Neonatal pontomedullary disconnection with aplasia or destruction of the lower brain stem: a case of pontoneocerebellar hypoplasia?

نویسندگان

  • A C Mamourian
  • G Miller
چکیده

We report a neonate who presented with marked hypotonia and absent suck reflex. MR demonstrated complete absence of the pons as well as absence of a basilar artery flow void. Our case exhibits features similar to those described in previous reports of pontoneocerebellar hypoplasia, but with a more severe degree of pontine involvement. The associated vascular findings suggest a vascular insult to the brain stem as the cause.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

A 46-Year-Old Woman with Unilateral Hearing Loss: A Recent Diagnosis of a Birth Condition

Cochlear nerve (CN) aplasia refers to the absence of a visible CN on oblique sagittal magnetic resonance images of the lateral aspect of the inner auditory canal (IAC). Magnetic resonance (MR) is the preferred technique in patients with sensorineural hearing loss and/or vertigo; however, computed tomography (CT) is used to evaluate the IAC or facial nerve canal. Three types of aplasia or hypopl...

متن کامل

Macrocephaly in association with pontocerebellar hypoplasia type 1: a paradox.

The present case describes a patient with one and a half syndrome due to pontine granuloma (neurocysticercosis). In one and a half syndrome, the lesion affects the paramedian pontine reticular formation (PPRF) (or the abducens nucleus) and the medial longitudinal fasciculus (MLF) on the same side (the MLF having crossed from the opposite side). It is most often caused by multiple sclerosis, bra...

متن کامل

Familial Cleidocranial Dysplasia in a Neonate: A Case Report

Background: Cleidocranial dysplasia (CCD) is a rare inherited skeletal dysplasia, with an incidence of 1 case per 1000,000 individuals. It is a form of predominantly autosomal dominant inheritance and is associated with a mutation in runt related transcription factor-2 gene mapped on chromosome 6p21. This disease primarily affects the bones formed by intramembranous ossification and is characte...

متن کامل

Association of Nasal Nostril Stenosis with Bilateral Choanal Atresia: A Case Report

Introduction: Neonatal nasal airway obstruction induces various degrees of respiratory distress. The management of this disease, including surgical repair, will depend on the severity and location of the obstruction. We describe here a case of congenital nasal nostril stenosis that required surgical repair for stenting of both nares after coanal atresia repair.   Case Report: A 2 days old femal...

متن کامل

Aplasia Cutis Congenita (ACC) and Seizure in a Premature Neonate: Could It Be a New Neurocutaneous Syndrome?

One of the few conditions associated with skin ulceration in the neonatal period is aplasia cutis congenita (ACC). ACC or congenital absence of the skin is considered an uncommon anomaly. This malformation commonly appears on the scalp as a solitary lesion, though it can be seen in other surfaces of the body such as the trunk, limbs and face. ACC can be associated with other physical anomalies ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • AJNR. American journal of neuroradiology

دوره 15 8  شماره 

صفحات  -

تاریخ انتشار 1994