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Worster-Drought syndrome (congenital bilateral perisylvian syndrome) with posterior pituitary ectopia, pituitary hypoplasia, empty sella and panhypopituitarism: a patient report.

Abstract
Worster-Drought syndrome (WDS) (congenital bilateral perisylvian syndrome, congenital pseudobulbar paresia) is characterized by neuronal migration defect, pseudobulbar paralysis, epilepsy, neuromotor retardation and perisylvian dysplasia. We report a patient with WDS associated with posterior pituitary ectopia, pituitary hypoplasia, partial empty sella and panhypopituitarism, not previously reported in the literature. The 16.4 year-old female patient had severe growth retardation with height SDS -4.5, delayed puberty, microcephaly, pes equinovarus deformity, developmental delay, speech disorder and epilepsy. Laboratory findings, which revealed abnormal electroencephalography and bilateral perisylvian cortical dysplasia on cranial magnetic resonance imaging (MRI) were consistent with WDS. Endocrinological evaluation revealed secondary hypothyroidism and combined deficiency of adrenocorticotropin, gonadotropin and growth hormone (GH). Sella MRI showed congenital empty sella, anterior pituitary hypoplasia, ectopic neurohypophysis, and stalk agenesis. Appropriate replacement therapy was started. GH treatment resulted in a final height of 150.3 cm, appropriate for her target height. This is the first reported patient with WDS associated with congenital structural hypothalamic-pituitary abnormalities, including empty sella, pituitary hypoplasia, posterior pituitary ectopia, stalk agenesis and panhypopituitarism. GH has been successful in the treatment of her short stature.
AuthorsFirdevs Baş, Feyza Darendeliler, Zuhal Yapici, Selman Gökalp, Rüveyde Bundak, Nurçin Saka, Hülya Günöz
JournalJournal of pediatric endocrinology & metabolism : JPEM (J Pediatr Endocrinol Metab) Vol. 19 Issue 4 Pg. 535-40 (Apr 2006) ISSN: 0334-018X [Print] Germany
PMID16759040 (Publication Type: Case Reports, Journal Article)
Topics
  • Adolescent
  • Adult
  • Cerebral Cortex (abnormalities)
  • Choristoma (diagnosis)
  • Empty Sella Syndrome (congenital, diagnosis)
  • Female
  • Humans
  • Hypopituitarism (complications, congenital, diagnosis)
  • Pituitary Gland (abnormalities)
  • Pituitary Gland, Posterior
  • Syndrome

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