Abstract
ABSTRACT West Syndrome is one of the rare and severe childhood epilepsies, starting in the first year of life and having an uncertain etiology. Even if some of the symptoms are missing, a triad of them defines West Syndrome, including epileptic spasms, arrest or regression of psychomotor development, and hypsarrhythmia on interictal electroencephalography. The objective of this study was to obtain updated data on West Syndrome literature and report a clinical case of a patient with the medical diagnosis of this syndrome, with gastrostomy feed tube, and clinical pattern of spastic quadriplegia. Initial clinical examination showed prolonged retention of deciduous teeth, periodontal disease, poor oral hygiene, mouth breathing, deep palate, anterior open bite, tongue interposition between the dental arches, and low caries experience. Over 9 years the patient presented complications in their sistemicas conditions, with need for gastrostomy and many periods of hospitalization that determined periods of absence for the dental monitoring. Despite this, currently his oral health condition is good and stable. Dental care for people with disabilities should be developed, encouraged and continuously extended, in agreement with the constitutional principles of human dignity and the rights for health and equality.
Highlights
West Syndrome (WS) and Infantile Spasms (IS) are often used as synonyms [1]
Children treated with an antiepileptic drug are more susceptible to developing periodontal disease
Some researchers speculated that the long-term use of an antiepileptic might be associated with mild and generalized gingival hyperplasia; for example, one side effect of valproate is gingival bleeding [3]
Summary
West Syndrome (WS) and Infantile Spasms (IS) are often used as synonyms [1]. WS is a rare and severe childhood epilepsy syndrome. The incidence of WS is 1 per 2000-4000 individuals born alive It is more frequent in males; it is responsible for 2-10% of the causes of childhood epilepsies [2] and can be associated with other systemic conditions or syndromes [6]. There is an abrupt onset and a gradual evolution, manifesting usually before the first year of life, from 4 to 8 months of age Some epileptic syndromes, such as WS, can evolve catastrophically by a major impact on the cognitive and neuromotor development of the patients due to the difficulty in controlling the crisis and the association with intellectual disabilities [1]. Oral health status can be improved if the family is daily involved with oral hygiene [6] routines
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