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A new case of Worster-Drought syndrome

  • Autori: Spitaleri, C; Caramella, F; Glorioso, P; Nardello, R; Fontana, A; Mangano, S
  • Anno di pubblicazione: 2012
  • Tipologia: eedings
  • Parole Chiave: Worster-Drought syndrome;Congenital bilateral perisylvian syndrome
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Introduction: Worster-Drought syndrome (WDS) consists of a congenital pseudobulbar palsy and is usually associated with spastic tetraplegia, learning impairment, behavioural problems, and epilepsy. Congenital bilateral perisylvian syndrome (CBPS) is characterized by bilateral perisylvian polymicrogyria on imaging. Clark et al, have previously proposed a WDS spectrum that includes CBPS, speculating that it may be due to malformation of the perisylvian region due to various perinatal or congenital causes, whether demonstrable on imaging, or functional and not visible with current imaging techniques. Worster-Drought suggested that the syndrome is probably a developmental defect of the motor tracts (corticobulbar) which run from the lower part of the motor (Rolandic) area of the cerebral cortex to the 10th and 12th cranial nerve nuclei. Methods: A 12-year-old boy presented with a long history of spastic tetraplegia, learning impairment, behavioural problems, and epilepsy. The seizures were refractive to several antiepileptic drugs. He also had variable difficulty in moving the tongue, soft palate and lips with weakness of the pharynx and laryngeal muscle. Results: Video-EEG monitoring captured paroxysms of PO in the center-front of both hemispheres, and that project contralaterally. Neuroimaging studies do not revealed abnormalities. Conclusion: Our patient presented the characteristic symptoms of Worster-Drought syndrome (WDS) with mild spastic tetraplegia, learning impairment, and epilepsy. MRI did not show dysplastic cortex in the region of the sylvian fissures, giving normal signal on T1- and T2-weighted images. Although the white matter had a normal appearance, an abnormality in the corticobulbar tracts cannot be excluded.