Developmentally anomalous cerebellar encephalocele arising within the cerebellopontine angle and extending into the adjacent skull base in a pediatric patient.

Lesions of the cerebellopontine angle (CPA) in young children are rare, with the most common being arachnoid cysts and epidermoid inclusion cysts. The authors report a case of an encephalocele containing heterotopic cerebellar tissue arising from the right middle cerebellar peduncle and filling the right internal acoustic canal in a 2-year-old female patient. Her initial presentation included a focal left 6th nerve palsy.

Brain Magnetic Resonance Imaging in Congenital Cytomegalovirus With Failed Newborn Hearing Screen.

Background: In 2013, Utah enacted legislation requiring that infants failing newborn hearing screening be tested for cytomegalovirus infection. As a result, cytomegalovirus-infected infants are being identified because of hearing deficits. The neuroimaging findings in this population have not been characterized.

Correction to: A consensus response on the complete picture: reply to Lynøe and Eriksson.

The original version on this paper contained an error. The COI statement is incorrectly presented.

Intracranial Calcifications in Young Children.

Intracranial calcifications in young infants, while suggesting intrauterine infections, can also be due to numerous other conditions, including rare genetic disorders. We describe 2 children in whom the presence and pattern of intracranial calcifications led to the diagnosis of uncommon genetic disorders, Adams-Oliver syndrome and Aicardi-Goutieres syndrome. Differentiating genetic conditions from intrauterine infections or other causes of intracranial calcifications enables practitioners to provide accurate counseling regarding prognosis and recurrence risk.

Consensus statement on abusive head trauma in infants and young children.

Abusive head trauma (AHT) is the leading cause of fatal head injuries in children younger than 2 years. A multidisciplinary team bases this diagnosis on history, physical examination, imaging and laboratory findings. Because the etiology of the injury is multifactorial (shaking, shaking and impact, impact, etc.

Recognition and Management of Acute Flaccid Myelitis in Children.

Background: In 2014-2015, several regions of the United States experienced an outbreak of acute flaccid myelitis in pediatric patients. A common, unique feature was disease localization to the gray matter of the spinal cord.

Methods: We report 11 children, ages 13 months to 14 years (median 9 years), in the Intermountain West who presented with extremity weakness (n = 10) or cranial neuropathy (n = 1) of varying severity without an apparent etiology.

Common pediatric head and neck congenital/developmental anomalies.

Pediatric head and neck neuroradiology is a broad and complex topic. This article focuses on several of the common and sometimes challenging pediatric head and neck congenital/developmental anomalies physicians may encounter in clinical practice. Although some diagnoses may be evident on physical examination, others may present a diagnostic dilemma.

Congenital cytomegalovirus infection and brain clefting.

Background: Human cytomegalovirus, a major cause of permanent neurodevelopmental disability in children, frequently produces intracranial abnormalities, including calcifications and polymicrogyria, in infants with congenital cytomegalovirus infections. This report describes the features of cerebral cortical clefting, including schizencephaly, in children with congenital cytomegalovirus infection.

Methods: This is a retrospective review of the medical records of infants and children with congenital cytomegalovirus infection evaluated at Primary Children's Medical Center, Salt Lake City, Utah, between 1999 and 2008.

Cerebral sinovenous thrombosis in pediatric practice.

Cerebral sinovenous thrombosis (CSVT) in the pediatric population is a relatively uncommon yet under-appreciated and potentially life-threatening neurological condition. Early symptoms and signs are often vague and the clinician requesting a cranial imaging study might not even suspect sinovenous thrombosis. If left undiagnosed, or if the diagnosis of CSVT is delayed, progressive neurological deterioration, coma and death can follow.

Primary lesions of the root of the tongue.

In the assessment of the head and neck, differential diagnoses can be formulated by subdividing the anatomy into spaces along identifiable and logical boundaries. In the oral cavity, the root of the tongue is notably unlike adjacent regions due to structural and tissue-specific differences. The majority of lesions found in the root of the tongue are congenital and benign, representing ectopic tissues of thyroidal, epidermal, dermal, foregut, venous, and lymphatic origin.

MPPH syndrome: two new cases.

This report describes 2 additional cases of megalencephaly and perisylvian polymicrogyria with postaxial polydactyly and hydrocephalus syndrome, a recently recognized disorder of infants and young children with macrocrania, developmental delay/mental retardation, and often epilepsy. Medulloblastoma, a previously unreported feature in megalencephaly and perisylvian polymicrogyria with postaxial polydactyly and hydrocephalus syndrome, developed in one child at 3 years of age. Although the disorder is presumed to be genetic, the cause of megalencephaly and perisylvian polymicrogyria with postaxial polydactyly and hydrocephalus syndrome has not yet been determined.

Neuroimaging of abusive head trauma.

The diagnostic process for evaluating suspected abusive head trauma in infants and children has evolved with technological advances in neuroimaging. Since Caffey first described a series of children with chronic subdural hematomas and multiple long bone fractures, radiologists have played an important role, along with pediatricians and pathologists, in evaluating abused children. Neuroimaging modalities include ultrasound, CT scans, and MRI technology.

Tumefactive intracranial presentation of precursor B-cell acute lymphoblastic leukemia.

In children, leukemia is the most common malignancy, and approximately 75% of leukemias are acute lymphoblastic leukemia (ALL). Central nervous system leukemia is found at diagnosis in fewer than 5% of children with ALL. Leukemic intracranial masses have been described with acute myeloid leukemia, but ALL presenting as a mass lesion is rare.

Progressive cerebral vascular degeneration with mitochondrial encephalopathy.

MELAS (mitochondrial encephalopathy with lactic acidosis and stroke-like episodes) is a maternally inherited disorder characterized by recurrent cerebral infarctions that do not conform to discreet vascular territories. Here we report on a patient who presented at 7 years of age with loss of consciousness and severe metabolic acidosis following vomiting and dehydration. She developed progressive sensorineural hearing loss, myopathy, ptosis, short stature, and mild developmental delays after normal early development.

Non-enhancing pilocytic astrocytoma of the spinal cord.

Pilocytic astrocytomas are among the most common intramedullary spinal cord tumors in the pediatric age group. The presence of contrast enhancement is a major factor used to distinguish these tumors from other spinal cord lesions. We present a case of histologically proved non-enhancing intramedullary spinal cord pilocytic astrocytoma in a 12-year-old girl.

Glutaric acidemia type 1.

Glutaric acidemias comprise different disorders resulting in an increased urinary excretion of glutaric acid. Glutaric acidemia type 1 (GA-1) is an autosomal recessive disorder of lysine, hydroxylysine, and tryptophan metabolism caused by deficiency of glutaryl-CoA dehydrogenase. It results in the accumulation of 3-hydroxyglutaric and glutaric acid.

Chiari I malformation in patients with FG syndrome.

Object: The FG syndrome (FGS) is a common, heterogeneous group of clinically indistinguishable X-linked disorders comprising congenital hypotonia, macrocephaly, psychomotor delay, abnormalities in sensory integration, agenesis of corpus callosum, an unusual personality with behavior abnormalities, and disturbances of gastrointestinal function. On magnetic resonance (MR) imaging, some patients have evidence of tonsillar ectopia. The authors describe the incidence of Chiari I malformation in patients with FGS and attempt to determine the optimal treatment of these patients.

Nitric oxide increases the signal intensity of the T1-weighted magnetic resonance image of blood.

Human blood was infused through a gas permeable membrane to determine if treatment with nitric oxide affects the signal intensity of its T1-weighted magnetic resonance image. Equal volumes of blood with increasing concentrations of methemoglobin were placed in glass tubes. T1-weighted images of the samples were obtained with a 1.

Spinal cord schwannomas mimicking drop metastases in a patient with intramedullary ependymoma and neurofibromatosis 2.

The authors describe a 9-year-old boy with a large spinal cord ependymoma whose apparent drop metastases on imaging studies were later demonstrated to be schwannomas. This finding led to the discovery of small bilateral acoustic neuromas and an intracranial meningioma, establishing the diagnosis of neurofibromatosis 2. The presence of additional radiographic abnormalities in a patient with a spinal cord tumor should prompt careful consideration of the diagnosis of neurofibromatosis 2, as early identification of this disorder may significantly affect patient management.

Percutaneous cholecysto-cholangiography in the diagnosis of obstructive jaundice in infants.

Background: Once it is established that a jaundiced infant has an elevated direct bilirubin level, the principal diagnostic concern is the differentiation of hepatocellular from obstructive cholestasis, of disorders of physiology from disorders of anatomy, and of disease that is managed medically from disease that is managed surgically. Traditional tests such as ultrasonography, liver biopsy, and technotium 99m HIDA scan are often not sufficiently discriminating. General anesthesia is required for invasive imaging with endoscopic retrograde cholangio pancreatography (ERCP) or operative cholangiogram.

Neuroradiology of the central nervous system in childhood.

The purpose of this article is to familiarize readers with new imaging applications, identify the relative strengths of imaging modalities, and emphasize practical applications of imaging the child's nervous system. Because of recent advances in MRI, the article emphasizes the expanding role of MRI in evaluating children with neurologic disease.