Case 266: Pontine Tegmental Cap Dysplasia
نویسندگان
چکیده
منابع مشابه
Cochlear implantation in pontine tegmental cap dysplasia.
Pontine tegmental cap dysplasia (PTCD) is an exceptionally rare brain stem and cerebellar malformation characterized by ventral pontine hypoplasia, vaulted pontine tegmentum, hypoplasia of the vermis, subtotal absence of middle cerebellar peduncles, lateralized course of the superior cerebellar peduncles, and absence or alteration of the inferior olivary nucleus. The main clinical features are ...
متن کاملPontine tegmental cap dysplasia: the neurotologic perspective.
OBJECTIVES Pontine tegmental cap dysplasia (PTCD) is a rare congenital malformation. Clinical and imaging findings in 3 patients and the authors' experience with bilateral cochlear implantation in 1 patient are described. STUDY DESIGN Retrospective review. SETTING Two tertiary medical centers. SUBJECTS AND METHODS Three patients were evaluated by an otolaryngologist and underwent magnetic...
متن کاملNovel clinical features in pontine tegmental cap dysplasia.
Pontine tegmental cap dysplasia is a rare neurologic condition first described by Barth et al. in 2007. It is characterized by a vaulted pontine tegmentum projecting into the fourth ventricle and ventral pontine hypoplasia. Patients present with developmental delay, cerebellar and pyramidal abnormalities, cranial nerve dysfunction, and various extracranial malformations. The condition is though...
متن کاملPontine tegmental cap dysplasia: a novel brain malformation with a defect in axonal guidance.
Four unrelated children are described with an identical brainstem and cerebellar malformation on MRI. The key findings are: vermal hypoplasia, subtotal absence of middle cerebellar peduncles, flattened ventral pons, vaulted pontine tegmentum, molar tooth aspect of the pontomesencephalic junction and absent inferior olivary prominence. Peripheral hearing impairment is present in all. Variable fi...
متن کاملPontine tegmental cap dysplasia: MR imaging and diffusion tensor imaging features of impaired axonal navigation.
BACKGROUND AND PURPOSE Malformations of the brain stem are uncommon. We present MR imaging and diffusion tensor imaging (DTI) features of 6 patients with pontine tegmental cap dysplasia, characterized by ventral pontine hypoplasia and a dorsal "bump," and speculate on potential mechanisms by which it forms. MATERIALS AND METHODS Birth and developmental records of 6 patients were reviewed. We ...
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ژورنال
عنوان ژورنال: Radiology
سال: 2019
ISSN: 0033-8419,1527-1315
DOI: 10.1148/radiol.2018162304