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CASE REPORT
Year : 2014  |  Volume : 32  |  Issue : 2  |  Page : 168-171
 

Dental findings in patients with West syndrome: A report of two cases


Departments of Paedodontics and Preventive Dentistry, University College of Medical Sciences (University of Delhi) & GTB Hospital, New Delhi, India

Date of Web Publication17-Apr-2014

Correspondence Address:
Amit Khatri
Department of Paedodontics and Preventive Dentistry, UCMS (Delhi University) & GTB Hospital, New Delhi - 110 095
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.130988

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   Abstract 

West syndrome a rare, severe form of epilepsy occurs in early infancy. It is characterized by a triad consisting of infantile spasms that occurs in clusters, arrest of psychomotor development and hypsarrhythmia on electroencephalogram. We present here two cases of west syndrome where patients required dental care due to the presence of certain dental findings. Preventive measurements such as controlled diet and proper oral hygiene along with professional dental management are recommended in patients with west syndrome to avoid dental problems.


Keywords: Infantile spasm, psychomotor development, West syndrome


How to cite this article:
Khatri A, Kalra N, Tyagi R, Baweja M, Khandelwal D. Dental findings in patients with West syndrome: A report of two cases. J Indian Soc Pedod Prev Dent 2014;32:168-71

How to cite this URL:
Khatri A, Kalra N, Tyagi R, Baweja M, Khandelwal D. Dental findings in patients with West syndrome: A report of two cases. J Indian Soc Pedod Prev Dent [serial online] 2014 [cited 2019 Oct 18];32:168-71. Available from: http://www.jisppd.com/text.asp?2014/32/2/168/130988



   Introduction Top


W. J West first described the West syndrome in 1841. [1] West syndrome is characterized by a triad of infantile spasm, arrest of psychomotor development and profound paroxysmal electroencephalogram (EEG) abnormalities (hypsarrhythmia), although diagnosis can be even made if one of the three components is missing. [2]

The peak age of onset of disease is between 3 and 7 months commencing usually before 2 year of age. [3] The incidence of west syndrome ranges from 2 to 3.5/10,000 live birth. [3] West syndrome occurs in children from all ethnic groups and boys are affected slightly more often compared with girls (ratio of 60:40). [4],[5],[6]

Consensus statement of West Delphi Group (2004) categorized West syndrome based on etiology of infantile spasm into three subgroups: Symptomatic, idiopathic and cryptogenic. Although the term symptomatic infantile spasm is used to describe cases where the origin of infantile spasm is identifiable such as pre/peri/post-natal cerebral ischemia; brain congenital abnormalities; chromosomal abnormalities; tuberous sclerosis; endocrine/metabolic etc. The idiopathic infantile spasms describe the cases with no identifiable underlying cause or neurological sign or symptoms. The term cryptogenic is used to describe cases where there is suspicion of being symptomatic due to preceding developmental delay or neurological symptoms, but with no identifiable underlying structural or biochemical cause. [7]

A literature search (PubMed/Medline/Medknow/Google scholar) retrieved only one paper addressing dental findings and dental care management in West syndrome patient. [8] In order to increase awareness and preventive dental management regarding the dental problems in patients with West syndrome, we present here two cases of West syndrome having certain orofacial characteristics which required professional dental care.


   Case Reports Top


Case 1

This was a first case of a 3.5-year-old male child with a chief complaint of pus discharge from upper anterior teeth child presented to Department of Paedodontics and Preventive Dentistry, UCMS and GTB Hospital Delhi. He was the only child of healthy consanguineous parents. The child was born by an uncomplicated normal vaginal delivery at full term and had a birth weight of 2.4 kg. The family history contained no report of similar cases. Patient had difficulty in breast feeding initially, at the age of 4 month the child had recurrent episode of a convulsive seizure. A diagnosis of West syndrome was based on EEG finding of hypsarrhythmia and infantile spasms (brief synchronous flexor or extensor spasm of head, trunk and limb), which occurs in cluster. Among the typical manifestation of west syndrome, neuropsychomotor delay is the most evident characteristic [Figure 1a], especially atrophy of upper and lower limbs [Figure 1]b and c]. Review of the child medical history revealed that he had a delay in neuropsychomotor development, which started at age of 8-9 month, even at age of 3.5 year he could not hold his neck or sit. Furthermore, the patient had poor concentration, not able to communicate and obey commands and displayed difficulties in understanding, focusing, restlessness and poor motor skills. The patient regularly takes valparin syrup (200 mg/5 ml), 3.5 ml BD Sanofi Synthelabo (India) Ltd., clonozapam 0.5 mg ½ tablet BD alcona tablet (India) Ltd., topiramate 25 mg ½-1 tablet BD topex tablet India Pvt., Ltd.

Intraoral examination revealed generalized tooth wear, gingival inflammation, mildly high palatal vault and extensive carious lesion on 51, 52, 61 and 62 [Figure 2]. Dental treatment consisted of extraction of 51, 52, 61 and 62. Glass ionomer restoration in 74 and 75 followed by Pit and fissure restoration with respect to 54, 55, 64, 84 and 85 under general anesthesia.

After completion of treatment patient was shifted to post-operative care, from where patient was shifted to dental ward for observation. Patient had 5-6 episode of seizure for 2-5 s after that the patient was shifted to pediatric emergency for monitoring of vital signs and seizure control. His stay was uneventful and stable. After discharge from hospital follow-up visits were scheduled every 3 month to monitor oral hygiene, possible onset of new lesion and the periodontal conditions.

Case 2

This was a second case report of a boy aged 4.5 years who reported to Department of Paedodontics and Preventive Dentistry, UCMS and GTB Hospital Delhi, with the chief complaint of multiple decayed teeth since few months. Review of medical history revealed that he was born to non-consanguineous parents, delivered after an uneventful full-term pregnancy at home. The family history contained no report of similar cases. He did not cry at birth and developed jaundice on the 3 rd day and displayed abnormal movements, intermittent jerks of head and recurrent spasms multiple times in a day. According to reports from the institution at which the patient receives therapy, the patient was diagnosed as the case of West syndrome with spastic quadriplegic cerebral palsy and microcephaly. The diagnosis of West syndrome was based on profound paroxysmal EEG abnormalities of hypsarrhythmia and infantile spasms. The etiology was found to be perinatal traumatic ischemic insult. When the patient first reported to dental department, he was unable to communicate verbally. In addition, the patient displayed difficulties in understanding and focusing, restlessness and very poor motor skills. According to medical reports, the child presented a delay of neurologic, psychological, motor development difficulties and atrophy of upper and lower limbs [Figure 3]a. Radiograph reveals deformity in upper limb [Figure 3]b. He was being treated by a pediatrician, speech therapist and physiotherapist and patient regularly takes syrup valparin (200 mg/5 ml) 5-6 ml, tablet frisium (5 mg) OD, tablet pyridoxine 400 mg TDS, tablet biotin 10 mg OD. The patient is currently controlled and has had no seizures in the last 9-10 months.
Figure 1:

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Figure 2: Intraoral examination shows multiple advanced carious lesion on 52, 51, 61, 62, slightly hypomineralized area on the buccal surface of a 64 infl ammation and high palatal vault

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Figure 3:

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An intraoral clinical examination found that 55, 54, 53, 52, 51, 61, 62, 63, 64 and 65 had multiples surface carious lesion, generalized tooth wear and mildly high palatal vault [Figure 4]. Generalized gingival inflammation was also observed and most likely due to poor oral hygiene and plaque accumulation. Thorough oral prophylaxis was performed and rehabilitation of all carious lesions was done. Topical fluoride varnish was applied and dietary modifications were made. Mother was instructed about caries and periodontal disease prevention, gave her diet counseling to reduce child sugar intake and trained her to maintain proper oral hygiene. Emphasis was given for a recall visit after every 3 months.
Figure 4: Intraoral examination shows multi surface carious lesion on 54, 53, 52, 51, 61, 62, 63, 64 generalized tooth wear, gingival infl ammation and high palatal vault

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   Discussion Top


Dental management of patients with special health care needs present unique challenges due to limited communication skill, severe intellectual deficit and inability to obey commands and follow oral hygiene instructions.

West syndrome is a severe form of epilepsy specific to early childhood, feature of this syndrome include axial spasm in cluster, characteristics and pathogonomics EEG abnormality called hypsarrhythmia and delayed psychomotor development. [9]

Patient (Case 1) had the entire characteristic feature and was diagnosed as cryptogenic form of west syndrome due to unknown etiology, normal pregnancy and birth and the child had normal development prior to onset of infantile spasm, whereas other patient (Case 2) was diagnosed as symptomatic form of west syndrome with identifiable underlying cause.

In a study done by Regis et al. [8] found in his study that major clinical feature of west syndrome were generalized tooth wear, gingival enlargement and multiple white spot lesions, even we have found similar associated finding in both patients. White spot lesion in these patients, most likely due to poor oral hygiene with plaque accumulation associated with sugar containing medications and fermentable carbohydrate rich diet.

Bruxism has been frequently reported in patients with special care need and neuromuscular disorder. Bruxism was even diagnosed in both of our patients based on the presence of generalized tooth wear and mother's report of clenching and grinding of teeth. [10]

Children being treated with antiepileptic drug are more susceptible to developing periodontal problem. [11] It may be speculated in patient mild generalized gingival hyperplasia was associated with long term use of antiepileptic, most common side effect of valproate is gingival bleeding, petechie, decreased platelet aggregate, but we have not noticed any associated side effect. [12]

Physical and behavioral characteristic of children with west syndrome, lack of motor coordination, neurological developmental delay and cognitive and communication deficits, inadequate oral hygiene presents an additional challenge to the clinician.

The high caries risk of patient with west syndrome is a result of their poor oral hygiene due to lack of motor coordination and cognitive impairment. The patient and patient's parent should be made aware of importance of preventive measure such as controlled and non-cariogenic diet, regular use of sugar free medication, fluoride mouth rinse and fluoride dentifrices. Professional care such as oral prophylaxis, pit and fissure sealant must be provided. Topical application of fluoride varnish can usually be used safely to prevent and treat enamel whit spot lesion.

Riikonen [4] study showed that children affected with west syndrome do have a normal lifespan although they often have profound mental and physical deficits. Therefore, patients with west syndrome need more dental care along with medical care to avoid dental problems with emphasis on preventive care.

 
   References Top

1.Wong V. West syndrome - The University of Hong Kong experience (1970-2000). Brain Dev 2001;23:609-15.  Back to cited text no. 1
[PUBMED]    
2.Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1989;30:389-99.  Back to cited text no. 2
    
3.Hrachovy RA, Frost JD. Severe encephalopathic epilepsy in infants: Infantile spasms (West syndrome). In: Pollock JM, Bourgeois BF, Dodson WE, Nordli DR Jr, Sankar R, editors. Pediatric Epilepsy: Diagnosis and Therapy. New York, NY: Demos Medical Publishing; 2008.p.249-68.  Back to cited text no. 3
    
4.Riikonen R. Epidemiological data of West syndrome in Finland. Brain Dev 2001;23:539-41.  Back to cited text no. 4
[PUBMED]    
5.Trevathan E, Murphy CC, Yeargin-Allsopp M. The descriptive epidemiology of infantile spasms among Atlanta children. Epilepsia 1999;40:748-51.  Back to cited text no. 5
    
6.Lúthvígsson P, Olafsson E, Sigurthardóttir S, Hauser WA. Epidemiologic features of infantile spasms in Iceland. Epilepsia 1994;35:802-5.  Back to cited text no. 6
    
7.Lux AL, Osborne JP. A proposal for case definitions and outcome measures in studies of infantile spasms and West syndrome: Consensus statement of the West Delphi group. Epilepsia 2004;45:1416-28.  Back to cited text no. 7
    
8.Regis RR, Rocha CT, Torres CP, Queiroz IF, de Queiroz AM. Oral findings and dental treatment in a child with West syndrome. Spec Care Dentist 2009;29:259-63.  Back to cited text no. 8
    
9.Appleton RE. West syndrome: Long-term prognosis and social aspects. Brain Dev 2001;23:688-91.  Back to cited text no. 9
[PUBMED]    
10.Monroy PG, da Fonseca MA. The use of botulinum toxin-a in the treatment of severe bruxism in a patient with autism: A case report. Spec Care Dentist 2006;26:37-9.  Back to cited text no. 10
    
11.Angelopoulos AP. A clinicopathological review. Diphenylhydantoin gingival hyperplasia: 2. Aetiology, pathogenesis, differential diagnosis and treatment. Dent J 1975;41:275-7, 283.  Back to cited text no. 11
    
12.Li LM, Russo M, O'Donoghue MF, Duncan JS, Sander JW. Allergic skin rash with lamotrigine and concomitant valproate therapy: Evidence for an increased risk. Arq Neuropsiquiatr 1996;54:47-9.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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