SpecialCareDentistry

Carole A Boyle

Caroline Frölander and Graham Manley

Providing Dental Care for Patients

with Huntington’s Disease
Abstract: This paper describes the genetic condition Huntington’s disease and its clinical relevance to dentists. The treatment of two
patients with the condition is described: one under general anaesthesia, the other with intravenous sedation.
Clinical Relevance: Dentists may have patients who develop Huntington’s disease in later life, so this paper will help them consider the
longer term effects of this disease and help in planning dental care.
Dent Update 2008; 35: 333-338

Huntington’s disease (HD) is a A small number of cases are sporadic difficulty driving and making decisions.
hereditary disorder of the central nervous caused by new gene mutations. As the disease progresses,
system. It affects both men and women Inheritance is autosomal speech becomes slurred and other bodily
and develops in adulthood presenting dominant: each child of an affected functions including swallowing, eating,
with a variety of symptoms. The incidence adult has a 50% chance of inheriting the and speaking begin to fail. Walking
is estimated to be 1 in 20 0001 in the UK, disease. Genetic testing is available but becomes more difficult and the assistance
although there is variance worldwide with is controversial: there is no cure for the of a wheelchair is required. Some people
lower incidences in Asian and African disease and many people choose not to remain aware and are able to express
populations. It is caused by a faulty gene know if they are affected. appropriate emotions whilst others cannot
on chromosome 4 which leads to cerebral Juvenile Huntington’s disease even recognize family members. In the
atrophy. The gene was discovered in 1993. (JHD) is relatively rare and refers to the later stages, full nursing care is required.
development of the signs or symptoms The duration of the disease ranges from 10
in someone less than 20 years old.2 to 30 years.
The cause of death is often
secondary to HD: infection, most often
Signs and symptoms pneumonia related to aspiration and
Carole A Boyle, BDS, MMedSci, FDS RCS, Symptoms usually develop injuries related to a fall. There is also a
MFGDP(UK), MSNDRCESEd, Consultant between the ages of 30 and 50 and can significant incidence of suicide, particularly
in Special Care Dentistry, Department be present for some time before being in people who have looked after a parent
of Sedation and Special Care Dentistry, recognized. This is particularly true or other family member with the disease.
King’s College London Dental Institute where there is no family history of HD.
at Guy’s, King’s College and St Thomas’ Early signs include
Hospitals, Floor 26, Guy’s Tower, London uncontrollable muscle movements, Diagnosis
SE1 9RT, Caroline Frölander, BDS, MFDS clumsiness, stumbling when walking An experienced neurologist can
RCS, MSc, Senior Dental Officer, Brambly’s and lack of concentration. There can make a diagnosis from the history with
Grange Dental Clinic, Brambly’s Drive, be significant mood and personality particular reference to a family history and
Basingstoke, Hants RG21 8UW and changes including depression, a physical examination. Tests of balance,
Graham Manley, BDS, DDPHRCS, MSc, aggression and anti-social behaviour. HD eye movement, strength, reflexes, balance,
PhD, Head of Dental Department, The can affect cognitive functions including sensation, hearing, co-ordination and
Royal Hospital for Neuro-disability, West memory. Early signs may include involuntary movements will be carried out.
Hill, Putney, London, SW15 3SW, UK. problems remembering new facts, The physician will enquire about changes
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in intellectual and emotional function.3 the location of the gene is the first step The screws were tightened and one year
Presymptomatic testing can on the way to discovering a cure. later the denture remained stable.
be performed on adults and children.
Difficulties can arise within families: if the
grandchildren of an affected adult test Dental implications Sedation and general
positive, it means that their at-risk parent Literature relating to dental anaesthesia
is also positive, even if they did not want care in people with Huntington’s disease In the latter stages of the
to know their status. Problems can arise is scarce. Moline and Iglehart4 describe disease, many patients with Huntington’s
between siblings if some are tested and the general characteristics of HD and will need general anaesthesia for
others are not. relate individual clinical features to dental treatment, and Cangemi and
It is possible to carry out potential problems in the provision Miller7 report a case of dental clearance
prenatal testing, but this involves risks of dental care. Dental examination for under general anaesthesia in a 69-year-
to the unborn child. A positive test on a 39-year-old woman, facilitated by old with Huntington’s. There are
the unborn child means the at-risk conscious sedation with intravenous special considerations in anaesthestic
parent is also a gene carrier. Because diazepam, is described, but the degree management, which include avoidance
genetic testing carries such practical of control of involuntary movements of anticholinergic drugs, which may
and emotional consequences, not just was insufficient to allow provision of the increase choreiform movements. Certain
for the person taking the test but for extensive dental care which was found maintenance agents, such as sodium
their whole family, this is a complex and to be necessary. The treatment was thiopental, are thought to be associated
controversial area. There are guidelines in carried out under general anaesthesia with prolonged apnoea but propofol has
place regarding genetic counselling and (GA); 19 carious lesions were restored been found to be safe. Dysphagia increases
the testing process typically involves a and three teeth required extraction. the risk of aspiration in the immediate
number of visits with different healthcare A dental recall interval of three postoperative period and care must be
professionals before the test is taken. months was advised and fluoride trays taken to ensure that airway reflexes have
Testing is not normally carried out in under constructed. Unfortunately, perhaps as returned before extubation.
18-year-olds. a result of a mood disorder associated There is no contra-indication to
with the condition, the patient was the use of sedation, either with intravenous
reluctant to accept assistance with oral midazolam or inhaled nitrous oxide.
Treatment hygiene from carers. However, the use of sedation requires
There is no treatment for HD, There are, however, reported a high degree of awareness of airway
but medication can improve the quality cases of provision of complex dental protection and management. People
of life for sufferers. Antipsychotic drugs procedures in people with Huntington’s. with HD have a reduced ability to protect
may help to alleviate muscle movements Bradford et al 5 report the case of a their airway and the additional muscle
and can help to control hallucinations, patient who claimed that his difficulties relaxation effect of the sedation can add to
delusions, and violent outbursts. in accessing dental care were related this problem. It is therefore essential that
Antidepressants and tranquillizers are to a reluctance of local practitioners to high velocity suction and local packing
commonly prescribed to improve mood treat him in view of his Huntington’s. are employed to ensure patient safety.
and reduce temper swings. Most drugs He was reluctant to lose any teeth and Inhalational sedation will be impossible
used in treatment can cause side-effects, successful endodontic treatment on in the later stages of the disease when
including xerostomia, excitement, two upper premolars was eventually uncontrolled movements will make it
restlessness and fatigue. It can be difficult completed at a dental hospital. difficult to maintain a good seal around
to distinguish if a symptom is a sign of the Jackowski and colleagues6 the nasal hood.
disease or due to the drugs.3 describe a case of the provision
Other treatment includes of an implant-retained complete
speech therapy to improve speech and lower overdenture for a patient with Case study 1: The use of
reduce swallowing problems. Patients with Huntington’s. Such prosthesis was able general anaesthesia
involuntary movements use up a great to overcome the problems associated Mrs A was referred to a dental
deal of energy and a high calorie diet is with denture retention which are teaching hospital by her general dental
often recommended to prevent weight described later and include orofacial practitioner for dental care. He found
loss. dyskinesia and xerostomia. Construction it difficult to examine her owing to her
of the prosthesis was technically difficult involuntary movements and her husband
and occlusal dimensions had to be felt she was in pain.
The future determined largely from anatomical The patient was accompanied
Research is continuing into landmarks. Fixtures became loose by her husband to the initial assessment
developing better drugs to control two months following completion of appointment and attended in a wheelchair.
symptoms and to delay the onset of the treatment which was thought to be Mrs A was diagnosed with HD in 1992 at
disease. It is hoped that identification of caused by excessive tongue movements. the age of 58. There was no family history
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of HD and it was thought to be due to full mouth scaling, extraction of all molar albeit being in the end stage of HD. Her
a spontaneous mutation. She had four teeth and anterior restorations. medication included Olanzapine 5 mg
children in their 40s all of whom had been Recovery was uneventful and once/day, Tetrabenazine 25 mg three
tested for the gene. The initial presentation Mrs A returned home the following day. A times daily, Lansoprazole 30 mg once/
of the disease had been a reduction in review with a hygienist was arranged for day, Clonazepam 1 mg at night. A decision
dexterity when playing the viola: she had advice regarding oral hygiene. She will be had been taken not to proceed with
been a music teacher. She had no other seen for yearly recalls. resuscitation in the event of an arrest
medical problems and was not taking any (DNAR). With the introduction of an
medication. intravenous sedation service within the
On examination, Mrs A was a Case study 2: Sedation hospital in 2006, it was decided to provide
thin lady with violent movements of her A 65-year-old lady with late treatment using intravenous sedation. Over
head and arms. She had no speech but stage HD was seen for a dental assessment three visits, a dental clearance was carried
made sounds. Although a wheelchair user, as part of a regular hospital review out. At each visit 3 mg of midazolam
she could transfer to the dental chair. Oral programme. She was a patient at The Royal was carefully titrated against response,
examination was impossible owing to Hospital for neuro-disability in London. resulting in the patient becoming very
facial movements, though a few broken The patient’s family history relaxed with the absence of choreic
down teeth could be seen. Until her illness, was inconclusive since close relatives had movements. Oxygen saturation was
Mrs A had been a regular dental attender died. However, it appears that her father maintained at 95−97% throughout and no
at her general dental practitioner. Mr A and two uncles had a condition suggestive supplemental oxygen was required. She
was his wife’s main carer, although there is of Huntington’s disease. The patient was returned to the ward following a period of
some support from healthcare assistants married and had two adopted children. recovery in the dental surgery.
who came to their house on a daily basis. Shortly following her divorce, she had The provision of intravenous
The different ways of providing severe personality deterioration with sedation for this patient provided an
treatment were discussed with Mr A: paranoid outbursts and profound social alternative management option when
either intravenous sedation (IVS) or change. She was originally diagnosed as general anaesthesia was not appropriate
general anaesthesia. He was keen that his having Alzheimer’s and sectioned under and symptomatic management was the
wife had care under sedation as his own the Mental Health Act. Following the only other care strategy.
experience of sedation was good. Consent provision of further family history, she was
Form 4 (form for adults who are unable to diagnosed as having HD and admitted to
consent to investigation or treatment)8 was the hospital in 1992 Oral care in people with
completed by two dental professionals. The patient’s first hospital Huntington’s disease
Unfortunately, treatment under dental review was carried out on There are no oral manifestations
sedation was unsuccessful: as a result of admission and, at that stage, routine of HD and affected individuals are not
involuntary movements cannulation was dental conservation and scaling could more susceptible to dental diseases when
not achieved and it was impossible to get be completed. Dental examinations compared with the general population.
accurate readings from the pulse oximeter. and treatment with local anaesthesia However, as Huntington’s progresses,
A small amount of Midazolam (10 mg) continued to be provided until 1997 certain features of the condition render
was administered intranasally and this did when her condition deteriorated such patients at a higher than average risk
allow an oral examination to be carried that only examinations and monitoring of caries and periodontal disease and,
out. Clinical examination revealed retained were possible. Two years later, this patient consequently, they present with more
roots in all four quadrants and deposits of experienced pain and swelling from a dental problems.9 Delivery of care becomes
plaque and calculus. In view of the amount lower molar tooth. Oral sedation was used more difficult in the latter stages of the
of treatment required, the difficulties of IVS to help with treatment: 5 mg midazolam disease and there are issues relating to
and because Mrs A was thought to be in given orally 25 minutes prior to the access to dental services, communication
pain, it was decided to carry out treatment appointment. Although some relaxation and consent.
under general anaesthesia. was achieved, this was insufficient to allow
Treatment went well; Mrs A treatment. Following medical consultation,
was admitted on the morning of surgery it was considered unwise to refer for Prevention
and had a bed in a side room to reduce treatment under general anaesthesia but Most dental patients with HD
her stress. Treatment was planned on an rather to manage symptomatically with are able to accept routine dental care
inpatient basis as Mr A was his wife’s sole antibiotics as required. before the onset of symptoms and in the
carer at night. Induction of anaesthesia She continued to be assessed early stages of the disease. From the outset,
was with gaseous isoflurane to avoid regularly and, following an examination at risk families should be educated about
the previous cannulation difficulties. in 2006, she was found to have fairly the condition and its implications for oral
Intubation was carried out with a nasal extensive amounts of oral disease. health and encouraged to seek out regular
endotracheal tube to allow easy access to This included 10 carious teeth in all 4 dental care. The instigation of a rigorous
carry out dental care. Treatment involved quadrants. Medically, she was fairly stable, preventive regime will be invaluable in
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minimizing future dental problems. straight. They often lose balance and any from being cared for by dental teams
Retention of the natural dentition is resultant fall can lead to traumatic dental who understand their condition and are
hindered by a number of factors: or (occasionally) facial injuries. experienced in providing care for this
„ Diet; group of patients. The general practitioner
„ Oral hygiene; has a role to play, particularly in the early
Difficulties in provision of restorative dental
„ Xerostomia; stages of the disease, in careful treatment
care
„ Susceptibility to trauma; planning bearing in mind future difficulties
The two cases presented
„ Difficulties in provision of restorative in providing dental treatment.
here provide examples of the provision
dental care.
of comprehensive dental care
using conscious sedation and general
Diet anaesthetic. These treatment modalities
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