Publications by authors named "Nilesh K Desai"

20 Publications

  • Page 1 of 1

ACR Appropriateness Criteria® Seizures-Child.

J Am Coll Radiol 2021 May;18(5S):S199-S211

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

In children, seizures represent an extremely heterogeneous group of medical conditions ranging from benign cases, such as a simple febrile seizure, to life-threatening situations, such as status epilepticus. Underlying causes of seizures also represent a wide range of pathologies from idiopathic cases, usually genetic, to a variety of acute and chronic intracranial or systemic abnormalities. This document discusses appropriate utilization of neuroimaging tests in a child with seizures. The clinical scenarios in this document take into consideration different circumstances at the time of a child's presentation including the patient's age, precipitating event (if any), and clinical and electroencephalogram findings and include neonatal seizures, simple and complex febrile seizures, post-traumatic seizures, focal seizures, primary generalized seizures in a neurologically normal child, and generalized seizures in neurologically abnormal child. This practical approach aims to guide clinicians in clinical decision-making and to help identify efficient and appropriate imaging workup. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2021.02.020DOI Listing
May 2021

After the initial COVID-19 surge: a phased radiology departmental re-opening plan.

Pediatr Radiol 2020 10 22;50(11):1551-1559. Epub 2020 Aug 22.

Edward B. Singleton Department of Radiology, Texas Children's Hospital, 6701 Fannin St., Suite 470, Houston, TX, 77030, USA.

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http://dx.doi.org/10.1007/s00247-020-04792-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7442886PMC
October 2020

Degos disease: A radiological-pathological correlation of the neuroradiological aspects of the disease.

Ann Diagn Pathol 2020 Aug 30;47:151545. Epub 2020 May 30.

Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States of America.

Malignant atrophic papulosis (Degos disease) is an unusual thrombotic microangiopathy of uncertain etiology. The disease characteristically involves the skin and internal organs, with nervous system involvement more common in children. We present a case with diverse neurological manifestations including cranial nerve palsies, gait instability, and urinary incontinence. The patient also developed white papular lesions on her lower extremities and back. Magnetic resonance imaging (MRI) demonstrated progressive intracranial and spinal abnormalities. Despite treatment with numerous biologic agents, the patient had persistent clinical deterioration and expired one month after admission. We highlight the extensive neurologic manifestations of Degos disease correlated with neuroradiological imaging and pathological features. Nervous system involvement in Degos disease requires careful neurologic and dermatologic exam with central nervous system (CNS) magnetic resonance imaging to distinguish it from non-organic etiologies of similar symptoms.
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http://dx.doi.org/10.1016/j.anndiagpath.2020.151545DOI Listing
August 2020

ACR Appropriateness Criteria® Cerebrovascular Disease-Child.

J Am Coll Radiol 2020 May;17(5S):S36-S54

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Stroke is an uncommon but an important and under-recognized cause of morbidity and mortality in children. Strokes may be due to either brain ischemia or intracranial hemorrhage. Common symptoms of pediatric acute stroke include headache, vomiting, focal weakness, numbness, visual disturbance, seizures, and altered consciousness. Most children presenting with an acute neurologic deficit do not have an acute stroke, but have symptoms due to stroke mimics which include complicated migraine, seizures with postictal paralysis, and Bell palsy. Because of frequency of stroke mimics, in children and the common lack of specificity in symptoms, the diagnosis of a true stroke may be delayed. There are a relatively large number of potential causes of stroke mimic and true stroke. Consequently, imaging plays a critical role in the assessment of children with possible stroke and especially in children who present with acute onset of stroke symptoms. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2020.01.036DOI Listing
May 2020

ACR Appropriateness Criteria® Head Trauma-Child.

J Am Coll Radiol 2020 May;17(5S):S125-S137

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Head trauma is a frequent indication for cranial imaging in children. The majority of accidental pediatric head trauma is minor and sustained without intracranial injury. Well-validated pediatric-specific clinical decision guidelines should be used to identify very low-risk children who can safely forgo imaging. In those who require acute imaging, CT is considered the first-line imaging modality for suspected intracranial injury because of the short duration of the examination and its high sensitivity for acute hemorrhage. MRI can accurately detect traumatic complications, but often necessitates sedation in children, owing to the examination length and motion sensitivity, which limits rapid assessment. There is a paucity of literature regarding vascular injuries in pediatric blunt head trauma and imaging is typically guided by clinical suspicion. Advanced imaging techniques have the potential to identify changes that are not seen by standard imaging, but data are currently insufficient to support routine clinical use. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2020.01.026DOI Listing
May 2020

ACR Appropriateness Criteria Suspected Spine Trauma-Child.

J Am Coll Radiol 2019 May;16(5S):S286-S299

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Choosing the appropriate imaging in children with accidental traumatic spine injuries can be challenging because the recommendations based on scientific evidence at this time differ from those applied in adults. This differentiation is due in part to differences in anatomy and physiology of the developing spine. This publication uses scientific evidence and a panel of pediatric experts to summarize best current imaging practices for children with accidental spine trauma. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2019.02.003DOI Listing
May 2019

Pontine tegmental cap dysplasia with a duplicated internal auditory canal.

Radiol Case Rep 2019 Jul 25;14(7):825-828. Epub 2019 Apr 25.

Department of Radiology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390, USA.

Pontine tegmental cap dysplasia (PTCD) is a rare neurological syndrome that results in a hypoplastic ventral pons, tegmental cap at the dorsal pons, and cranial nerve dysfunction. The most common symptoms are hearing loss and speech problems. We present a case of a 9-month-old male who presented with developmental delay and hypotonia. Magnetic resonance imaging revealed ectopic dorsal transverse pontine fibers and a cap-like protrusion of the dorsal pons. Diffusion tensor imaging showed that the ventral pontine fibers were absent. The cause of PTCD is undiscovered, but proposed hypotheses include dysfunction in axonal guidance, neuronal migration, and ciliary protein function. PTCD is a rare neurological disorder, but the diagnosis can be suggested with MRI using diffusion tensor imaging as an aid.
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http://dx.doi.org/10.1016/j.radcr.2019.04.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6484283PMC
July 2019

Microdeletions excluding YWHAE and PAFAH1B1 cause a unique leukoencephalopathy: further delineation of the 17p13.3 microdeletion spectrum.

Genet Med 2019 07 20;21(7):1652-1656. Epub 2018 Dec 20.

Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA.

Purpose: Brain malformations caused by 17p13.3 deletions include lissencephaly with deletions of the larger Miller-Dieker syndrome region or smaller deletions of only PAFAH1B1, white matter changes, and a distinct syndrome due to deletions including YWHAE and CRK but sparing PAFAH1B1. We sought to understand the significance of 17p13.3 deletions between the YWHAE/CRK and PAFAH1B1 loci.

Methods: We analyzed the clinical features of six individuals from five families with 17p13.3 deletions between and not including YWHAE/CRK and PAFAH1B1 identified among individuals undergoing clinical chromosomal microarray testing or research genome sequencing.

Results: Five individuals from four families had multifocal white matter lesions while a sixth had a normal magnetic resonance image. A combination of our individuals and a review of those in the literature with white matter changes and deletions in this chromosomal region narrows the overlapping region for this brain phenotype to ~345 kb, including 11 RefSeq genes, with RTN4RL1 haploinsufficiency as the best candidate for causing this phenotype.

Conclusion: While previous literature has hypothesized dysmorphic features and white matter changes related to YWHAE, our cohort contributes evidence to the presence of additional genetic changes within 17p13.3 required for proper brain development.
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http://dx.doi.org/10.1038/s41436-018-0358-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6586530PMC
July 2019

ACR Appropriateness Criteria Sinusitis-Child.

J Am Coll Radiol 2018 Nov;15(11S):S403-S412

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Sinusitis is common in children that usually resolves spontaneously. Imaging is not part of the standard of care for initial diagnosis, however may be necessary in cases with persistent or chronic sinusitis to guide surgical intervention, or to rule out intracranial and vascular complications of sinusitis. Computed tomography (CT) and magnetic resonance imaging (MRI) are the leading imaging modalities. In this article, appropriateness in use of imaging modalities are discussed under common/clinically relevant scenarios. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2018.09.029DOI Listing
November 2018

In Reply.

J Oral Maxillofac Surg 2016 Sep 17;74(9):1711-2. Epub 2016 May 17.

Atlanta, GA.

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http://dx.doi.org/10.1016/j.joms.2016.05.003DOI Listing
September 2016

Magnetic Resonance Imaging of Temporomandibular Joints of Children.

J Oral Maxillofac Surg 2016 Sep 28;74(9):1723-7. Epub 2016 Mar 28.

Assistant Professor, Division of Oral and Maxillofacial Surgery, Department of Surgery, Emory University, Atlanta, GA. Electronic address:

Purpose: To identify magnetic resonance imaging (MRI) findings of temporomandibular joints (TMJs) in healthy children.

Materials And Methods: This was a retrospective study of children younger than 18 years undergoing MRI of the head for non-autoimmune indications at Children's Healthcare of Atlanta (Atlanta, GA). Patients with congenital or acquired conditions associated with potential TMJ pathology were excluded. Medical records and MRIs were reviewed to document demographic data. Three neuroradiologists independently recorded specific predetermined MRI findings of the TMJ. The outcome variable was the presence or absence of TMJ findings at MRI. Descriptive and bivariate statistics were used to identify associations (significant at P ≤ .05).

Results: Eighty-seven patients (54 boys, 33 girls; mean age, 11.2 yr) met the inclusion criteria. Seventy percent of TMJs had condylar enhancement, 64% of joints had glenoid marginal synovial enhancement, 56% had condylar margin synovial enhancement, and 19% had condylar T1 hyperintense signal. There were no joints with condylar erosion, condylar volume loss, or effusion. Findings of normal marrow development were decreased condylar enhancement and greater condylar T1 signal with increased age.

Conclusion: This study found a greater than 60% prevalence of synovial enhancement in healthy children. In a healthy child, MRI findings of TMJ synovial enhancement should be carefully correlated with a clinical evaluation.
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http://dx.doi.org/10.1016/j.joms.2016.03.022DOI Listing
September 2016

Utility of double inversion recovery MRI in paediatric epilepsy.

Br J Radiol 2016 3;89(1057):20150325. Epub 2015 Nov 3.

1 Department of Radiology and Imaging Sciences, Neuroradiology Division, Emory University, Atlanta, GA, USA.

Detecting focal abnormalities in MRI examinations of children with epilepsy can be a challenging task given the frequently subtle appearance of cortical dysplasia, mesial temporal sclerosis and similar lesions. In this report, we demonstrate the utility of double inversion recovery MRI in the detection of paediatric epileptogenic abnormalities, promoted primarily by increased lesion conspicuity due to complementary suppression of both cerebrospinal fluid and normal white matter signal.
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http://dx.doi.org/10.1259/bjr.20150325DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4985945PMC
May 2016

White matter injury and the inflammatory response following neonatal cardiac surgery.

Pediatr Cardiol 2015 Jun 20;36(5):942-9. Epub 2015 Jan 20.

Division of Neuroradiology, Department of Radiology and Imaging Sciences, Children's Healthcare of Atlanta, Emory University School of Medicine, 1364 Clifton Rd. NE, EUH B115, Atlanta, GA, 30322, USA,

White matter injury (WMI) is a known complication following neonatal heart surgery in term infants. In preterm infants, WMI has been associated with the degree of systemic inflammation. It is not known whether inflammation is an important mechanism of WMI as documented by magnetic resonance imaging (MRI) following neonatal heart surgery with cardiopulmonary bypass. Term neonates with congenital heart disease were enrolled in a prospective study with postoperative MRI. White matter injury was recorded by the number of T1 hyperintense foci with >5 foci consistent with significant WMI. Eleven candidate cytokine markers (INF-gamma, TNF-alpha, IL-1 beta, IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, IL-12, and IL-13) were assayed preoperatively and daily for 5 days following surgery. Multiple clinical factors were recorded and correlated with WMI. Ninety-two subjects were enrolled in the study. The median age at surgery was 5 days (interquartile range 4-7 days). Compared with the presurgery level, there were statistically significant increases (p < 0.005) for 8 out of 11 inflammatory markers. In all, 64 postoperative MRIs were performed. No significant correlation was detected between WMI and clinical variables or inflammatory markers assessed immediately postoperative and on postoperative days 1 and 3, with exception of IL-1 beta on postoperative day 1. WMI correlates poorly with the systemic inflammatory response after congenital heart surgery and a number of herein measured clinical factors. WMI following neonatal heart surgery is a complex, still incompletely understood phenomenon that warrants continued investigation.
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http://dx.doi.org/10.1007/s00246-015-1104-xDOI Listing
June 2015

Transverse sinus stenosis in adult patients with Chiari malformation type I.

AJR Am J Roentgenol 2014 Oct;203(4):890-6

1 Department of Radiology and Imaging Sciences, Emory University School of Medicine, BG22, 1364 Clifton Rd NE, Atlanta, GA 30322.

Objective: Transverse sinus stenosis is an imaging finding very highly associated with elevated intracranial pressure (ICP). Patients with the Chiari I malformation may potentially have elevated ICP due to impairment of CSF flow at the foramen magnum. This study evaluated whether patients with Chiari I malformation have transverse sinus stenosis and other imaging findings indicative of elevated ICP.

Materials And Methods: Thirty patients with Chiari I malformation treated surgically and 76 control subjects were identified retrospectively. All control subjects and all patients with Chiari I malformation (preoperatively) underwent standardized contrast-enhanced brain MRI including a contrast-enhanced 3D T1-weighted sequence from which curved reformats of the transverse sinuses were generated. Two different readers blinded to the diagnosis then independently evaluated these curved reformats for severity of transverse sinus stenosis. Orbital and skull-base findings previously described in association with elevated ICP were also evaluated. Frequency of MRI findings between the two groups was compared.

Results: Patients with Chiari I malformation had significantly greater frequency of unilateral or bilateral transverse sinus stenosis than did control subjects (p < 0.001). There was complete interreader agreement on presence or absence of transverse sinus stenosis by patient (κ = 1.0 [95% CI, 0.89-1.0]). Logistic regression analysis controlling for age, sex, and body mass index found that transverse sinus stenosis significantly predicted Chiari I malformation versus control status (odds ratio, 11.2 [95% CI, 2.1-59.0]; p = 0.004) but that no other features were significantly associated with the Chiari I malformation. Patients with Chiari I malformation who had transverse sinus stenosis had significantly greater pituitary flattening than did those without transverse sinus stenosis (p = 0.02).

Conclusion: Patients with Chiari I malformation have higher likelihood of transverse sinus stenosis, which may reflect associated elevated ICP.
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http://dx.doi.org/10.2214/AJR.14.12528DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4277891PMC
October 2014

The role of magnetic resonance imaging in diagnosing optic nerve hypoplasia.

Am J Ophthalmol 2014 Dec 13;158(6):1164-1171.e2. Epub 2014 Aug 13.

Department of Ophthalmology, Division of Pediatric Ophthalmology and Strabismus, Emory University School of Medicine, Emory Eye Center, Atlanta, Georgia.

Purpose: To establish objective lower limits of normal optic nerve (ON) size in children based on high-resolution orbital magnetic resonance imaging (MRI).

Design: Case-control study of patients with ON hypoplasia vs normal controls.

Methods: A neuroradiologist made 4 measurements of each ON at 2 locations (5 mm posterior to the optic disc and just posterior to the optic canal) in patients with ON hypoplasia and controls aged 0-17 years from an academic eye center and children's hospital. Primary analyses were performed using mixed linear models.

Results: Measurements were made in 26 cases of clinically confirmed ON hypoplasia and 31 controls (median age: ON hypoplasia, 1 year; controls, 5.5 years). Nine of 26 cases (35%) and 19 of 31 controls (61%) underwent high-resolution T2-weighted imaging of the orbits. Mean ON diameter was 1.36 mm (95% CI: 1.19-1.54; P < .001) smaller for clinically hypoplastic ONs than for controls. Optic nerve diameter increased by 0.05 mm per year of age (95% CI: 0.03-0.07; P < .001). A lower bound to the 95% prediction interval for normal optic nerves was (2.24 mm + 0.052 × [age in years]) mm and excluded all but 1 case.

Conclusions: Age was independently associated with normal ON size by MRI and should be taken into consideration when evaluating ON hypoplasia, based on MRI criteria. We have provided a formula to assist clinicians in objectively determining if ON hypoplasia is present.
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http://dx.doi.org/10.1016/j.ajo.2014.08.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4252492PMC
December 2014

An imaging approach to diffuse white matter changes.

Radiol Clin North Am 2014 Mar 28;52(2):263-78. Epub 2013 Dec 28.

Division of Neuroradiology, Department of Radiology and Imaging Sciences, Emory University School of Medicine, 1364 Clifton Road Northeast, Room D125A, Atlanta, GA 30322, USA.

White matter disorders represent a large, heterogeneous group of disorders that span the continuum of congenital metabolic disorders to acquired processes, such as chronic ischemic microvascular white matter disease. Magnetic resonance imaging has dramatically revolutionized the diagnostic evaluation of patients with these disease processes.
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http://dx.doi.org/10.1016/j.rcl.2013.11.006DOI Listing
March 2014

Performance of spin-echo and gradient-echo T1-weighted sequences for evaluation of dural venous sinus thrombosis and stenosis.

AJR Am J Roentgenol 2013 Jul;201(1):162-9

Department of Radiology and Imaging Sciences, Emory University Hospital and Emory University School of Medicine, 1364 Clifton Rd NE, BG22, Atlanta, GA 30322, USA.

Objective: Dural venous sinus abnormalities are clinically important but can potentially be overlooked using various MRI techniques. This study evaluates the diagnostic accuracy of spin-echo (SE) T1-weighted imaging, 3D gradient-recalled echo (GRE) T1-weighted imaging, and contrast-enhanced MR venography (MRV) for the detection of dural venous sinus thrombosis and transverse sinus (TS) stenosis.

Materials And Methods: Seventy-three patients underwent MRI evaluation with unenhanced and contrast-enhanced axial SE T1-weighted imaging, contrast-enhanced sagittal 3D GRE T1-weighted imaging, and contrast-enhanced MRV sequences. Three neuroradiologists each evaluated these 219 total datasets in a randomized blinded fashion for the presence or absence of TS stenosis and for dural venous sinus thrombosis in each of 10 venous sinus segments (730 total segments). Diagnostic performance characteristics and kappa statistics were calculated for each technique.

Results: Thirteen patients (37 segments) had suspected dural venous sinus thrombosis by one or more readers; of those 13 patients, nine (23 segments) were thought to have definite thrombosis on contrast-enhanced MRV. Compared with contrast-enhanced MRV, the positive predictive value (PPV) and negative predictive value (NPV) for thrombosis were 60% and 97%, respectively, for both unenhanced and contrast-enhanced SE T1-weighted imaging and 100% and 98% for 3D GRE T1-weighted imaging. Kappa values calculated per venous segment were as follows: 0.41 for SE T1-weighted imaging, 0.72 for 3D GRE T1-weighted imaging, and 0.95 for contrast-enhanced MRV. Thirty patients (58 segments) had TS stenosis suspected by one or more readers; of those 30 patients, TS stenosis was deemed to be definite on contrast-enhanced MRV in 25 patients (50 segments). Compared with contrast-enhanced MRV, the PPV and NPV were 75% and 80%, respectively, for SE T1-weighted imaging and 91% and 92% for 3D GRE T1-weighted imaging for the detection of stenosis. Kappa values calculated per patient were -0.038 for SE T1-weighted imaging, 0.58 for 3D GRE T1-weighted imaging, and 0.98 for contrast-enhanced MRV.

Conclusion: Contrast-enhanced 3D GRE T1-weighted imaging is superior to SE T1-weighted imaging for the detection of dural venous sinus thrombosis and TS stenosis but does not substitute for dedicated MRV. Hyperintensity on unenhanced SE T1-weighted imaging is unreliable for the detection of dural venous sinus thrombosis.
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http://dx.doi.org/10.2214/AJR.12.9095DOI Listing
July 2013

Pontine tegmental cap dysplasia: the neurotologic perspective.

Otolaryngol Head Neck Surg 2011 Dec 25;145(6):992-8. Epub 2011 Jun 25.

Department of Radiology, Division of Neuroradiology, Emory University School of Medicine and Children's Healthcare of Atlanta, Georgia 30322, USA.

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 resonance imaging (MRI) of the temporal bones and brain. High-resolution computed tomography (CT) scanning of the temporal bones was performed in 2 patients. Imaging findings of the brain, the presence and course of resolvable cranial nerves, the membranous labyrinth, and internal auditory canals were reviewed. Clinical data were reviewed.

Results: All patients demonstrated typical brain characteristics of PTCD. Mild, bilateral cochlear dysplasia was noted in 2, and all had a normal vestibular labyrinth. The cochleovestibular nerves were universally absent bilaterally. The facial nerves were subjectively deficient bilaterally in 1 patient, unilaterally in the second patient, and normal in the third. An accessory canal for the seventh cranial nerve, referred to as a duplicated internal auditory canal, was present in all patients. Auditory brainstem response testing revealed profound bilateral sensorineural hearing loss in all of the patients; none suffered facial weakness. A single patient underwent bilateral cochlear implantation with only minimal response.

Conclusion: The authors report 3 cases of PTCD with emphasis on imaging of the seventh and eighth cranial nerves and clinical neurotologic findings. All patients manifested duplicated internal auditory canals, a previously unreported finding in PTCD. Bilateral profound sensorineural hearing loss is due to absence of the cochleovestibular nerve. Prognosis for cochlear implantation is poor.
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http://dx.doi.org/10.1177/0194599811412729DOI Listing
December 2011

Contrast use at low field: a review.

Top Magn Reson Imaging 2003 Oct;14(5):360-4

Department of Radiology, Scott and White Clinic and Hospital, Texas A&M University Health Science Center, Temple, Texas, USA.

The use of gadolinium-based contrast agents for magnetic resonance imaging at low fields (
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http://dx.doi.org/10.1097/00002142-200310000-00002DOI Listing
October 2003

Magnetic resonance imaging of the brain in glutaric acidemia type I: a review of the literature and a report of four new cases with attention to the basal ganglia and imaging technique.

Invest Radiol 2003 Aug;38(8):489-96

Texas A&M University College of Medicine, Temple, TX, USA.

Rationale And Objectives: In glutaric acidemia type I (GA I), a pediatric neurometabolic disease that may be mistaken for nonaccidental trauma, expeditious detection is critical as early treatment may substantially improve psychomotor dysfunction. In this study, we examine in depth the magnetic resonance (MR) findings, with special attention to the basal ganglia, in 4 new cases and compare the findings with those described in the literature.

Methods: MR studies of 4 children, diagnosed to have GA I via cultured fibroblast enzyme studies or urine metabolite assays, were performed on a 1.5 T system in the axial plane using spin echo T(1)-weighted, fast spin echo T(2)-weighted, and fluid-attenuated inversion recovery (FLAIR) technique. Three of 4 patients were followed with serial exams to document temporal evolution of the disease.

Results: On T(2)-weighted images, abnormal increased signal intensity was seen in both the putamen and globus pallidus in all cases. However, in contradistinction to cases reported in the literature, involvement of the caudate nucleus was minimal or absent even on serial MR exams. In children 15 months and older, FLAIR improved recognition of basal ganglia and white matter abnormalities. The previously described widened cerebrospinal fluid spaces anterior to the temporal lobes, increased T(2)-weighted signal intensity in the periventricular white matter, and widened sylvian fissures characteristic of GA I were noted in all patients.

Conclusions: Abnormalities of the caudate nucleus are not a prominent presentation of these patients and the absence of this finding should not exclude a diagnosis of GA I. FLAIR scans, as an adjunct to more conventional T(1)- and T(2)-weighted sequences, can play an important role in children 15 months or older despite immature myelination in these patients.
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http://dx.doi.org/10.1097/01.rli.0000080405.62988.f6DOI Listing
August 2003