✦ LIBER ✦

MR imaging of Joubert's syndrome

✍ Scribed by R.Nuri Sener

Publisher: Elsevier Science
Year: 1995
Tongue: English
Weight: 552 KB
Volume: 19
Category: Article
ISSN: 0895-6111
DOI: 10.1016/0895-6111(96)00005-5

No coin nor oath required. For personal study only.

✦ Synopsis

Joubert's syndrome is a rare developmental defect of the cerebellar vermis associated with episodic hyperpnea and apnea, abnormal eye movements, and mental retardation. The condition is usually diagnosed clinically during the neonatal period. This article reports nine patients with the syndrome (six males, three females; ages ranging from 2.5 to 9 yrs), and describes MR imaging findings in seven of these. Besides the previously described characteristic and relatively common changes of the syndrome, the MR imaging findings in these patients revealed thinned optic tracts, enlarged temporal horns in the absence of hydrocephalus, high-signal of the cerebral periventricular white matter, abnormal signal in the decussation of the superior cerebellar peduncles, and abnormal embryonic vessels associated with the dysplastic folia of the cerebellar hemispheres.

📜 SIMILAR VOLUMES

Cytomegalovirus infection and Joubert's

Cytomegalovirus infection and Joubert's syndrome

✍ R. Nuri Sener 📂 Article 📅 1997 🏛 Elsevier Science 🌐 English ⚖ 268 KB

A patient with serologically proven congenital cytomegalovirus (CMV) infection is described with periventricular calcifications on CT. The condition was associated with the typical cerebellar changes of clinicoradiologically proven Joubert's syndrome. Although this may be a chance association only,

Cranial sonographic findings in Joubert'

Cranial sonographic findings in Joubert's syndrome

✍ Al Talabani, Joanne A.; Shubbar, Adil I.; Sobkey, Ezat 📂 Article 📅 1999 🏛 John Wiley and Sons 🌐 English ⚖ 368 KB 👁 2 views

The striking clinical picture of periodic hyperpnea and apnea, hypotonia, ataxia, mental retardation, retinal dystrophy, and oculomotor abnormalities found in association with radiologic evidence of agenesis of the cerebellar vermis characterizes Joubert's syndrome. We describe the cranial sonograph

Hajdu-Cheney Syndrome: MR imaging

✍ J. Kawamura; Y. Miki; S. Yamazaki; M. Ogawa 📂 Article 📅 1991 🏛 Springer 🌐 English ⚖ 479 KB

MR imaging of Duane retraction syndrome

✍ Menachem M. Gold; Jacques Romano 📂 Article 📅 2008 🏛 Springer-Verlag 🌐 English ⚖ 77 KB

Rare association of Hirschsprung’s disea

Rare association of Hirschsprung’s disease and Joubert syndrome

✍ Hamit Ozyurek; Ozlem Eroglu Kayacik; Olcay Gungor; Filiz Karagoz 📂 Article 📅 2007 🏛 Springer 🌐 English ⚖ 131 KB

MR imaging in Guillain-Barré syndrome

✍ F. Iwata; Y. Utsumi 📂 Article 📅 1997 🏛 Springer-Verlag 🌐 English ⚖ 445 KB