nach oben

Pediatric Radiology

Erschienen in:

01.11.2004 | Case Report

Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

verfasst von: Ensar Yekeler, Meral Ozmen, Hakan Genchellac, Memduh Dursun, Gulden Acunas

Erschienen in: Pediatric Radiology | Ausgabe 11/2004

Einloggen, um Zugang zu erhalten

Abstract

Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented.

Kuzniecky R, Andermann F, Guerrini R (1993) Congenital bilateral perisylvian syndrome: study of 31 patients. CBPS Multicenter Collaborative Study. Lancet 341:608–612CrossRefPubMed

Guerreiro MM, Andermann E, Guerrini R, et al (2000) Familial perisylvian polymicrogyria: a new familial syndrome of cortical maldevelopment. Ann Neurol 48:39–48CrossRefPubMed

Sejima H, Takusa Y, Kimura M, et al (2001) A variant case of congenital bilateral perisylvian syndrome with asymmetric findings on neuroimaging and septum pellucidum defect. Brain Dev 23:131–134CrossRefPubMed

Powell HW, Hart PE, Sisodiya SM (2003) Epilepsy and perisylvian polymicrogyria in a patient with Kabuki syndrome. Dev Med Child Neurol 45:841–843CrossRefPubMed

Yamanouchi H, Ota T, Imataka G, et al (2002) Congenital bilateral perisylvian syndrome associated with congenital constriction band syndrome. J Child Neurol 17:448–450PubMed

Kuker W, Friese S, Riethmuller J, et al (2000) Congenital bilateral perisylvian syndrome (CBPS): do concomitant esophageal malformations indicate a poor prognosis? Neuropediatrics 31:310–313CrossRefPubMed

Echaniz-Laguna A, de Saint-Martin A, Lafontaine AL, et al (2000) Bilateral focal polymicrogyria in Ehlers–Danlos syndrome. Arch Neurol 57:123–127CrossRefPubMed

Sener RN (1996) Bilateral, perisylvian and rolandic cortical dysplasia in trisomy 13 syndrome. J Neuroradiol 23:231–233PubMed

Naidich TP, McLane DG, Bauer BS, et al (1983) Midline craniofacial dysraphism: midline cleft upper lip, basal encephalocele, callosal agenesis, and optic nerve dysplasia. Concepts Pediatr Neurosurg 4:186–207

10.

Blinder G, Corat-Simon J, Hershkovitz E (2001) Micropituitarism and cortical dysplasia: an unknown association of two uncommon CNS disorders. Eur Radiol 11:1070–1072CrossRefPubMed

11.

Mitchell LA, Thomas PQ, Zacharin MR, et al (2002) Ectopic posterior pituitary lobe and periventricular heterotopia: cerebral malformations with the same underlying mechanism? AJNR 23:1475–1481

12.

Friede RL (1989) Developmental neuropathology, 2nd edn. Springer, Berlin Heidelberg New York

13.

Barkovich AJ, Kuzniecky RI, Dobyns WB, et al (1996) A classification scheme for malformations of cortical development. Neuropediatrics 27:59–63PubMed

14.

Barkovich AJ, Gressens P, Evrard P (1992) Formation, maturation, and disorders of brain neocortex. AJNR 13:423–446PubMed

15.

Barkovich AJ, Hevner R, Guerrini R (1999) Syndromes of bilateral symmetrical polymicrogyria. AJNR 20:1814–1821

16.

Ikeda H, Suzuki J, Sasano N, et al (1988) The development and morphogenesis of the human pituitary gland. Anat Embryol 178:327–336PubMed

17.

Gleiberman AS, Fedtsova NG, Rosenfeld MG (1999) Tissue interactions in the induction of anterior pituitary: role of the ventral diencephalon, mesenchyme, and notochord. Dev Biol 213:340–353CrossRefPubMed

Titel: Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis
verfasst von: Ensar Yekeler
Meral Ozmen
Hakan Genchellac
Memduh Dursun
Gulden Acunas
Publikationsdatum: 01.11.2004
Verlag: Springer-Verlag
Erschienen in: Pediatric Radiology / Ausgabe 11/2004
Print ISSN: 0301-0449
Elektronische ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-004-1221-9

Update Radiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

Abstract

Neu im Fachgebiet Radiologie

Akuter Schwindel: Wann lohnt sich eine MRT?

Screening-Mammografie offenbart erhöhtes Herz-Kreislauf-Risiko

S3-Leitlinie zu Pankreaskrebs aktualisiert

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

Update Radiologie

Springer Medizin

Abstract

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 11/2004

Interrupted aortic arch in a neonate: multidetector CT diagnosis

Resolution of calcific brown fat necrosis associated with prostaglandin therapy for cyanotic congenital heart disease in neonates: report of two cases

Definitive neuroradiological diagnostic features of tuberculous meningitis in children

Comparison of ultrasound with plain radiography and CT for the detection of mediastinal lymphadenopathy in children with tuberculosis

Dr. Jack O. Haller

Modern imaging of tuberculosis in children: thoracic, central nervous system and abdominal tuberculosis

Neu im Fachgebiet Radiologie

Akuter Schwindel: Wann lohnt sich eine MRT?

Screening-Mammografie offenbart erhöhtes Herz-Kreislauf-Risiko

S3-Leitlinie zu Pankreaskrebs aktualisiert

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

Update Radiologie