OraL MaNIFEstatIONs OF cErEbraL PaLsY

CASE REPORTS
ORAL MANIFESTATIONS OF CEREBRAL PALSY - THE
SPASTIC TETRAPARESIS TYPE: A LITERATURE REVIEW
AND CLINICAL CASES
Anca Maria Raducanu, Irina Cristea, Victor I. Feraru
REZUMAT
Introducere: Paralizia cerebral\ este un termen care descrie o constela]ie de deregl\ri neuro-musculare, necontagioase [i neevolutive, provocate de lezarea
creierului n stadiile precoce ale dezvolt\rii sale. Tetrapareza spastic\ reprezint\ forma cea mai grav\ a paraliziei cerebrale spastice. Copiii cu paralizie cerebral\
sunt mai susceptibili la unele boli buco-dentare fa]\ de copiii normali. Scop: Sistematizarea informa]iilor din literatura de specialitate cu privire la manifest\rile
generale [i orale ale paraliziei cerebrale - forma cu tetraparez\, urmat\ de prezentarea a dou\ cazuri. Metod\: Sunt prezentate dou\ cazuri clinice cu tetraparez\
spastic\, ale c\ror semne [i simptome generale [i orale se nscriu n tabloul clasic al paraliziei cerebrale spastice, descris n literatura de specialitate. Primul caz
este al unei feti]e de 6 ani, cu tetraparez\ spastic\ provocat\ de hipoxia la na[tere, cazul al doilea fiind o feti]\ de 11 ani, cu acela[i diagnostic, dar la care nu s-au
putut depista factorii etiologici ai bolii. Pentru prezentarea acestor cazuri au fost folosite date din: anamnez\, copia fi[ei generale de s\n\tate ntocmit\ de medicul
curant neurolog, date din examenul clinic precum [i rezultatele investiga]iilor complementare generale [i orale efectuate. Rezultate [i concluzii: Conduita
terapeutic\ aplicat\ celor dou\ paciente atrage aten]ia, pe de o parte, asupra dificult\]ilor ntmpinate iar, pe de alt\ parte, asupra faptului c\ aceste greut\]i pot fi
dep\[ite chiar [i n condi]iile unui cabinet stomatologic obi[nuit care nu este dotat pentru practicarea anesteziei generale sau a inhalosed\rii.
Cuvinte cheie: tetraparez\ spastic\, paralizie cerebral\, spasticitate [i tonicitate muscular\
abstract
Introduction: The cerebral palsy (CP) is a term describing a constellation of neuro-muscular disturbances, non-contagious and non-progressive, that are
determined by brain damage in its early stages of development. Spastic tetraparesis represents the most severe form of spastic cerebral palsy. Compared to
normal children, those with cerebral palsy are more susceptible to oro-dental diseases such as caries, gingivitis, malocclusion, bruxism, deglutition, mastication
and speaking disorders, etc. Aim: To review the literature regarding the general and oral manifestations of cerebral palsy with tetraparesis and to present two
clinical cases. Method: Two clinical cases with spastic tetraparesis, of different severity and etiology are presented. Discussions and conclusions: Both
cases with spastic tetraparesis present typical CP general and oral features. The most important therapeutic goals were the control of the oral bacterial plaque with
a consecutive reduction of dental disease and the change of the negative behavior pattern in both cases. Dental treatment could be performed without sedation
or general anesthesia in both cases. Physical and mechanical restraint may me necessary at times, when serious problems in the safe maneuvering of the
instruments appear. Dental treatment in patients with CP can be performed, in certain situations, even in a dental office with average conditions and equipment.
Key Words: spastic tetraparesis, cerebral palsy, muscular spasticity and tonicity
INTRODUCTION
The cerebral palsy (CP) is a term that describes
a constellation of neuro-muscular non-contagious
and non-evolutionary disturbances, caused by a brain
lesion in the early stages of its development (in the
first 5 years of life). Spastic tetraparesis represents the
most severe form of spastic cerebral palsy. The CP
produces handicapping changes of movement and
posture.1-8
Department of Pediatric Dentistry, Faculty of Dental Medicine, Carol Davila
University of Medicine and Pharmacy, Bucharest
Correspondence to:
Anca Maria Raducanu, 12 Ionel Perlea Str., Bucuresti, Tel. +40-21-314.20.80
Email: [email protected]
Received for publication: Feb. 22, 2008. Revised: May 19, 2008.
The prevalence of CP ranges from 1 to 4 children
/1000 alive new-born.3,6,8 The etiology of CP is
multifactorial and includes prenatal causes (genetic
diseases, embryonic anomalies), perinatal (hypoxia, Rh
incompatibility, premature birth, underweight at birth,
etc.) and postnatal (infections, trauma, etc.). In 30% of
the cases, the risk factors can be traced. The frequency
is 10 times higher in premature children and 25 times
higher in small-for-birth children.1,2,6,8
The CP classification includes four types: spastic,
dyskinetic, hypotonic, and mixed, the spastic type
being the most common one. Spastic tetraparesis
represents the most severe form of spastic cerebral
palsy in which the motor disturbance affects the whole
body: face, trunk, legs, arms, which usually puts the
patient in a wheelchair.4,9
General manifestations of the CP are diverse,
ranging from minimal limitations of the movement,
that don’t call for treatment, to severe alteration of
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Anca Maria Raducanu et al 91
the motor function, necessitating complex, long term,
multidisciplinary treatment and permanent home care.
Depending on which areas of the brain have been
damaged, one or more of the following symptoms
may occur: muscular rigidity or spasms, involuntary
movements, difficulties of the “gross motor skills”
(walking, running), difficulties of the “fine motor
skills” (writing or doing up buttons, brushing the teeth
ertc.). These may cause associated problems such as
difficulties in feeding, poor bladder and bowel control,
breathing problems etc.1-3,5,6,8
The patients with cerebral dysfunction may
also display mental retardation, epilepsy, sensorial
deficiencies (sight and hearing impairment), persistent
primitive reflexes, attention-, memory-, learningand emotional problems, language and speaking
disturbances.8
ORAL MANIFESTATIONS
Children with CP display the same oral pathology
as healthy persons. However, they present a higher
susceptibility to several oro-dental diseases (dental
caries, periodontal disease, dental trauma, malocclusion,
bruxism, temporomandibular joint disorders, enamel
hypoplasia, abnormal oral habits - tongue thrust,
mouth breathing, drooling etc.) due to the abnormal
neuromuscular coordination of the tongue, lips, and
cheeks and to the low level and the reduced quality of
the oral care.1,7
The treatment of CP is complex and
multidisciplinary, consisting in general (kinetotherapy,
surgical interventions, myorelaxants medication) and
oral treatment (behavior management, prophylactic,
curative).
- Both patients presented tensed position of the
head, spastic facial muscles, opened mouth and oral
breathing. (Fig. 1)
Figure 1. Case 1. Opened mouth, protruded tongue position.
The oral features are:
- Multiple dental caries (complicated and
uncomplicated), gingivitis, tonic and propulsive
tongue, dental malpositions, protrusion, Angle II/1
malocclusion, atypical deglutition, difficult mastication
and swallowing, poor oral hygiene (high caries risk);
(Figs. 2-4)
CASE REPORTS
Two cases that present typical oral and general
features for the spastic tetraparesis with uncontrolled
extrapyramidal movements are described. The two
cases present many general and oral similarities but
also some differences.
The general common features are:
- Neither of the patients displays hearing problems
or epilepsy;
- The motor functions and the posture are
deteriorated at both patients but in different degrees,
therefore they are unable to perform simple current
activities, like washing, dressing, hair doing, feeding,
teeth brushing etc., and necessitate permanent
caretaking;
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Figure 2. Case 2. Radiographic aspect of periapical infections of 3.1, 4.1.
The differences are mainly related to the etiology,
to the gravity of the motor and postural dysfunctions,
to the severity of the general and oral CP-related
manifestations and to the general treatment.
Figure 3. Case 1. Malocclusion, extensive caries on 5.1, 6.1.
Figure 4. Case 1. Opened mouth, Angle class II/1 malocclusion.
1. Patient I.N., 6 years and 4 months old,
from Bucharest presenting at our Pediatric Dentistry
Department at the initiative of her mother who has
noticed the progressive destruction of several teeth.
- Etiology: perinatal causes (premature birth, low
birth weight, natal hypoxia) and many risk factors.
During pregnancy her mother had vaginal bleeding
from 2.5 months up to 4 months, abnormal insertion
of the placenta, risk of placenta detachment and
abortion impendence. The birth was premature (at
31 weeks), in pelvian presentation, and the labor was
long. At birth the girl had 2000 g and the Apgar score
was initially 1, reaching 3 after 3 minutes. The neonatal
jaundice was persistent, and associated with the fact
that the girl did not gain weight and presented with
nosocomial Klebsiella infection, blood transfusions
were necessary.
- The patient presents no mental retardation;
- The motor functions and the posture are
deteriorated; (Fig. 5)
Figure 5. Case 1. Affected posture, tensed hands, flexed knees, inward
rotated feet.
- Delay in development of language and speaking
functions, improper pronunciation of certain
phonemes;
- Converging strabismus and hypermetropia, nasal
polyposis; (Fig. 6)
Figure 6. Case 1. Convergent strabismus.
- Frankl rating 2 at first visit and Frankl rating 3 after
behavior management (“Tell-Show-Do” technique); (Fig. 7)
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Anca Maria Raducanu et al 93
current medication consists of neurotrophics and
myorelaxants.
On oral examination, following data could be
collected:
- Dental history: primary dentition and 3.1, 4.1,
3.6, 4.6 in eruption; the dental age fits the biological
age; (Figs. 8, 9)
- Localized eruption gingivitis; (Fig. 10)
- Enamel’s non-penetrating coronary fracture of
the distal-incisal angle of 5.2;
- The masticatory function is not affected (normal
alimentation).
Figure 8. Case 1. View of the maxillary arch: primary dentition, multiple
caries, deep palate.
Figure 9. Case 1. View of the mandibular arch: mixed dentition, first molar
in eruption, multiple caries, localized gingivitis.
Figure 7. Case 1. The patient soon after being set on the dental chair:
relaxed attitude, affected position.
- The patient has benefited of sustained general
rehabilitation treatment: kinetotherapy, surgical
treatment in order to elongate some tendons of the
lower members, home educational therapy, logopaedic
and ophthalmologic treatment. In time, the medication
has included different myorelaxants, which did not
prove efficient, as well as botulinic toxin administration
two years ago with a temporary good effect. The
general treatment has led to the improvement of
the walking, posture and language. Yet, the affected
posture and a difficult walking can be noticed. The
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TMJ 2008, Vol. 58, No. 1 - 2 94
Figure 10. Case 1. Lateral view of the mandibular arch: 4.6 erupting,
localized gingivitis, 8.5 amalgam restoration.
2. Patient I.R., 11 years and 4 moths old, from
Bucharest, presented at our Department at the initiative
of her mother who has noticed the progressive
destruction of several teeth.
- Etiology: spastic tetraparesis of unknown origin;
- The computer tomography performed around
the age of eight months, when the mother, after
noticing the motor deficit and the severe motive
aphasia, presented at the doctor, revealed the frontal
cortical atrophy;
- The patient presents slight mental retardation
(attention disorder, distractibility, poor attention,
poor memory, verbal stereotypy, emotional lability,
disinhibition, impulsive behavior);
- The motor functions and the posture are severely
deteriorated: currently the patient is not able to walk,
and moves in a wheelchair and necessitates permanent
care for all the daily activities. (Fig. 11) At the same
time, her hands are tensed, cannot grab objects or do
current self-care activities such as washing, dressing,
hair-doing, feeding, etc.
- Partial bilateral retina atrophy, iatrogenic left eye
amaurosis; (Fig. 12)
- Speaking severely disturbed (disturbance in the
predictive function of speech which takes part in
structuring sentences, altered pronunciation of only
certain monosyllabic words: the girl pronounces
only the vowels, by replacing the consonants with
other vowels - for instance instead of “no” she says
“yo”);
- The treatment with neurotrophics and myorelaxants did not produce the expected results and it was
interrupted. Surgical treatment and botulinic anatoxin
treatment were not indicated in this case. Currently,
the recovery treatment consists only in kinetotherapy,
with satisfying results. Although indicated, home
educational therapy could not be undertaken, as
persons specialized in this field were not available and
no student group has been set up.
- Frankl rating 1 at first visit; (Fig. 13) Frankl rating
2, reached 3 after behavior management (“Tell-ShowDo” technique); (Figs. 14, 15) the physical restraint is
necessary in certain moments of the treatment, as the
extrapyramidal component of the disease may cause
unexpected and uncontrolled movements of the head,
arms and legs. (Fig. 16)
On oral examination, following data could be
collected:
- Permanent dentition, except the third molar; the
dental age fits the biological one; (Fig. 16)
- Enamel hypoplasia / hypomineralisation;
- Generalized chronic gingivitis.
Figure 11. Case 2. Severe postural and motor deficit, impossibility to walk,
tensed hands, flexed knees, rotated feet.
Figure 12. Case 2. Partial bilateral retina atrophy, iatrogenic right eye
amaurosis.
In both cases, the following therapeutic procedures
have been performed:
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Anca Maria Raducanu et al 95
Figure 13. Case 2. The girl soon after wheelchair transfer, extremely negative
behavior (Frankl 1), dental team performing the "tell-show-do" technique.
Figure 15. Case 2. Final Frankl rating 3.
Figure 14. Case 2. The increase of the Frankl rating from 1 to 2.
1. Behavior management techniques: “tell-showdo”, positive reinforcement, voice control, repetition
of the commands and requests; taking into account
the emotional context and the cognitive disability.
Thus, the increase of Frankl rating: from 1 to 3 in the
first case and from 2 to 3 in the second case.
2. Prophylactic treatment: dietary counseling, oral
hygiene correction and control, topic fluoridations,
fissures sealing of permanent molars, periodic
dental recall appointments at every three months,
use of mechanical toothbrushes, modified handle
toothbrushes (Case 1), special teeth brushing positions
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Figure 16. Case 2. Open mouth lateral view, one day after endodontic
drainage and before the root canal treatment: the physical restraint is
performed by 4-6 hands: the father is holding the patient by immobilizing
the head and the arms, while the mother holds the legs and the metallic
mouth prop, which contains the involuntary movements of the head.
by the attendant (Case 2), active co-participation in oral
hygiene control of both the parent and the patient,
use of oral anti-microbial agents, topical and systemic
fluorides. (Figs. 17, 18)
DISCUSSIONS AND CONCLUSIONS
Figure 17. Modified tooth brushes.
Both cases of spastic tetraparesis with different
severity present the typical CP general and oral
features. Although CP is a non-progressive condition,
its effects may change over time depending on the
general treatment (e.g., improvement in the first case
and worsening in the second one). The control of
the oral bacterial plaque with a subsequent reduction
of the dental caries and the change of the negative
behavior pattern are the most important therapeutic
goals. The curative dental treatment may be performed
without sedation or general anesthesia. Physical and
mechanical restraint may be necessary at times, when
serious problems in the safe maneuvering of the
instruments appear.
Dental treatment in patients with CP can be
performed, in certain situations, even in a dental office
with medium conditions and appliances.
REFERENCES
Figure 18. Modified teeth brushing position.
3. Curative treatment: there was no need of
sedation or general anesthesia; the physical restraint was
necessary at times in order to reduce the effects of the
uncontrolled movements or of the negative attitude.
The treatment was performed in short sessions,
the appointments were planned early in the day,
comfortable patient’s position in order not to trigger
primitive reflexes, with stable midline position of the
head, the legs allowed to bend, relaxed atmosphere,
modified atypical operator’s position when needed
in stand-up dentistry, high volume aspiration and 6handed dentistry with control of the extrapyramidal
movements of the patient.
The dental treatment in both cases was the same
as for healthy patients: endodontic therapy when
needed, amalgam direct restorations on molars and
glass ionomer direct restorations on incisors.
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Anca Maria Raducanu et al 97