Volume 6, Issue 9, September – 2021 International Journal of Innovative Science and Research Technology

ISSN No:-2456-2165

Functional Rehabilitation of Tooth in a Patient

Affected with Spinocerebellar Ataxia
1.
Dr. Drishti, Senior Resident, Department of Pedodontics, UCMS, Delhi, India
2.
Dr. Krishna P Biswas, Assistant Professor, Department of Conservative Dentistry and Endodontics, ESIC Medical College and
Hospital, Patna, India
3.
Dr. Navin Mishra, Associate Professor, Department of Conservative Dentistry and Endodontics, IGIMS, Sheikpura, Patna, India
4.
Dr. Priyankar Singh, Assistant Professor, Department of Oral and Maxillofacial surgery, IGIMS, Sheikpura, Patna, India
5.
Dr. Isha Narang, Deputy Civil Surgeon, HCMS, Haryana, India
6.
Dr. Jaspreet Kaur Deo, Senior Resident, Department of Oral and Maxillofacial surgery, Lady Hardinge Medical College, New
Delhi, India
Corresponding Author- Dr Krishna P Biswas, Assistant Professor, Department of Conservative Dentistry and Endodontics,
ESIC Medical College and Hospital, Patna, India

Abstract:- In the current study we have tried to explain This expansion may increase when transmitted from one
how emphasis should be put on healthcare of medically generation to next generation. 2
compromised children since childhood so that positive
attitude can be instilled in these children and their Gene testing may be a powerful tool for diagnosis and
parents towards oral health care so that dental health is prediction of spinocerebellar ataxias. 3The clinical features
not neglected and becomes an integral part of essential of spinocerebellar ataxia may include significant central
medical care. This article gives an insight about the nervous system involvement that extends beyond the
spinocerebellar ataxia and the treatment modality to be cerebellum to the brainstem (medulla and pons) and spinal
given. cord. Symptoms of brainstem motor neuron loss may
include temporalis muscle atrophy, tongue atrophy, facial
Keywords:- Spinocerebellar Ataxia, Functional weakness, and fasciculations. Spasticity and hypereflexia
Rehabilitation, Crown. may be seen in upper motor neuron involvement. Sensory
and motor problem may occur in peripheral nerve
I. INTRODUCTION involvement. Dystonia or bradykinesia may be seen in basal
ganglion involvement. Many of the patients may be bound
Ataxia also known as loss of limb co-ordination affects to a wheelchair prone state as they progress in age.
particularly gait and causes problems in gross and fine Nystagmus may occur in pure cerebellar ataxia’s without
motor control. It may be acquired or genetic in nature. The extracerebellar involvement. 4,5
timing of onset and family history may help in
differentiation between an acquired or genetic defect. The pathogenesis of many neurodegenerative disease
Spinocerebellar ataxia are autosomal dominant progressive occurrence is poly Q encoding CAG repeats. A current
disorders in which degeneration of cerebellum slowly prevailing view is suggestive that a toxic action may occur
occurs, often associated with degenerative changes in at protein level 2,6,7. The oral aspects and management of
brainstem, parts of the central nervous system and at times such cases has been infrequently spoken of hence this case
peripheral nervous system as well.1There are at least 27 report aims at presenting the aspects that were kept in mind
known variants of spinocerebellar ataxia which continue to while treating the case. This case report represents the dental
grow. management by functional rehabilitation of a tooth in a case
affected with spinocerebellar ataxia.
There are 3 predominant genetic categories observed
in spinocerebellar ataxia: Expanded CAG/ poly Q ataxias, II. CASE REPORT
ataxia caused due to conventional mutations (missense,
insertion, deletion, duplication) and lastly non protein An 8-year-old patient reported to the Department of
coding repeat expansion ataxias.2Many SCA present with Dentistry, as a referral from Department of Paediatrics with
extensive cerebellar atrophy with involvement of all regions a chief complaint of severe pain in upper left back tooth
of cerebellum, including molecular, granular cell layer, region in the past 2 days. The patient had reported with a
purkunje cells and deep cerebellar nuclei. SCA’s are often medical history of unstable gait, frequent falls, requirement
differentiated on the basis of their extracerebellar brain of assistance in carrying out activities of daily living like
involvement. 2SCA’s may show a varied phenotype and bathing and dressing, no delay in milestones of development
dynamic mutations which expand to change size and thus and no history of seizures. The child’s IQ assessment done
larger expansions may show an earlier onset of disease using Binet-Kamath test revealed the child to have an IQ of
wherein small expansion may show a later onset of disease. 84 which falls into an IQ category of below average. No
relevant family history was reported in pedigree analysis. In

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Volume 6, Issue 9, September – 2021 International Journal of Innovative Science and Research Technology
ISSN No:-2456-2165
drug history the child had been consuming Cap Evion 1 interim restorative material (IRM). In his next visit, the
capsule once daily since the past 1 month. The diet history patient was relatively free of symptoms, so working length
of patient revealed that the patient was mainly consuming was taken with the help of Propex Pixi apex locator
divided proportion of fruits, pulses, cereals, milk and eggs in (dentsply Sirona and Maillefer), and canal patency was
his diet in three proportional meals with less in between established with size 10 K file and then 15 K file. Rotary
meal snacking. The child had infrequent exposure to sugar, preparation of canal space was done with Kedo-S rotary file
aerated beverages, toffee and chocolates. The diet mainly system (Reeganz dental care Pvt Ltd. India) up to size D1
comprised on non-sticky fibre rich food items and less sugar for mesiobuccal and distobuccal canal due to its narrow
rich food items. anatomy, and size U1 for palatal canal at a rotational speed
of 300 rpm per minute and torque of 2.4 Ncm. After
The physical examination of child revealed that the adequate preparation of canals, obturation was done with
patient had an unstable gait, difficulty in walking requiring Metapex (Meta Biomed)(Figure 2). The chamber was then
often parental assistance and easy fatiguability. The child given a closed dressing with Cavit- G(3M ESPE) temporary
had difficulty in being seated on the dental chair by himself filling. In the third visit the tooth was asymptomatic hence a
and needed assistance. The child was slow in speech and permanent post endodontic restoration was done with
spoke with complete yet very short and slow responses and packable posterior composite (Ivoclar Vivadent Tetric N
was slow in compliance of any commands. The child Ceram)(Figure 3). In the next visit, crown cutting was
however was very co-operative and adaptable. No other performed for the patient and stainless steel crown (3M
delayed milestones of development were noted. ESPE) was luted on the tooth 65 with luting GIC(3M ESPE)
for its functional rehabilitation(Figure 4). The patient came
On intraoral examination of child, it was observed that for follow up after 1 week and was relatively free of
the child had no delay in eruption pattern, development of symptoms and was able to chew from the involved side
dentition or shedding pattern of teeth. The patient had an without any discomfort. Fones technique of brushing was
overall good oral hygiene with good gingival health. The demonstrated to the child and parents and possibly the use of
soft tissue findings of the child including tongue, hard newer battery-operated tooth brush was instructed so that the
palate, soft palate, floor of mouth, anterior and posterior child can continue to maintain a good oral hygiene despite
faucial pillars were essentially normal and healthy in his decreased manual dexterity. However, a long follow up
appearance. The intraoral hard tissue findings revealed that could not be scheduled as the parents refused for any follow
the child had mixed dentition with the eruption pattern of up visits as they wanted to focus on child’s medical
permanent teeth falling in line with the age of the patient. treatment due to financial burden of treatment and the
Only one carious tooth, left deciduous upper second molar pressure of parenting a special child.
(65) was reported. Other teeth were free of caries. The tooth
had deep occlusal decay with symptoms of an irreversible III. DISCUSSION
pulpal involvement, as the child had severe pain, sudden in
onset lasting for longer duration, sharp shooting in nature The present case represents dental management of a
which was exacerbated with hot and cold fluids and food child affected with Spinocerebellar ataxia and presents an
and relieved by medication, ibuprofen(200mg). overview of systemic manifestations seen in a child affected
with it and how they might affect treatment strategies.
Treatment plan was made and explained to the parent. Children are less often affected with spinocerebellar
Physician consent for carrying out necessary dental ataxia as its onset occurs usually after 18 years of age
intervention was taken. Parental consent was as well sought. mostly except for in certain cases. The symptoms gradually
The child was made comfortable in the dental setup, chair worsen over the period of years. 8
was adjusted so that the child could easily settle in, and was
explained about the procedure planned to be carried out. Tell In the present case when the child made his first dental
show-do technique and euphemisms were used to explain visit it was observed that the child had difficulty in
the armamentarium and procedure to make child maintaining balance while walking, needed assistance while
comfortable with the clinical environment. Child showed a walking, was easily fatigued and had difficulty in being
very co-operative behaviour towards beginning of the seated on the dental chair. He needed support in the form of
procedure and to the commands that were being given to towel wrappings around legs and arms to keep him upright
him. A intraoral radiograph was taken which revealed while undergoing the procedure. Children with Spino-
proximal caries involving enamel, dentin and progression cerebellar ataxia often are affected by progressive in-
towards pulp chamber (Figure 1). Emergency access coordination of walking, poor- coordination of hand
opening was planned in the first visit keeping in mind that movements, speech and eye movements. 8
the child was compliant enough for undergoing such
procedure in his first visit and the intensity of pain that he The child could comprehend all commands being
had reported with. Emergency access opening was carried given to him but was slow pertaining to his response levels.
out in the first visit, pulp chamber and canals was debrided His IQ assessment done using Binet-Kamath test revealed
with copious irrigation with sodium hypochlorite (5.25%), that he had an IQ level of 84 which falls into an IQ category
chlorhexidine, EDTA (17%) and saline keeping good of below average. Dysarthria or speech impairment may
suction to prevent the child from swallowing the irrigant present as a finding in cases affected with spinocerebellar
solutions. The child was given a closed dressing with ataxia. 8

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Volume 6, Issue 9, September – 2021 International Journal of Innovative Science and Research Technology
ISSN No:-2456-2165
However, rubber dam could not be used in the present traditional tooth brushes. Also, they are cost effective as
case as the child had easy fatiguability and difficulty with they are not as expensive as electric tooth brushes.
keeping the mouth open for a prolonged period. Children
with Spino-cerebellar ataxia may also present with The child however, did not report for his subsequent
symptoms of brainstem motor neuron loss including follow up visits as the parents were already going through
temporalis muscle atrophy, tongue atrophy, facial weakness, tumultuous pressure of handling a special child, his medical
and fasciculations. 4,5 visits and slow progress in school due to intellectual deficit.
Their refusal to report for subsequent visits also puts an
After emergency access opening, instrumentation of eminent light on the amount of pressure these parents go
canal was done with Kedo- S rotary file system. This newly through while caring for a special child.
introduced paediatric rotary file system is the first file
system introduced for primary teeth. It comprises of three The dental management of children with special health
Ni-Ti rotary files with a total length of 16 mm and working care needs is often ignored due to such reasons and thereby
length of 12mm. the three files are D1, E1 and U1. D1 has a should be given a priority so that these children can maintain
tip diameter of 0.25 mm and can be used in narrow canals of good oral health and pain free life. A caries free mouth and
molars while E1 has a wider tip diameter of 0.30 mm and well-maintained oral hygiene may aid them in achieving
can be used in wider molar canals. Rotary instrumentation their nutritional requirements thus developmental milestones
was preferred in this case to decrease instrumentation time in a better manner. Also, the help of general anaesthesia
due to easy fatiguability of the child and improve the quality may be taken in highly uncooperative cases.
of obturation. Jeevanandan and Govindaraju evaluated
instrumentation time and quality of obturation between REFERENCES
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as they may aid in increasing the efficacy of cleaning and
reduce the manual dexterity required while utilizing

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Volume 6, Issue 9, September – 2021 International Journal of Innovative Science and Research Technology
ISSN No:-2456-2165
FIGURES

Figure 1:Pretreatment radiograph of tooth 65 showing

carious progression involving enamel, dentin and pulp.

Figure 2: Post treatment radiograph post completion of

pulpectomy in tooth 65.

Figure 3: Intra-oral photograph after completion of post

endodontic restoration.

Figure 4: functional rehabilitation of tooth 65 with stainless

steel crown.

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