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Congenital Malformations CONGENITAL VERMIAN HYPOPLASIA 34 Sagittal T1WI MR shows vermian remnant (arrows). Fastigial point and primary fissure are lacking. The brainstem and pituitary axis are small. Axial T2WI MR shows the typical "molar tooth" (arrow) appearance of the brainstem. There is clefting of the vermis (curved arrow). Gleeson JG et al: Molar tooth sign of the midbrain-hindbrain junction: Occurrence in multiple distinct syndromes. Am J Med Genet. J Child NeuroI17(12):911-3, 2002 Yachnis AT et al: Neuropathology of Joubert syndrome.

J Child NeuroI14(10):655-9, 1999 Quisling RG et al: MRI features and classification of CNS malformations in Joubert syndrome. J Child Neurol 14:628-36, 1999 Satran D et al: Cerebello-oculo-renal syndromes including Arima, Senior-Loken and Coach syndromes: More than just variants of Joubert syndrome.

Am J Med Genet 86(5):459-69, 1999 Maria BL et al: Clinical features and revised diagnostic criteria in Joubert syndrome. J Child NeuroI14(9):583-90, 1999 Yachnis AT et al: Cerebellar Nifedipine Extended Release Tablets (Procardia XL)- FDA brainstem development: An overview in relation to Joubert syndrome. J Child NeuroI14(9):570-3, 1999 Maria BL et al: Molar tooth sign in Joubert syndrome: Clinical, radiologic, and pathologic significance.

The superior cerebellar peduncles (curved arrow) are well seen due to hypoplasia of the usually intervening anterior vermian lobules. Typical (Left) Axial T2WI MR shows hypertelorism and frontonasal dysplasia.

Note large aqueduct of Sylvius with disturbed flow (curved arrow). Superior CBLL peduncles (arrow) are well seen. American association of diabetes combination gives the appearance of an open "umbrella".

Typical (Left) Coronal T2WI Nifedipine Extended Release Tablets (Procardia XL)- FDA shows apposed cerebellar hemispheres and heterotopic nodule (arrow) embedded within the Nifedipine Extended Release Tablets (Procardia XL)- FDA matter.

Abnormal axis due to central up-tilting of the hemispheres. Prominent cisterna magna (curved arrow). Congenital Malformations HOLOPROSENCEPHALY 38 Coronal oblique 30 SPCR surface reconstruction shows absence of interhemispheric fissure and fusion of the gyri across the midline.

The frontal lobe is hypoplastic and made by the indications of the and white matter are fused across the midline (open arrow). REFERENCES Hayashi M et al: Neuropatholigcal evaluation of the diencephalon, basal ganglia and upper brainstem in alobar holoprosencephaly.

Acta Neuropathol107(3):190-6, 2004 Blaas HG et al: Brains and faces in holoprosencephaly: Preand postnatal description DDAVP Nasal Spray (Desmopressin Acetate Nasal Spray)- Multum 30 cases. Ultrasound Obstet GynecoI19(1):24-38, 2002 Simon EM et al: The middle biography johnson variant of holoprosencephaly.

AJNR23(1):151-6,2002 Barkovich AJ et al: Analysis of the cerebral cortex in HPE with attention to the Sylvian fissures. AJNR23:143-50, 2002 Simon EM et al: The dorsal cyst in holoprosencephaly and the role of the thalamus in its formation. Note the partially fused thalami (curved arrow). Small posterior tissue band (arrows) represents hippocampal formation. Cyst wall (arrows) is comprised of telencephalic roof plate and tela choroidea remnants.

Be and thalami form midline fusion mass (open arrow). Congenital Malformations HOLOPROSENCEPHALY VARIANTS 42 SMMCI. Coronal NECT shows a single median maxillary central incisor (SMMCI) (arrow). Note the precise midline location.

Axial 3D SPCR shows interhemispheric fusion of the sylvian fissure (SF), posterior frontal and parietal lobes. Note branches of the middle cerebral artery in the SF (arrows). Simon EM et al: The middle interhemispheric variant of holoprosencephaly. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Axial NECT shows an unerupted single median maxillary central incisor (SMMCI). Note normal incisor morphology. Nifedipine Extended Release Tablets (Procardia XL)- FDA prominent vomerine ridge is seen in the mid palate Nifedipine Extended Release Tablets (Procardia XL)- FDA. Axial NECT in a patient with SMMCI shows overgrowth of the nasal process of maxilla (arrows) consistent with nasal pyriform aperture stenosis (NPAS).

Sagittal T1WI MR shows a dysgenetic corpus callosum (CC) with only a normal genu (arrow) identified. In classic forms HPE, the genu is the least well formed portion of the cc.

Axial T2WI MR shows fusion of posterior ventricles, absent septum pellucidum, findings also seen in classic HPE. However, note normal cleavage of the basal ganglia, findings typical of MIH. Axial T1WI MR shows clear separation of the frontal and occipital poles with the interhemispheric fissure (lHF) identified anteriorly and posteriorly abnormal ecg. Axial 30 SPCR through the rostral brain shows interhemispheric fusion of posterior frontal and parietal lobes with focal absence of IH F (arrow).

Pfizer vaccine doses right frontal lobe pachygyria (open arrow). Anterior horns are draped inferiorly around fornices (open arrow). Optic chiasm (arrow) small. Campbell CL: Septo-optic dysplasia: a literature review.

Wakeling EL et al: Septo-optic dysplasia, subglottic stenosis and skeletal abnormalities: a case report.



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