Foix–Chavany–Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster-Drought syndrome
Autor: | Finkenstaedt M, Imhäuser S, Hanefeld F, Ernst Jp, Christen Hj, Kruse E |
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Rok vydání: | 2000 |
Předmět: |
Male
Pediatrics medicine.medical_specialty Pathology Adolescent Worster Drought syndrome Developmental Disabilities Bulbar Palsy Progressive Status epilepticus Speech Disorders Diagnosis Differential Dysgenesis Dysarthria Developmental Neuroscience Meningoencephalitis medicine Humans Congenital bilateral perisylvian syndrome Child Anarthria Brain Diseases Palsy biology Brain Infant Syndrome medicine.disease biology.organism_classification Cranial Nerve Diseases Foix–Chavany–Marie syndrome Child Preschool Pediatrics Perinatology and Child Health Female Neurology (clinical) medicine.symptom Deglutition Disorders Tomography X-Ray Computed Psychology |
Zdroj: | Developmental Medicine & Child Neurology. 42:122-132 |
ISSN: | 0012-1622 |
Popis: | Foix-Chavany-Marie syndrome (FCMS) is a distinct clinical picture of suprabulbar (pseudobulbar) palsy due to bilateral anterior opercular lesions. Symptoms include anarthria/severe dysarthria and loss of voluntary muscular functions of the face and tongue, and problems with mastication and swallowing with preservation of reflex and autonomic functions. FCMS may be congenital or acquired as well as persistent or intermittent. The aetiology is heterogeneous; vascular events in adulthood, nearly exclusively affecting adults who experience multiple subsequent strokes; CNS infections; bilateral dysgenesis of the perisylvian region; and epileptic disorders. Of the six cases reported here, three children had FCMS as the result of meningoencephalitis, two children had FCMS due to a congenital bilateral perisylvian syndrome, and one child had intermittent FCMS due to an atypical benign partial epilepsy with partial status epilepticus. The congenital dysgenetic type of FCMS and its functional epileptogenic variant share clinical and EEG features suggesting a common pathogenesis. Consequently, an increased vulnerability of the perisylvian region to adverse events in utero is discussed. In honour of Worster-Drought, who described the clinical entity in children 40 years ago, the term Worster-Drought syndrome is proposed for this unique disorder in children. |
Databáze: | OpenAIRE |
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