CASE REPORT |
https://doi.org/10.5005/jp-journals-10077-3278 |
Dental Considerations and Treatment Approaches in West Syndrome—Infantile Spasms: A Case Report
1–7Department of Pedodontics and Preventive Dentistry, University College of Medical Sciences, Delhi, India
Corresponding Author: Shruti V Kulkarni, Department of Pedodontics and Preventive Dentistry, University College of Medical Sciences, Delhi, India, Phone: +91 8390705180, e-mail: shrutikulkarni894@gmail.com
Received: 29 June 2023; Accepted: 25 July 2023; Published on: 30 December 2023
ABSTRACT
Introduction: West syndrome (WS) comprises a classic triad of infantile spasms (ISs), hypsarrhythmia, and psychomotor delay. It is considered a rare epileptic encephalopathy typical to manifest in infancy or early childhood.
Case description: This is a case report of a 6-year-old male child who was a known case of global developmental disorder (GDD) with structural WS. The child presented with typical dental findings and a history of ISs in clusters. The case report elucidates the dental treatment modalities followed, which emphasized preventive approaches and home care to complement professional care. Recall appointments were planned to reinforce preventive measures.
Conclusion: Dental care for special needs patients can become more challenging in patients with severe intellectual disabilities, poor communication skills, and a diminished capacity to participate in even routine dental procedures. Thus, the major emphasis of oral health care in patients of WS should be on preventive measures.
Clinical significance: This case report emphasizes the role of a pediatric dentist in the early diagnosis and management of oral health issues in a child with WS. Home care instructions for parents should focus on prevention and aid in inculcating an adequate home care regime to maintain good oral health status.
How to cite this article: Khatri A, Kalra N, Tyagi R, et al. Dental Considerations and Treatment Approaches in West Syndrome—Infantile Spasms: A Case Report. J South Asian Assoc Pediatr Dent 2023;6(3):139–142.
Source of support: Nil
Conflict of interest: None
Keywords: Case report, Hypsarrhythmia, Infantile spasm, West syndrome
INTRODUCTION
The first case of West syndrome (WS) was reported in the 19th century when, in 1841, a physician by the name of WJ West claimed to observe a typical convulsion in his son and wrote a detailed note to the editor of the Lancet.1 The infant boy had no apparent health problems up until the age of 4 months when he started exhibiting clusters of head bobbing. His developmental progress came to a halt subsequently, as these symptoms became more frequent and severe over time. He had obvious signs of mental retardation and passed away when he was 20 years old; an autopsy found no explanation.2 In the early 1960s, Dr Gastaut, Poirier, and Pampiglione gave the term ”West syndrome” to describe a condition that is the most common subtype of infantile spasms (ISs) (approximating 90% of cases of ISs).3-5 The classic triad of WS comprises ISs, hypsarrhythmia, and psychomotor delay. It manifests in infants or early childhood and is classified as malignant.6,7 Around two to 3.5 per every 10,000 live births is the incidence of WS.8,9 A male predilection has been observed, with the ratio being approximately 60:40.10 The classic manifestation of WS is characterized by episodic adduction of the upper limbs and abrupt flexion of the neck and trunk, each episode lasts for a few seconds with bilaterally symmetric tonic spasms in clusters when the patient awakens and also throughout the day. The typical hypsarrhythmic pattern found in an electroencephalogram (EEG) recording demonstrated discharges of waves and spikes varying in amplitude, shape, duration, and location, as well as a random assembly of slow waves with high amplitude. There is an association between this condition and psychomotor delay or developmental regression.5
CASE DESCRIPTION
A 6-year-old male patient reported to the Department of Pediatric and Preventive Dentistry with the complaint of decayed teeth and bleeding gums. Prenatal and perinatal history revealed that the patient was the first offspring born out of the cesarean section from a nonconsanguineous marriage with a birth weight of 2.5 kg. No history of delayed crying at the time of birth was reported. The intake of antenatal iron and folic acid supplements by the mother was mentioned. An antenatal scan revealed ventriculomegaly with a single umbilical artery. Previous medical records revealed that the patient was a known case of global developmental disorder (GDD) with structural WS. The patient was apparently normal till 3 months of age when he presented with seizures, occurring almost daily till the patient was 4.5 months old when the patient was taken to the hospital and diagnosed. The seizures were characterized by severe jerking of the upper and lower limbs. The EEG depicted abnormal signs of arrhythmia hypsarrhythmia and neuronal migration defects (pachygyria lissencephaly, noticeable on magnetic resonance imaging). The parents had no associated comorbidities, and their 2-year-old second child did not present with any medical history. No history of seizures on the maternal or paternal side in the immediate three generations was retrieved. The patient was on lavera 100 mg/mL and valproate 200 mg/5 mL. Neuropsychomotor delay with optic atrophy, atrophic upper and lower limbs, and poor motor skills with the patient’s inability to sit/stand/hold head was revealed in general physical examination. The patient did not respond to verbal commands and was unable to form coherent speech (Fig. 1).
Fig. 1: Neuropsychomotor delay, atrophy of limbs, poor motor skills, unable to sit/stand/hold the head, and optic atrophy
Apparently, a symmetrical face, arched eyebrows, hypertelorism, upward eye-rolling, and incompetent lips with obvious mouth breathing were noted upon extraoral examination (Fig. 2). Intraoral examination revealed generalized attrition, gingival inflammation, high palatal vault, tongue thrust, narrow maxillary arch, generalized hypoplasia, and dental caries with respect to 52, 53, 54, 55, 61, 62, 63, 64, and 65 (Fig. 3).
Fig. 2: Apparently, symmetrical face, arched eyebrows, hypertelorism, upward eye-rolling, and incompetent lips
Fig. 3: Generalized attrition, hypoplasia, dental caries, gingival inflammation, high palatal vault, tongue thrust, and narrow maxillary arch
The radiographic examination could not be attempted due to the physical instability of the patient. The treatment plan was devised in accordance with the patient’s underlying systemic condition and his signs and symptoms, encompassing both preventive and restorative aspects and, at the same time, catering to the patient’s underlying behavior and intellectual deficit. Preprocedural parental counseling sessions were carried out to alleviate their anxiety, and informed consent was obtained for the purpose of publication.
Oral hygiene instructions, along with diet counseling for the avoidance of a cariogenic diet (reduced sugar intake), were given to the parents. A shift to a ketogenic diet was advised. Parents were instructed to consult the pediatrician regarding the replacement of sugar-sweetened medications. Efforts were made patiently to emphasize the importance of family commitment and partnership in providing oral home care support to professional care, which is necessary for maintaining optimal oral health. The child was accommodated on his father’s lap on the dental chair throughout the treatment in order to provide a favorable working position and also to make the child comfortable. Casein phosphopeptide (CPP)–amorphous calcium phosphate (ACP) (GC Tooth Mousse, GC Dental, India) applications were made for the white spot lesions. Caries arrest was done using silver diamine fluoride [38% silver diamine fluoride (SDF) FAgamin Tedequim SRL, Argentina]. Recall appointments were planned to reinforce preventive measures.
DISCUSSION
West syndrome (WS) and infantile spasm are still the most frequently quoted terms in the literature. As a result, the phrase ”epileptic spasm” has been integrated into the umbrella term ”IS syndrome.”11 This is due to the fact that the various types of seizures, EEG findings, and developmental delay tend to occur altogether and simultaneously. The ISS falls under the category of ”early epileptic encephalopathies,” which are severe drug-resistant epileptic disorders first appearing in infancy and characterized by persistent EEG aberrations and cognitive deficits. The seizures themselves or their effects may be attributed directly to dysfunction or to the progression of cerebral dysfunction.12
The average age of onset is 5 months, and the majority of infants experience their first infantile spasm within the first year of life.1 In 2% of patients, it can be after 1 year of age, although WS cases have been reported from infancy to 4 years of age.13 Although the majority of patients are cured of spasms before the age of 5, children may be left with other seizure types, neuropsychomotor delay, and frequent behavioral issues.6
Symptomatic and cryptogenic WS are the two categories that make up this condition. Symptomatic groups occur as a result of pathologies of the central nervous system, which are either suspected or recognized. The root cause of the cryptogenic group is unknown. Idiopathic types of WS have not been included in the International Classification of Diseases.14 The characteristic feature of clusters of spasms is characterized by a rapid and transient axial contraction that is most prominent in the upper limbs and is accompanied by upward eye-rolling. They come in clusters every 5–15 seconds for as long as several minutes and last anywhere from a fraction of a second to 2–3 seconds. In most cases, spasms will have a symmetrical shape. During these motions, the EEG reveals a slow wave with a high amplitude, which is then followed by a protracted decrement. An extended EEG study revealed that spasms occur more frequently when the subject is awake as opposed to when they are asleep.15 Since the EEG is chronically aberrant in WS, the impact on brain function does not fully reverse when the paroxysmal activity is brought under control; hence, there is no interictal time between seizures.16
Published scientific literature states that due to limited dexterity and lack of cooperation with dentists, patients with WS typically have poor oral hygiene, necessitating dental treatment under general anesthesia (GA). Antiepileptic drugs and/or ACTH, which constitute the pharmacological treatment for WS, can induce gingival enlargement, increasing the risk of gingivitis and plaque deposition.17 Two examples of WS requiring dental treatment were described in detail by Khatri et al. Patients with WS were advised to take preventive measures, including a regulated diet, good oral hygiene, and competent dental management, to lessen the likelihood of developing dental problems. Examining the oral cavity of the first patient revealed significant carious lesions, gingival inflammation, somewhat elevated palatal vault, and generalized tooth wear. The surgery was performed under GA. Examining the oral cavity of the second patient found gingival inflammation, several surface carious lesions on deciduous teeth, generalized attrition, and a high palatal vault.18 Multiple carious lesions, molar-incisor hypoplasia, abnormal eruption patterns, gingival hyperplasia, and a high-arched palate were reported in a patient with WS by Della Vella et al.17 A female patient with WS was described by Goswami et al., who noted that upon extraoral examination, the patient displayed hypertelorism, inadequate lips, and arched eyebrows. Symptoms manifested in the oral cavity included a high arch palate, excessively worn teeth, enlarged gums, and many white spot lesions.19
In the present case, the hypoplastic teeth were most likely due to plaque accumulation and poor oral hygiene, a diet high in fermentable carbohydrates, and persistent use of sugar-containing medications. Periodontal problems are more prevalent in children consuming antiepileptic medications. It is possible that the generalized gingival hyperplasia in our patient was associated with long-term antiepileptic medication consumption. Moreover, the patient had persistent mouth breathing that added to the gingival inflammation. To reduce the risk of accidental ingestion of fluoride, the use of CPP-ACP gel was done in the office as it was challenging to control salivation and keep the mouth open for a sufficient duration during treatment. SDF was used to arrest the ongoing caries process since it is an atraumatic method that is widely used in pediatric dental patients.
CONCLUSION
Special needs patients present greater challenges in dental treatment due to significant intellectual disabilities, poor communication skills, and a decreased capacity to cooperate with even basic treatment procedures. Therefore, prevention should be the main goal of oral health care for those with WS.
Clinical Significance
Dental professionals should become familiar with the potential implications and clinical features of WS so as to provide adequate oral health care to these patients. Additionally, it is crucial for dentists to be a part of the medical team treating patients with WS in order to register them in personalized preventative programs as soon as possible and prevent oral issues that might cause additional pain and discomfort.
ORCID
Amit Khatri https://orcid.org/0000-0001-8071-0501
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