Neil R. Miller's research while affiliated with Johns Hopkins University and other places

Publications (390)

Article
Full-text available
Idiopathic intracranial hypertension (IIH) has an increasing incidence worldwide over the past decade, with a high economic burden on patients and society. Up to 10% of patients with IIH have progressive visual decline requiring an invasive intervention (including cerebrospinal fluid shunting, cerebral dural sinus stenting, or optic nerve sheath fe...
Article
Background: To date, deep learning-based detection of optic disc abnormalities in color fundus photographs has mostly been limited to the field of glaucoma. However, many life-threatening systemic and neurological conditions can manifest as optic disc abnormalities. In this study, we aimed to extend the application of deep learning (DL) in optic d...
Article
A case of atypical blepharospasm with oromandibular dystonia is presented in a patient found to have cerebral amyloid angiopathy on magnetic resonance imaging and a shared mechanism is discussed.
Chapter
Traumatic optic neuropathy (TON) is a clinical diagnosis, with imaging frequently adding clarification to the full nature/extent of the lesion(s) in question. Each pattern of injury carries its own unique prognosis and theoretical best treatment; however, the optimum management of patients with TON remains unclear. Observation, steroids, surgical m...
Article
Full-text available
Nonarteritic anterior ischemic optic neuropathy (NAION) commonly causes sudden optic nerve (ON)-related vision loss. The rodent NAION model (rAION) closely resembles NAION in presentation and physiological responses. We identified early rAION-associated optic nerve head (ONH) inflammatory gene expression responses and the anti-inflammatory prostagl...
Article
Background: Cavernous malformations (CMs) of the optic nerve and chiasm are extremely rare, accounting for less than 1% of all intracranial CMs. Acute, subacute, or progressive visual loss from CM may occur with or without hemorrhage. Prompt surgical excision of the CM offers the best hope to improve or stabilize vision. Given its rarity, optic ne...
Article
Full-text available
The rodent model of nonarteritic anterior ischemic optic neuropathy (rNAION) is similar in many of its pathophysiological responses to clinical NAION. Like human NAION, there is significant variability in the severity of the lesion produced, and little is known of the parameters associated with rNAION induction severity or if pre- or early post-ind...
Article
A host of different types of direct and indirect, primary and secondary injuries can affect different portions of the optic nerve(s). Thus, in the setting of penetrating as well as nonpenetrating head or facial trauma, a high index of suspicion should be maintained for the possibility of the presence of traumatic optic neuropathy (TON). TON is a cl...
Preprint
Full-text available
The rodent model of nonarteritic anterior ischemic optic neuropathy (rNAION) is similar in many of its pathophysiological responses to clinical NAION. However, little is known of the parameters associated with rNAION induction severity and if pre- or early post-induction biomarkers can be identified that enable prediction of lesion severity and ult...
Article
Posterior globe flattening has been well-documented in astronauts both during and after long-duration space flight (LDSF) and has been observed as early as 10 days into a mission on the International Space Station. Globe flattening (GF) is thought to be caused by the disc centred anterior forces created by elevated volume and/or pressure within the...
Article
For many years, it was unclear whether Duane retraction syndrome was a myopathic or neuropathic condition. This article describes the direct and indirect contributions of William F. Hoyt, MD, to the eventual determination that the condition is caused by a congenital absence of the sixth nerve combined with innervation of the lateral rectus muscle b...
Article
Objective To compare the diagnostic performance of an artificial intelligence deep learning system with that of expert neuro‐ophthalmologists in classifying optic disc appearance. Methods The deep learning system was previously trained and validated on 14,341 ocular fundus photographs from 19 international centers. The performance of the system wa...
Article
Background: Nonophthalmologist physicians do not confidently perform direct ophthalmoscopy. The use of artificial intelligence to detect papilledema and other optic-disk abnormalities from fundus photographs has not been well studied. Methods: We trained, validated, and externally tested a deep-learning system to classify optic disks as being norm...
Article
Background: En face optical coherence tomography (OCT) uses the data acquired during OCT of the optic disc, which typically is used to determine measurements of the peripapillary retinal nerve fiber layer (ppRNFL), to generate a coronal composite fundus image rather than a cross-sectional image. En face OCT has been reported to identify retinal ch...
Chapter
With the growth of basic science studies, clinical research and updated diagnostic and imaging modalities, the subspecialty of neuro-ophthalmology has continued to evolve. Clinical updates include novel treatment approaches, new disease entities and improved understanding of the underlying pathophysiology for many well-known neuro-ophthalmic diagno...
Article
Full-text available
We performed two experiments to investigate how monocular vision and a monocular generalized reduction in vision (MRV) impact driving performance during racing. A total of 75 visually normal students or professional racing drivers, were recruited for the two experiments. Driving performance was evaluated under three visual conditions: normal vision...
Article
Importance: Optic nerve dysfunction is a common feature of different diseases. The pathophysiology is not yet fully understood. Background: This study describes five patients with optic nerve sheath compartment syndrome and contributes to the hypothesis that impaired cerebrospinal fluid flow can play a role in the development of optic nerve dysf...
Article
We read with interest the article by Ross et al that discussed 27 patients with diplopia and giant cell arteritis (GCA). We contribute the perspective of 40 additional patients with diplopia and biopsy-proven GCA, based on our recent retrospective study of 1,666 subjects who underwent consecutive temporal artery biopsy for suspected GCA at 12 medic...
Article
Background: Deep learning (DL) has demonstrated human expert levels of performance for medical image classification in a wide array of medical fields, including ophthalmology. In this article, we present the results of our DL system designed to determine optic disc laterality, right eye vs left eye, in the presence of both normal and abnormal opti...
Article
Purpose: In a recent study, it was found that, although intrinsic midbrain signal abnormality (IMSA) on MRI is associated with Parinaud's syndrome (PS) in patients with pineal gland masses (PM), it had no predictive value with respect to resolution of PS. We sought to compare the PM and non-pineal etiologies (NPE) of PS by reviewing imaging featur...
Article
Full-text available
Purpose The association between MRI findings in patients with pineal lesions and the presence or absence of Parinaud’s syndrome (PS) remains poorly described. We sought to better understand what MRI characteristics of a pineal lesion make PS more likely. Can these features predict prognosis for clinical resolution? Based on the anatomical relations...
Article
Purpose To develop and validate neural network (NN) vs logistic regression (LR) diagnostic prediction models in patients with suspected giant cell arteritis (GCA). Design: Multicenter retrospective chart review. Methods An audit of consecutive patients undergoing temporal artery biopsy (TABx) for suspected GCA was conducted at 14 international med...
Chapter
Optic neuritis may be caused by a variety of infectious, demyelinating, and inflammatory disorders. Following an attack of acute demyelinating optic neuritis (ON), there is a risk of developing multiple sclerosis (MS) especially if one or more periventricular demyelinating white-matter lesions is seen on magnetic resonance imaging (MRI) of the brai...
Article
Background/Aim Fractionated conformal radiotherapy (FCRT) is now used to treat vision-threatening optic nerve sheath meningioma (ONSM), but long-term efficacy and safety data are lacking; the purpose of this study was to assess these key data. Methods This is a retrospective chart review with prospective follow-up of adult patients treated with FC...
Article
Each year, more than 700 physicians from around the world register for, and more than 600 participate in, the United States Ophthalmology Match, vying for approximately 460 residency positions.¹ The average participant applies to about 70 programs, many of which receive more than 400 applications for 5 to 8 positions.¹ The buyer’s market for most o...
Article
Full-text available
Objective This study aimed to determine if there is any association between the subject of pre-residency research publications (PRPs) and the academic settings in which they are produced with post-residency academic productivity among graduates from an academic ophthalmology residency program. Design, Setting, Participants This is a cross-sectional...
Chapter
A variety of different types of direct and indirect and primary and secondary injuries can affect different portions of the optic nerve(s), producing the condition known as traumatic optic neuropathy (TON). Thus, in the setting of penetrating as well as non-penetrating head or facial trauma, a high index of suspicion should be maintained for the po...
Chapter
Thyroid eye disease (TED) is an autoimmune phenomenon that involves both antigen-dependent and antigen-independent pathways. It occurs in about 20 % of patients with dysthyroidism and can develop in patients who are hyperthyroid, hypothyroid, or euthyroid. The fundamental change that underlies most clinical manifestations of TED is enlargement or e...
Article
Importance: This study offers a new approach for the quantification of CSF-dynamics. Background: Non-invasive method to quantify the CSF-dynamics in the subarachnoid-space of the optic nerve is highly desirable. The aim of the study was to measure slow-flow CSF-velocities in healthy controls and normal-tension glaucoma patients between the intra...
Article
Cavernous angiomas of the cranial nerves are extremely rare, and those of the oculomotor (third) cranial nerve are rarer still, with no previous cases having been reported in the literature. We report herein the first case of a presumed cavernous angioma involving the subarachnoid portion of the left third nerve presenting as an acute left third ne...
Article
Gorham-Stout disease (GSD) is a rare angiomatous disorder characterized by osteolysis. We report the case of a 66-year-old woman who developed sudden left eye proptosis and facial pain. Neuroimaging demonstrated a destructive mass involving the roof and lateral wall of the left orbit with adjacent abnormal orbital soft tissue. An initial biopsy of...
Article
To the Editor I read with interest the article by Fang et al¹ describing the incidence and etiologies of acquired third nerve palsy among people living in Olmsted County, Minnesota. The authors correctly emphasize that although compressive lesions have a higher likelihood of pupil involvement, the lack of pupil involvement does not rule out a compr...
Article
Full-text available
Cerebrofacial arteriovenous metameric syndrome (CAMS) is a recent classification of vascular malformations that encompasses a spectrum of phenotypic expression involving arteriovenous malformations (AVMs) of the cerebral, orbital, and facial region. Recognizing the embryologic basis of CAMS is important for diagnosing other AVMs along the same meta...
Article
Cerebral high-grade astrocytomas typically result in poor outcomes for patients because their invasive nature makes resection difficult and because their resistance to chemoradiation usually leads to local recurrence. Herein, we report a case of acute vision loss with a dramatic presentation due to a recurrent cerebral malignant glioma.
Article
Background: Several ophthalmic findings including optic disc swelling, globe flattening and choroidal folds have been observed in astronauts following long-duration space flight. The authors now report asymmetric choroidal expansion, disc swelling and optic disc morphologic changes in a 45-year-old astronaut which occurred during long-duration spa...
Article
Purpose: Diabetic patients have a greater risk of nonarteritic anterior ischemic optic neuropathy (NAION) than nondiabetic patients. We compare visual outcomes, prevalence of bilateral/sequential ION, and predictors of visual outcomes in NAION between diabetic and nondiabetic patients. Design: Case-control study. Participants: All 231 patients...
Article
Purpose: To reassess the prevalence and incidence of post-cataract surgery optic neuropathy (PCSON) in the modern era. Design: Retrospective cohort study. Setting: Single center, tertiary care practice. Study population: All patients with a diagnosis of non-arteritic anterior ischemic optic (NAION) seen in the Wilmer Eye Network system betwe...
Article
In this issue of JAMA Ophthalmology, Kilduff et al¹ report the largest published observational series to date of the use of alleviating maneuvers by patients with benign essential blepharospasm (BEB) or hemifacial spasm (HFS). The authors found that 39 of 74 patients with BEB (52.7%) and 25 of 56 patients with HFS (44.6%) used alleviating maneuvers...
Article
Full-text available
Purpose: To evaluate quantitatively the relationship between the displacement of anterior visual pathway structures by pituitary tumors and visual field damage with the goal of improving diagnosis and management. Methods: Subjects had pituitary macroadenomas and both magnetic resonance imaging (MRI) and static perimetry. Neuroradiologists measur...
Article
Full-text available
Introduction: Polyamidoamine dendrimer nanoparticles (~ 4 nanometers) are inert polymers that can be linked to biologically active compounds. These dendrimers selectively target and accumulate in inflammatory cells upon systemic administration. Dendrimer-linked compounds enable sustained release of therapeutic compounds directly at the site of dam...
Article
Full-text available
Purpose To determine the relationship between peripapillary retinal nerve fiber layer (PRNFL) swelling and eventual PRNFL atrophy, and between PRNFL swelling/atrophy and neural function, in a nonhuman primate model of nonarteritic anterior ischemic optic neuropathy (pNAION). Methods pNAION was induced in five normal, adult male rhesus monkeys by l...
Article
Full-text available
Purpose: Benign essential blepharospasm (BEB) generally is considered a disorder of adults; however, it rarely can present in childhood or adolescence. The main purpose of this study was to determine the prevalence of BEB in children and adolescents. Our research question was whether blepharospasm is seen in children or adolescents as well as in t...
Article
Full-text available
Objectives The purpose of this paper is to measure the change in body weight after a 6‐month telephone‐based weight loss intervention in overweight and obese subjects with idiopathic intracranial hypertension (IIH) and mild visual loss randomized to receive either acetazolamide or placebo. Methods One hundred sixty‐five subjects with IIH, aged 29....
Article
Background context: Perioperative visual loss (POVL) after spinal deformity surgery is an uncommon but severe complication. Data on the incidence and risk factors of this complication after corrective surgery in the pediatric population are limited. Purpose: The present study aimed to investigate nationwide estimates of POVL after corrective sur...
Article
Full-text available
Purpose: Ranibizumab, a vascular endothelial growth factor-antagonist, is said to be neuroprotective when injected intravitreally in patients with nonarteritic anterior ischemic optic neuropathy (NAION). We evaluated the efficacy of a single intravitreal (IVT) injection of ranibizumab in a nonhuman primate model of NAION (pNAION). Methods: We in...
Article
The authors present three patients with idiopathic exercise-induced esotropia. The history, physical findings, and testing that led to the diagnosis are discussed. To the authors' knowledge, this is the largest series of patients with this rare condition, with this series representing half of reported patients in the literature. It is hoped that a...
Data
Video Legend: Thirteen year old boy presented after several years of forceful squeezing of his eyelids that is involuntary. Triggers include bright lights and objects coming toward his face. The patient controls the involuntary squeezing by touching his lateral eyebrow and eyelid. He is still having symptoms but has had improvement using FL-41, tak...
Article
Objective: The objective of this study was to test the hypothesis that bitemporal hemianopsia (BHA) is the most common visual field (VF) defect in patients with pituitary macroadenoma and to assess the degree of optic pathway compression necessary to produce visual defects. Materials and methods: We reviewed the MRI findings and medical records...
Article
A 67-year-old man developed jaw claudication followed by loss of vision in the left eye caused by anterior ischemic optic neuropathy (AION). An erythrocyte sedimentation rate was normal, but C-reactive protein was slightly elevated. Although the patient had no evidence of a right optic neuropathy, magnetic resonance imaging (MRI) revealed bilateral...
Article
Ewing sarcoma (ES) is a part of a larger family of round blue-cell tumors that occasionally manifest as osseous or extraosseous lesions adjacent to or within the central nervous system (CNS). Although a large body of literature exists on ES of bone, data are lacking on tumors with cranial or spinal components that affect the CNS. Moreover, primary...
Article
Full-text available
Sivashakthi Kanagalingam,1–3 Neil R Miller1–31Department of Ophthalmology, 2Department of Neurology, 3Department of Neurosurgery, The Johns Hopkins Hospital, Baltimore, MD, USAAbstract: Horner syndrome consists of unilateral ptosis, an ipsilateral miotic but normally reactive pupil, and in some cases, ipsilateral facial anhidrosis, all resulting fr...
Article
Ischemic optic neuropathies (IONs) describe a group of diseases that specifically target the optic nerve and result in sudden vision loss. These include nonarteritic and arteritic anterior ischemic optic neuropathy (NAION and AAION) and posterior ischemic optic neuropathy (NPION, APION). Until recently, little was known of the mechanisms involved i...
Article
Over the past 10 years, transverse sinus stenting has grown in popularity as a treatment for idiopathic intracranial hypertension. Although promising results have been demonstrated in several reported series, the vast majority of patients in these series have been treated on an elective basis rather than in the setting of fulminant disease with acu...
Article
Purpose: Prostaglandin J₂ (PGJ₂) is neuroprotective in a murine model of nonarteritic anterior ischemic optic neuropathy (NAION). After assessing for potential toxicity, we evaluated the efficacy of a single intravitreal (IVT) injection of PGJ₂ in a nonhuman primate model of NAION (pNAION). Methods: We assessed PGJ₂ toxicity by administering it...
Article
: A 62-year-old man developed bilateral blurred vision associated with bilateral optic disc swelling shortly after receiving his third dose of the tumor necrosis factor-alpha (TNF-α) inhibitor golimumab, that he took for psoriatic arthritis. An extensive assessment including magnetic resonance imaging, lumbar puncture, and serologies was negative....
Article
Full-text available
End stage kidney disease is a well-known complication of methylmalonic acidemia (MMA), and can be treated by dialysis, kidney transplant, or combined kidney-liver transplant. While liver and/or kidney transplantation in MMA may reduce the risk of metabolic crisis and end-organ disease, it does not fully prevent disease-related complications. We per...
Article
Full-text available
To compare visual outcomes in pseudotumour cerebri (PTC) patients who underwent either optic nerve sheath fenestration (ONSF) or cerebrospinal fluid (CSF) diversion (shunt) for treatment of visual loss from papilloedema. Comparative case series of thirty-three patients (33 eyes) who underwent either ONSF (14 patients) or CSF shunt (19 patients) for...
Article
Full-text available
Orbital meningiomas can be classified as primary optic nerve sheath meningiomas (ON), primary intraorbital ectopic meningiomas (Ob), and spheno-orbital meningiomas (Sph-Ob) based on anatomic site. Single-nucleotide polymorphism (SNP)-based array analysis with the Illumina 300K platform was performed on formalin-fixed, paraffin-embedded tissue from...
Article
Purpose: To improve the neurological hemifield test (NHT) using visual field data from both eyes to detect and classify visual field loss caused by chiasmal or postchiasmal lesions. Methods: Visual field and clinical data for 633 patients were divided into a training set (474 cases) and a validation set (159 cases). Each set had equal numbers of...