Case 1 : Huntington’s Disease Management
Medical History
- Huntingdon’s Disease (Dx 2000)
- Diagnosed 25 years prior, indicating advanced progression with severe chorea (involuntary, dance-like movements).
- Moderate Chorea
- Dysphagia ( Severe )
- declined PEG
- The patient declined a percutaneous endoscopic gastrotomy (PEG) feeding tube, preferring to maintain her quality of life.
- Speech and Language Therapist, stabilised BMI
- The stabilized BMI indicates successful management of her swallowing.
Social History
- used to work + housekeep
- The patient was a former accountant and housekeeper, indicating she was a very capable individual.
- Walking stick
Dental History
- Moderate caries risk
- Food pouching
- This leads to characteristic buccal caries , which are uncommon in the general population.
- Self-brushing after meals
- The patient is unable to perform effective toothbrushing due to severe movements.
- MEDS: Tetrabenazine, Citalopram (SSRI)
Management
The main challenge for management was the patient’s severe, involuntary movements.
- I/O Exam with Clinical Holding/ Protective Stabilisation
- Shared-decision/informed consent
- This involved the patient, her next of kin, and her physician.
- Mild Sedation: 6mg Midazolam Orally, 20mins prior
- This very low dose was enough to attenuate the dyskinesia significantly without requiring full sedation.
- CAP 15 Distal Occlusal
- Water control
- Water spray was carefully controlled due to the patient’s dysphagia.
- Mouth prop