Viewpoint: Decline in Speech and Language Skills May Signal Childhood Dementia

PURPOSE: Childhood dementia is an umbrella term for over 140 genetic disorders that cause devastating neurodegeneration and death. Early diagnosis of these disorders is essential to inform future decision making and provide tailored therapies and supports. Diagnosis may allow access to treatments, such as gene therapy, which are rapidly evolving. Speech and language impairments are often the first signs of childhood dementia. Yet, limited awareness of childhood dementia and poor speech and language characterization results in serious diagnostic delays. This viewpoint seeks to both delineate the early speech and language features in childhood dementia and support timely referral of children to specialists. METHOD: We reviewed speech and language features reported before, and at genetic diagnosis, across childhood dementia disorders. We identify three key speech and language red flags for childhood dementia: (a) delayed or plateaued speech and language milestones, (b) onset or change in dysarthria, and (c) speech and language regression. We also highlight other medical features that may co-occur with these speech and language impairments. CONCLUSIONS: We identified early speech and language impairments in children with dementia that may support referral to specialists. Speech pathologists and other pediatric clinicians should be aware of childhood dementia and the presenting speech and language features. Early access to genetic testing is imperative for best outcomes for children with childhood dementia and their families. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.32336961.