Backenzahn-Zeichen (ZNS)

Joubert

syndrome • Joubert syndrome related disorders (JSRD) - Ganzer Fall bei Radiopaedia

Ashraf A, Joubert syndrome related disorders (JSRD). Case study, Radiopaedia.org (Accessed on 27 Sep 2023) https://doi.org/10.53347/rID-164328 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

Choudhary, G. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-11049 • CC by-nc-sa 3.0 (modified)

X-linked

disorders with cerebellar dysgenesis. Brain MRI studies of patients with XLCD. 1 a-c (male proband of a family with X-linked congenital ataxia) Midsagittal T1-weighted image showing an overall size reduction of the cerebellum predominant in the vermis with widened interfolia sulci. Brainstem is normal (a). Axial T2-weighted image showing global cerebellar hypoplasia without dysplasia (b). Axial T2-weighted image showing normal cortex and ventricles (c). d-f (male patient carrying a OPHN1 mutation): midsagittal T1-weighted image showing hypoplastic cerebellar vermis and enlarged cisterna magna (d) axial T2-weighted image showing cerebellar hypoplasia and vermis dysplasia (e) axial T2-weighted image showing lateral ventricle dilatation and mild cortical atrophy (f). g-i (female patient carrying a CASK mutation): Midsagittal T1-weighted image showing moderate vermis hypoplasia and flat pons (g). Axial T2-weighted image showing simplified gyral pattern and ventricular dilatation (h). Coronal T2-weighted image showing global cerebellar hypoplasia and simplified cortical gyri (i).

ojrd.biomedcentral.com • CC-by-2.0

Molar tooth

sign (CNS) • Joubert syndrome - Ganzer Fall bei Radiopaedia

ElBeialy, M. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-38590 • CC by-nc-sa 3.0 (modified)

Molar tooth

sign (CNS) • Joubert syndrome - Ganzer Fall bei Radiopaedia

Ibrahim, D. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-51880 • CC by-nc-sa 3.0 (modified)

Molar tooth

sign (CNS) • Joubert syndrome - Ganzer Fall bei Radiopaedia

T, S. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-88886 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

km, D. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-26602 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

Gaillard, F. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-8237 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

Salam, H. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-8411 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

Salam, H. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-8595 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

Salam, H. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-9389 • CC by-nc-sa 3.0 (modified)

Molar tooth

sign (CNS) • Molar tooth sign - Ganzer Fall bei Radiopaedia

Greif, E. Molar tooth sign. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-25424 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

Gaillard, F. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-8238 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

ElBeialy, M. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-27965 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

km, D. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-17990 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

St-Amant, M. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-18886 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

St-Amant, M. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-22264 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Congenital vermian hypoplasia (Joubert's syndrome) - Ganzer Fall bei Radiopaedia

ElBeialy, M. Congenital vermian hypoplasia (Joubert's syndrome). Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-23415 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

St-Amant, M. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-55004 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome and related disorders - Ganzer Fall bei Radiopaedia

Abdrabou, A. Joubert syndrome and related disorders. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-72464 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome • Joubert syndrome - Ganzer Fall bei Radiopaedia

Almoghazy, S. Joubert syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-86223 • CC by-nc-sa 3.0 (modified)

Joubert

syndrome with associated Dandy-Walker complex. Axial inversion recovery Iiage shows "molar tooth" appearance of midbrain.

www.eurorad.org • CC by-nc-sa-4.0

Molar tooth

sign (CNS) • Molar tooth (photo) - Ganzer Fall bei Radiopaedia

Gaillard, F. Molar tooth (photo). Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-8240 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

The molar tooth sign refers to the appearance of the midbrain in an axial section in which the elongated superior cerebellar peduncles give the midbrain an appearance reminiscent of a molar or wisdom tooth.

It was initially described in Joubert syndrome and related disorders (JSRD) but is now recognized to occur in a number of other conditions, e.g. nephronophthisis, hepatic fibrosis, Cogan's syndrome, and pontine tegmental cap dysplasia .

Molar tooth appearance results from a lack of normal decussation of superior cerebellar peduncular fiber tracts which in turn leads to enlargement of the peduncles, which also follow a more horizontal course .

The absence of crossing fibers also leads to a reduction in the anteroposterior diameter of the midbrain and deepening of the interpeduncular cistern .