Management of Increased Confusion and Dysarthria in a 78-Year-Old Patient with Huntington's Disease
The optimal management approach for a 78-year-old patient with Huntington's disease presenting with increased confusion and dysarthria requires ruling out delirium first, then addressing both symptoms with a combination of pharmacological and non-pharmacological interventions tailored to the underlying causes.
Initial Assessment: Distinguishing Delirium from Disease Progression
First, it's critical to determine whether the increased confusion and dysarthria represent:
- Acute delirium (potentially reversible)
- Disease progression (requiring symptomatic management)
Delirium Assessment
According to geriatric emergency guidelines 1, delirium has these distinguishing features:
- Acute onset (vs. insidious in dementia)
- Fluctuating course
- Disordered attention and consciousness
- Often with hallucinations
Common Precipitating Factors to Investigate:
- Infections (particularly UTI and pneumonia)
- Medications (especially anticholinergics)
- Metabolic disturbances (dehydration, electrolyte abnormalities)
- Hypoxia
- Pain
Management Algorithm
Step 1: Address Reversible Causes
- Discontinue high-risk medications - particularly benzodiazepines which are contraindicated in decompensated conditions 1
- Treat infections if present
- Correct dehydration and electrolyte disturbances
- Provide adequate pain control if needed
- Optimize oxygenation if required
Step 2: Manage Dysarthria
For functional articulation disorders 1, implement:
Reduction of excessive musculoskeletal tension in speech and non-speech muscles
Speech therapy interventions:
- Slow speech down or elongate sounds
- Focus on normal movements and sounds
- Use nonsense words or syllable repetitions to demonstrate potential for normal function
- Redirect patient focus from speech to other topics
- Consider dual tasking while speaking as a form of distraction
Collaborative treatment with physiotherapy or occupational therapy if there is functional facial weakness, spasm, or trismus
Step 3: Manage Chorea if Contributing to Confusion/Dysarthria
Tetrabenazine is the most effective medication for controlling chorea in Huntington's disease 2. However, caution is required:
- Starting dose: 12.5 mg once daily in the morning
- Titration: Increase slowly at weekly intervals by 12.5 mg daily
- Maximum dose: Up to 100 mg daily (divided three times daily) based on response and tolerability 3
WARNING: Tetrabenazine carries a black box warning for depression and suicidality 3. Close observation is required, particularly in patients with a history of depression.
Step 4: Supportive Care
- Foster orientation: Use calendars, clocks, caregiver identification; clearly explain all activities 1
- Ensure adequate nutrition and hydration
- Regulate bowel/bladder function
- Increase supervised mobility when possible
- Provide appropriate sensory stimulation
- Minimize use of restraints whenever possible
- Minimize chemical sedation whenever possible
Pitfalls to Avoid
- Misattributing symptoms to HD progression without investigating reversible causes
- Overuse of antipsychotics for behavioral symptoms (can worsen parkinsonism)
- Inadequate monitoring for depression when using tetrabenazine
- Overlooking the possibility of late-onset HD - even at 86 years, new diagnoses can occur 4
- Failure to recognize atypical presentations - HD can sometimes present with cerebellar features rather than chorea 5
Follow-up and Monitoring
- Regular reassessment of mental status as changes may wax and wane 1
- Monitor for medication side effects, particularly depression with tetrabenazine
- Adjust interventions based on response and disease progression
By systematically addressing reversible causes first, then implementing targeted interventions for dysarthria and chorea, quality of life can be optimized even in advanced Huntington's disease.