Oral health challenges in siblings diagnosed with west syndrome: Report of rare case

West syndrome is a rare epileptic encephalopathy of infancy characterized by a triad of infantile spasms, hypsarrhythmia on EEG, and developmental regression. Etiologies include genetic mutations and structural brain abnormalities, and familial cases being exceptionally rare. This report describes two siblings, aged 4 and 7 years, both diagnosed with West syndrome. Both children exhibited neurological deficits, global developmental delay, intellectual disability, and frequent seizures. In addition, they presented with oral manifestations including dental attrition, dental caries, and sialorrhea (excessive salivation), likely due to neuromuscular incoordination and poor oral hygiene. Dental management was challenging due to the high frequency and limited cooperation. As invasive treatment posed significant risks, preventive care was prioritized. This included the application of topical fluoride varnish to arrest carious lesions and dietary modifications to reduce cariogenic exposure. Although general anesthesia was considered for comprehensive care, it was contraindicated due to the children’s classification as ASA Physical Status III, indicating significant systemic compromise. This case highlights the complexities of managing dental health in patients with West syndrome and underscores the importance of early, noninvasive, and multidisciplinary approaches to optimize oral and general health outcomes in neurologically compromised pediatric patients. Keywords: Dentistry, Dental care, Oral manifestations, West syndrome

Authors

Publication Details

Journal
Clinical Dentistry
Published
2025-10-21
DOI
https://doi.org/10.33882/clinicaldent.15.40009
Primary Topic
Epilepsy research and treatment
Type
article
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0.00
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article

Oral health challenges in siblings diagnosed with west syndrome: Report of rare case

Amit Gaikwad, KS Poonacha, Sushil Chavan, Seema Bargale et al.
Clinical Dentistry
Epilepsy research and treatment
article

Oral health challenges in siblings diagnosed with west syndrome: Report of rare case

Amit Gaikwad, KS Poonacha, Sushil Chavan, Seema Bargale, Mrunal Borawake
article en

Abstract

West syndrome is a rare epileptic encephalopathy of infancy characterized by a triad of infantile spasms, hypsarrhythmia on EEG, and developmental regression. Etiologies include genetic mutations and structural brain abnormalities, and familial cases being exceptionally rare. This report describes two siblings, aged 4 and 7 years, both diagnosed with West syndrome. Both children exhibited neurological deficits, global developmental delay, intellectual disability, and frequent seizures. In addition, they presented with oral manifestations including dental attrition, dental caries, and sialorrhea (excessive salivation), likely due to neuromuscular incoordination and poor oral hygiene. Dental management was challenging due to the high frequency and limited cooperation. As invasive treatment posed significant risks, preventive care was prioritized. This included the application of topical fluoride varnish to arrest carious lesions and dietary modifications to reduce cariogenic exposure. Although general anesthesia was considered for comprehensive care, it was contraindicated due to the children’s classification as ASA Physical Status III, indicating significant systemic compromise. This case highlights the complexities of managing dental health in patients with West syndrome and underscores the importance of early, noninvasive, and multidisciplinary approaches to optimize oral and general health outcomes in neurologically compromised pediatric patients. Keywords: Dentistry, Dental care, Oral manifestations, West syndrome

Clinical Dentistry
Openalex Percentile: Top 56%
Epilepsy research and treatment
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Oral health challenges in siblings diagnosed with west syndrome: Report of rare case — Amit Gaikwad, KS Poonacha, et al. · Clinical Dentistry (2025) | TGRS Research Map | TGRS