14.4
Motor Neuron Disease
Section I: Clinical Scenario and Dental Considerations
Clinical Scenario
A 59‐year‐old woman is seen for a dental review appointment during a domiciliary visit in her private home. Her main concern is that she is finding it increasingly difficult to speak as her mouth feels dry. She also reports that food constantly gets trapped in between her upper right molar teeth and the gums in this area occasionally feel swollen/bleed.
Medical History
- Amyotrophic lateral sclerosis (ALS) diagnosed 7 years ago
- Combination of oral and percutaneous gastrostomy (PEG) feeding for the last 3 years; PEG feeding predominantly used for liquids
- Recurrent episodes of pneumonia, last hospitalisation 2 years ago
- Type 2 diabetes mellitus
- Hypertension
- Gastro‐oesophageal reflux disease
Medications
- Salbutamol
- N‐acetylcysteine
- Doxofylline
- Metformin
- Linagliptin
- Telmisartan
- Amlodipine
- Lansoprazole
- Escitalopram
- Calcium carbonate
- Vitamin B complex
Dental History
- Attended the dental practice regularly until 4 years ago when she requested domiciliary visits due to her reducing mobility
- Prefers dental treatment in her wheelchair which can recline by a 25° angle
- Uses a handheld toothbrush to brush her teeth independently 2–3 times daily with 1450 ppm fluoride toothpaste
- Single‐tufted toothbrush used with chlorhexidine 1% gel to remove food packing
- Commercial mouthwash (not alcohol‐free) almost 4 times daily to wet her mouth
Social History
- Lives with her husband and 2 adult daughters
- Company director; works from home; holding company meetings remotely
- Able to talk with the aid of a wireless microphone
- Feels increasingly anxious as her ALS progresses
- Nurse and care‐giver support for all activities of daily living
- Not mobile; uses an electric wheelchair; travels if required using an adapted private car; requires a hoist for transfer
- Tobacco consumption nil; alcohol consumption 6 units a week
Oral Examination
- Xerostomia with change in consistency of saliva (frothy) (Figure 14.4.1)
- Bilateral tongue biting
- Incompetent lips/mouth breathing
- Drooling
- Full mouth rehabilitation, with porcelain fused to metal crowns and bridges on multiple teeth
- Only teeth without full coverage restorations: #17, #18, #28, #38 and #48
- Extensive composite restorations in #17 and #18 with food packing interdentally (Figure 14.4.2)
Radiological Examination
- Limited to the upper right quadrant due to poor tolerance
- Long cone periapical radiograph taken bedside during domiciliary visit (Figure 14.4.3)
- Defective contact point distal to #17; #16 and #17 root filled with posts in situ
Structured Learning
- What factors are aggravating the symptoms of xerostomia in this patient?
- Polypharmacy (including telmisartan, amlodipine, escitalopram, lansoprazole, salbutamol, N‐acetylcysteine)
- Frequent use of alcohol‐containing mouth rinse
- Anxiety
- Diabetes mellitus
- Decreased fluid intake
- Frequent suctioning of secretions
- Mouth breathing
- What advice would you give this patient to help manage her dry mouth?
- Frequent sips of water throughout the day; avoid sugary drinks
- Rinse with water after meals
- Avoid dry or hard crunchy foods
- Eat cool food with high liquid content
- Avoid exacerbating factors including non‐essential drugs associated with xerostomia, alcohol, alcohol‐containing mouthwashes, caffeine
- Lubricate the lips frequently with petroleum jelly
- Salivary stimulants
- Salivary substitutes
- Conversely, the patient reports that she also frequently experiences drooling of saliva which she finds increasingly distressing. What could be contributing to this?
- Weakness of the muscles of the mouth and throat can cause difficulties in managing saliva/swallowing, even when the overall quantity of saliva is reduced
- This can worsen if the head position falls forward due to weakness of the neck muscles
- Given that the patient participates in meetings remotely and is very embarrassed about being unable to control her drooling, she asks for urgent help. What options would you discuss with her?
- Keep the head position upright – the patient may benefit from a head support strap attached to the headrest of her electric wheelchair
- Medications to reduce the production of saliva may help but should be used with caution given that the patient also reports a dry mouth; these can be prescribed by the neurologist/physician and include hyoscine or scopolamine patches, atropine, amitriptyline and glycopyrronium (some of them could be used only occasionally ‘on request’)
- Botulinum toxin injections directly into the salivary glands can cause a temporary reduction in salivary gland production (3–6 months) and may be repeated if beneficial
- Radiotherapy of the salivary glands causes a permanent reduction in salivary gland flow; it may be given unilaterally to start with, when drooling is severe
- The patient asks for a permanent solution to the food packing in the upper right quadrant. What options would you discuss with her and what are their associated risks and benefits?
- Removing part/all of the existing restoration in the #17 and replacing it with a new composite resin restoration with a better contact point with the #18
- Risks: limited access; placement of a rubber dam will be challenging; lengthy treatment; debris generated from drilling increases the risk of aspiration; marginal leakage/further deterioration may occur
- Benefits: avoid removal of further tooth structure; can be reviewed, replenished and replaced as required
- Placing a porcelain crown on the #17
- Risks: access for crown preparation; placement of gingival retraction cord and dental impressions will be limited; additional risk of aspiration related to impression materials (risks could be reduced with a digital intraoral scanner and fabrication of an indirect composite restoration)
- Benefits: cast restorations usually deliver a better contact area with the adjacent teeth and protect the extensively restored tooth which is particularly vulnerable to fracture as it is root filled
- Dental extraction of the #17
- Risks: access to the palatal aspect of the tooth with extraction forceps may be limited; adjacent crown/bridge work may become damaged; tooth may fracture as it is root filled/heavily restored
- Benefits: permanent solution; tooth not visible; allows easier access to the adjacent teeth for cleaning
- The patient decides that she wants the #17 extracted. What other factors do you need to consider in your risk assessment?
- Social
- Complete dependence on family and carers in mobilising
- Reduced mobility and preference to have dental treatment undertaken in her wheelchair
- Increasing anxiety/depression
- Medical
- High‐risk patient due to dysphagia, recurrent aspiration pneumonia and requirement for invasive ventilation
- Increased risk of hypertensive crisis
- Diabetes mellitus is known to be associated with increased risk of infection, impaired wound healing and risk of hypoglycaemia/hyperglycaemia in the dental setting (see Chapter 5.1)
- Regurgitation of gastric contents due to gastro‐oesophageal reflux disease
- Stress from the dental environment can trigger an anxiety crisis (see Chapter 15.1)
- Dental
- Limited access for oral hygiene and dental treatment interventions
- Easily fatigued and difficulty in keeping mouth open too long
- Crown/root fracture risk during tooth extraction
- Removing part/all of the existing restoration in the #17 and replacing it with a new composite resin restoration with a better contact point with the #18