Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech.
نویسندگان
چکیده
AIM Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing, feeding, speech, and saliva control owing to a non-progressive disturbance in early brain development. As such, it falls within the cerebral palsies. The aim of this study was to describe the physical and neuropsychological profiles of children with WDS. METHOD Forty-two children with WDS (26 males, 16 females; mean age 7y 10mo, SD 3y 1mo; range 2y 6mo to 16y 5mo) were studied prospectively using a standard protocol. RESULTS All of the children had severe bulbar dysfunction; 36 out of 42 had feeding difficulties and 23 of 38 had unintelligible speech, which was poorly compensated for by augmentative communication. There were accompanying disturbances in cognition (mean non-verbal IQ 59), behaviour (12/40 attention-deficit-hyperactivity disorder [ADHD]), social communication (8/42 autism), and epilepsy (12/39). The severity of bulbar dysfunction and impact of additional impairments made it difficult to use formal assessments. INTERPRETATION WDS causes severe and persistent bulbar dysfunction that is often accompanied by additional impairments, as in other cerebral palsies. Speech prognosis is particularly poor. Early diagnosis with appreciation of the underlying neurology would encourage critical evaluation of interventions and long-term planning to improve outcome.
منابع مشابه
Speech difficulties in Joubert syndrome
Introduction: "Joubert syndrome" was first introduced in1969. This syndrome is a rare genetic disease with autosomal dominantpattern. Hypotonia, ataxia and motor delay of the disease known as clinical manifestations. In the few reports of this syndrome, mostly functional and structural components studied and radiographic images such as speech and language developmental delay symptoms has been l...
متن کاملCri du Chat Syndrome: a Case Report with Recurrent Pneumonia and Chronic Stridor
Introduction Cri du chat syndrome is a rare genetic disorder due to deletion of variable length of short arm of chromosome 5(5p). It mainly presents with typical cat like cry, facial dysmorphism, poor growth with feeding problems and severe cognitive, speech, and motor delays. Case Report We present here a one year old child who did not presented with typical features but presented with recurre...
متن کاملPrevalence of Feeding Problems in Children with Intellectual Disability
Objectives: Feeding is an essential function which affects quality of life of the intellectually disabled (ID) persons. Approximately 80% of the severe and profound mentally retarded population have some feeding difficulties. This study aimed to determine the prevalence of feeding problems in children with ID. Methods: In this cross-sectional descriptive study, 144 individuals with Intellect...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Developmental medicine and child neurology
دوره 52 1 شماره
صفحات -
تاریخ انتشار 2010