life and potentially extend survival

Common Pitfalls and Caveats

• Silent aspiration: Many ALS patients have silent aspirations without obvious clinical symptoms, making instrumental assessment crucial 1
• Delayed diagnosis: ALS diagnosis typically takes 12 months from symptom onset, delaying proper dysphagia management 3
• Focus on motor symptoms: Swallowing difficulties may be overlooked when focusing on limb weakness and other motor symptoms
• Underestimating early dysphagia: Even patients with "normal eating habits" may show swallowing alterations (66.7% in one study) 1

Special Considerations

• Bulbar vs. Spinal Onset: While dysphagia is more common in bulbar-onset ALS, patients with spinal-onset can also develop swallowing disorders and require screening 1
• Disease Progression: As ALS progresses through early, middle, advanced, and terminal stages, dysphagia assessment must be adapted accordingly 2
• Medication Impact: Riluzole, the standard ALS treatment, may cause nausea as a side effect (incidence ≥5%), potentially complicating dysphagia management 4

Early detection and management of dysphagia in ALS patients is crucial for reducing morbidity and mortality and improving quality of life. The systematic approach outlined above provides a comprehensive framework for screening and monitoring this critical aspect of ALS care.