Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech

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Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech.

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 neuropsychologica...

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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...

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The Worster-Drought syndrome and other syndromes of dementia with spastic paraparesis: the paradox of molecular pathology.

The molecular demarcation of otherwise indistinguishable clinicopathologic syndromes is providing us with clear evidence of the limited spectrum within which the brain can respond to distinct molecular aberrations. At the same time, surprisingly varied phenotypes may also occur within and between pedigrees with the same or similar genetic defect. This paradox is particularly well illustrated in...

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Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

BACKGROUND Congenital hyperinsulinism (CHI) is a rare but severe disorder of hypoglycemia in children, often complicated by brain injury. In CHI, the long-term prevention of hypoglycemia is dependent on reliable enteral intake of glucose. However, feeding problems (FPs) often impede oral glucose delivery, thereby complicating the management of hypoglycemia. FPs have not been systematically char...

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ژورنال

عنوان ژورنال: Developmental Medicine & Child Neurology

سال: 2010

ISSN: 0012-1622,1469-8749

DOI: 10.1111/j.1469-8749.2009.03475.x