N R Miller

Johns Hopkins University, Baltimore, MD, USA

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Publications (74)252.15 Total impact

  • Article: Septo-optic dysplasia.
    M A Fard, W Y Wu-Chen, B L Man, N R Miller
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    ABSTRACT: Septo-optic dysplasia is a rare disorder characterized by optic nerve hypoplasia; midline developmental defects including agenesis of the septum pellucidum, thinning or absence of the corpus callosum, or both; and deficiencies of pituitary hormones. The majority of cases are sporadic but rare familial cases occur. The clinical manifestations include poor visual function in one or both eyes, developmental delay, seizures, sleep disturbances, and precocious puberty. A life-long multidisciplinary approach is crucial in the management of these patients to optimize their growth and development and to help them lead as normal lives as possible.
    Pediatric endocrinology reviews: PER 09/2010; 8(1):18-24.
  • Article: Papilloedema and vision loss with elevated cerebrospinal fluid protein in a patient with systemic lupus erythematosus: diagnosis and management challenges.
    The British journal of ophthalmology 01/2010; 94(1):131-42. · 2.92 Impact Factor
  • Article: Delayed progressive visual loss following wrapping of bilateral clinoidal aneurysms: recovery of vision and improvement in neuroimaging during corticosteroid treatment.
    British Journal of Ophthalmology 01/2006; 89(12):1666-8. · 2.90 Impact Factor
  • Article: Cook detachable coil embolization of a symptomatic, isolated orbital arteriovenous fistula via a superior ophthalmic vein approach.
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    ABSTRACT: Isolated arteriovenous fistulas of the posterior orbit occur with exceptional rarity, and their evaluation and management are not well characterized. We describe the clinical presentation and treatment of a spontaneous arteriovenous fistula of the right posterior orbit via a superior ophthalmic vein approach for embolization using platinum detachable coils.
    Neuroradiology 02/2005; 47(1):62-5. · 2.82 Impact Factor
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    Article: Long term visual and neurological prognosis in patients with treated and untreated cavernous sinus aneurysms.
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    ABSTRACT: To determine the long term visual and neurological outcome of patients diagnosed with cavernous sinus aneurysms (CCAs). Prospective follow up for at least five years or until death of 31 retrospectively recruited patients (27 women, 4 men) with treated and untreated CCAs. There were 40 aneurysms in all. Mean age at diagnosis was 60.4 years (range 25 to 86; median 64). The most common symptoms were diplopia (61%), headache (53%), and facial or orbital pain (32%). Fifteen patients (48%) were diagnosed after they developed cranial nerve pareses, four (13%) after they developed carotid-cavernous sinus fistulas (CCFs), and 12 (39%) by neuroimaging studies done for unrelated symptoms. Twenty one patients (68%) had treatment to exclude the aneurysm from circulation, 10 shortly after diagnosis and 11 after worsening symptoms. Immediate complications of treatment occurred in six patients and included neurological impairment, acute ophthalmoparesis, and visual loss. Ten patients (32%) were observed without intervention. Over a mean (SD) follow up period of 11.8 (7.7) years, eight had improvement in symptoms, five remained stable, and eight deteriorated. Among the 10 patients followed without intervention, none improved spontaneously, three remained stable, and seven worsened. Most treated patients in this series improved or remained stable after treatment, but none improved without treatment. The long term prognosis for treated cases is relatively good, with most complications occurring immediately after the procedure. Endovascular surgery has decreased the morbidity and mortality of treatment so should be considered for any patient with a CCA.
    Journal of Neurology Neurosurgery &amp Psychiatry 07/2004; 75(6):863-7. · 4.76 Impact Factor
  • Article: Reversible bilateral internuclear ophthalmoplegia associated with FK506.
    M M Lai, J B Kerrison, N R Miller
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    ABSTRACT: A 50 year old man developed tonic-clonic seizures while receiving cyclosporin A after orthotopic cardiac transplant. The seizures resolved after cessation of cyclosporin A. Thirteen months later, he developed diplopia from bilateral internuclear ophthalmoplegia while receiving intravenous FK506. A temporal association was found between his symptoms and the serum FK506 concentrations. Withdrawal of the intravenous FK506 led to prompt resolution of the bilateral internuclear ophthalmoplegia.
    Journal of Neurology Neurosurgery &amp Psychiatry 06/2004; 75(5):776-8. · 4.76 Impact Factor
  • Article: A controlled study comparing visual function in patients treated with vigabatrin and tiagabine.
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    ABSTRACT: Vigabatrin treatment is frequently associated with irreversible retinal injury and produces retinal electrophysiological changes in nearly all patients. Concern has been raised that tiagabine and other antiepilepsy drugs (AEDs) that increase brain gamma-aminobutyric acid (GABA) might produce similar electrophysiological and clinical changes in visual function. The study compared visual function between groups of patients with epilepsy treated long term with tiagabine, vigabatrin, and patients treated with other AEDs. A cross sectional study comparing visual acuity, colour vision, static and kinetic perimetry, and electroretinograms between groups of patients treated with tiagabine, vigabatrin, and other AEDs (control patients). Patients were adults receiving stable AED treatment for >6 months. Vigabatrin treated patients had marked visual field constrictions in kinetic perimetry (mean radius 39.6 degrees OD, 40.5 degrees OS), while tiagabine patients had normal findings (mean 61 degrees OD, 62 degrees OS) (differences OD and OS, p=0.001), which were similar to epilepsy control patients (mean 60 degrees OD, 61 degrees OS). Vigabatrin patients had abnormal electroretinographic photopic B wave, oscillatory, and flicker responses, which correlated with visual field constrictions. These electroretinographic responses were normal for tiagabine patients and control patients. Patients were treated with vigabatrin for a median of 46 months compared with 29 months for tiagabine. Patients taking other AEDs that may change brain GABA had normal visual function. Unlike vigabatrin, tiagabine treatment is associated with normal electroretinography and visual fields and ophthalmological function similar to epilepsy control patients. Differences between vigabatrin and other GABA modulating AEDs in retinal drug concentrations and other effects might explain why tiagabine increases in GABA reuptake do not cause retinal injury.
    Journal of Neurology Neurosurgery &amp Psychiatry 03/2003; 74(3):339-43. · 4.76 Impact Factor
  • Article: Recurrent ectopic craniopharyngioma.
    S K Freitag, N R Miller, H Brem
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    ABSTRACT: A 66-year-old woman developed an asymptomatic mass in the right frontal lobe 5 years after undergoing a right frontal craniotomy and removal of a craniopharyngioma. The mass progressively enlarged over the next 3 years, during which time it became multiloculated and partially cystic. Repeat craniotomy was performed 8 years after the original operation, at which time the mass was found to be an ectopic craniopharyngioma. The lesion probably resulted from seeding of tumour cells along the surgical tract at the time of the initial surgery.
    British Journal of Neurosurgery 01/2002; 15(6):511-3. · 0.88 Impact Factor
  • Article: Clinical spectrum of posterior ischemic optic neuropathy.
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    ABSTRACT: To describe the systemic and visual characteristics and prognosis in patients with posterior ischemic optic neuropathy (PION). Observational case series. Retrospective chart review in a multicenter setting. Seventy-two patients (98 eyes) with a clinical diagnosis of PION. Co-morbid systemic diseases and visual function were recorded at both initial presentation and after mean visual follow-up of 4.1 years and systemic follow-up of 5.4 years. PION occurred in three main settings: in the perioperative period following a variety of surgical procedures (28 patients), associated with giant cell (temporal) arteritis (6 patients), and associated with nonarteritic systemic vascular disease (38 patients). Patients with perioperative and arteritic PION were more likely to have severe, bilateral visual loss that did not improve. Among eyes with nonarteritic PION, 34% experienced improvement in vision, 28% remained stable, and 38% worsened. Among patients with nonarteritic PION, carotid artery disease and a history of stroke (with or without carotid artery disease) were both associated with a statistically significant increased risk of poor final visual outcome. There are three distinct subtypes of PION: perioperative, arteritic, and nonarteritic. Patients with PION that is unassociated with surgery should undergo an evaluation for systemic vascular diseases, including giant cell arteritis, that may or may not be apparent at the time of vision loss. The visual prognosis for patients with perioperative or arteritic PION is poor, whereas that for nonarteritic PION is similar to that for patients with nonarteritic AION.
    American Journal of Ophthalmology 12/2001; 132(5):743-50. · 4.22 Impact Factor
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    Article: Quantification of optic nerve axon loss associated with a relative afferent pupillary defect in the monkey.
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    ABSTRACT: To quantify the amount of optic nerve axonal loss associated with the presence of a mild relative afferent pupillary defect (RAPD) in an experimental monkey model. The right macula of 5 rhesus monkeys (Macaca mulatta) was treated with concentrically enlarging diode laser burns until an RAPD was detected using a transilluminator light and measured with neutral density filters. Intervals between treatments were 3 to 7 days over a period of 2 months. Pupillary responses to light stimulation were recorded with a monocular infrared television pupillometer. Two months after detection of an RAPD, 5 treated and 4 control monkeys underwent euthanasia and enucleation. Histopathologic analysis and quantification of optic nerve axon counts using an image analysis system were performed. No RAPD was observed despite an estimated ganglion cell loss of up to 26%. A 0.6 log unit RAPD was present in 5 monkeys when the laser scar incorporated the entire macula within the temporal vascular arcades. One eye had progressive vitreomacular traction with worsening of the RAPD to 1.8 log units without further laser treatment. Histopathologic evaluation disclosed complete loss of the normal retinal architecture within the macula. The average fiber loss for the 4 treated eyes with 0.6 log unit RAPDs compared with fellow eyes was 53.3% (95% confidence interval [CI], 45.0%-61.6%). The average difference in axon counts between untreated pairs of optic nerves was 12.8% (95% CI, 10.0%-15.6%). Optic nerve axon loss between pairs of experimental and control eyes was statistically significant (P<.001). In rhesus monkeys, an RAPD develops after an approximate unilateral loss between 25% and 50% of retinal ganglion cells. Owing to redundancy in the anterior visual pathways, unilateral retinal ganglion cell loss may occur prior to the observation of an RAPD. The presence of an RAPD measuring 0.6 log units implies that significant retinal ganglion cell injury has occurred.
    Archives of Ophthalmology 09/2001; 119(9):1333-41. · 3.71 Impact Factor
  • Article: Visual function is stable in patients who continue long-term vigabatrin therapy: implications for clinical decision making.
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    ABSTRACT: Vigabatrin (VGB) has been shown to cause visual field constriction and other forms of mild visual dysfunction. We determined the safety of continuing VGB therapy in patients who had received prolonged treatment (>2 years) with the drug by serially monitoring changes in visual function over a 1-year period of continued therapy. We also followed up patients who discontinued VGB to see whether alternative therapies are effective. Fifteen of 17 patients who continued VGB therapy had visual-function testing (visual acuity, color vision, kinetic and static perimetry) every 3 months for 1 year. Eighteen patients who discontinued VGB were given alternative antiepileptic drugs (AEDs); their seizure responses were measured after > or =3 months of treatment. Patients continuing VGB showed no worsening of visual acuity, color vision, or visual-field constriction beyond that measured in the initial test. Many patients who discontinued VGB had good seizure control with either newer or previously unsuccessful AEDs. For patients who have an excellent response to VGB and only mild visual changes, continued therapy may be safe with close visual monitoring. Patients who do not have a significant reduction in seizures or who experience considerable visual dysfunction with VGB may respond well to alternative therapies.
    Epilepsia 05/2001; 42(4):525-30. · 3.96 Impact Factor
  • Article: Choroidal infarction, anterior ischemic optic neuropathy, and central retinal artery occlusion from polyarteritis nodosa.
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    ABSTRACT: Ocular ischemia from polyarteritis nodosa (PAN) is rare. The authors present a case of multifocal ocular infarction from PAN. A 70-year-old woman developed hand and foot numbness followed by intermittent blurred vision and binocular horizontal diplopia. Two weeks later, she suddenly lost vision in the right eye from a central retinal artery occlusion and then developed a left anterior ischemic optic neuropathy and bilateral triangular choroidal abnormalities consistent with infarction. Her erythrocyte sedimentation rate and C-reactive protein were elevated. Although giant cell arteritis was suspected, a multiple mononeuropathy was demonstrated by electromyogram and nerve conduction velocity studies. Biopsy specimens from her sural nerve and biceps muscle showed a necrotizing vasculitis with fibrinoid necrosis, consistent with PAN. Polyarteritis nodosa can produce ischemia of a variety of ocular structures, including the retina, choroid, and optic nerve. In our patient, all three structures were affected. To our knowledge, this is the first reported case of the triangular sign of Amalric in PAN.
    Retina 02/2001; 21(4):348-51. · 2.81 Impact Factor
  • Article: A case-control study of tobacco and alcohol consumption in Leber hereditary optic neuropathy.
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    ABSTRACT: To determine if tobacco or alcohol consumption is associated with vision loss among sibships harboring pathogenic mitochondrial mutations associated with Leber hereditary optic neuropathy. Retrospective case-control study with questionnaires obtained from both affected and unaffected siblings from 80 sibships with Leber hereditary optic neuropathy. Sibships harbored molecularly confirmed mitochondrial DNA mutations at nucleotide positions 11778 (63), 14484 (10), and 3460 (7). Exposure in affected individuals was calculated based on reported consumption before vision loss. For male probands (67 sibships), the recurrence risk within a sibship was 10.3% (eight of 78) for males and 3.1% (three of 98) for females. For female probands (13 sibships), the recurrence risk within a sibship was 17.6% (three of 17) for males and 0% (zero of 22) for females. Greater risk of vision loss was associated with male sex (odds ratio [OR] = 6.63; 95% confidence interval [CI] = 2.96 to 14.84; P =.00001) and harboring a 3460 or 14484 in comparison with the 11778 mutation (OR = 2.071; 95% CI = 1.19 to 3.58; P =.0095). No significant association of maximal intensity of smoking or cumulative smoking, whether light or heavy, with vision loss was observed. Light (OR = 0. 31; 95% CI = 0.17 to 0.56; P =.0001) and heavy alcohol consumers (OR = 0.25; 95% CI = 0.11 to 0.58; P =.0011) were less likely to be affected than individuals who did not consume alcohol after adjusting for age, sex, and mutation. In a categorical analysis of sibships with the 3460 or 14484 mutation, no relationship of vision loss with tobacco or alcohol consumption was observed. Unlike previous studies, the present study calculated exposure based on self-reported consumption of tobacco or alcohol before vision loss. No significant deleterious association between tobacco or alcohol consumption and vision loss among individuals harboring Leber hereditary optic neuropathy mutations was observed. Tobacco and alcohol do not appear to promote vision loss in Leber hereditary optic neuropathy.
    American Journal of Ophthalmology 01/2001; 130(6):803-12. · 4.22 Impact Factor
  • Article: Long-term visual outcome after nonradical microsurgery patients with parasellar and cavernous sinus meningiomas.
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    ABSTRACT: To determine the long-term visual outcome in patients with parasellar and cavernous sinus meningiomas treated with nonradical surgery. Retrospective clinical review of 29 patients with parasellar or cavernous sinus meningiomas and visual sensory or ocular motor dysfunction at presentation, all of whom had at least 10 years of follow-up after initial diagnosis and treatment with nonradical surgery. Nineteen of 29 patients had a unilateral or bilateral optic neuropathy at presentation, and 7 patients developed a unilateral or bilateral optic neuropathy during a mean follow-up period of 13.6 years. However, 27 (93%) of 29 patients retained vision of 20/40 or better in at least one eye, and 14 patients (48%) retained vision of 20/40 or better in both eyes. New ocular motility deficits developed in 3 (10%) of 29 patients during the follow-up period. Radical surgery is not required to achieve long-term useful visual function for patients with parasellar or cavernous sinus meningiomas.
    Neurosurgery 08/2000; 47(1):24-31; discussion 31-2. · 2.79 Impact Factor
  • Article: Visual function loss from vigabatrin: effect of stopping the drug.
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    ABSTRACT: To determine if visual function loss from vigabatrin use recovers after the drug is discontinued. Vigabatrin is an effective antiepileptic drug, but it is known to cause a variety of changes in visual function, including reductions in the visual field, visual acuity, color vision, and in electroretinogram (ERG) and electro-oculogram amplitudes. It is not known whether these changes are reversible. Measurements of static and kinetic visual fields, visual acuity, color vision, and the ERG were recorded while patients were taking vigabatrin and again in 13 patients who had discontinued the drug because of lack of efficacy or reductions in visual field. Most of the patients had been off the drug for 3 to 6 months, although two patients had been drug-free for almost 1 year. Although ERG cone implicit time improved, most of the patients did not show improvement in either clinical measures of visual function (i.e., visual acuity, color vision, visual fields) or in ERG amplitudes. However, several patients who showed minimal visual field loss while on the drug had substantial recovery of ERG amplitudes. There was no statistical association between recovery of function and either duration of treatment or cumulative dosage. The multifocal ERG showed a diffuse loss of function that was not isolated to the periphery. Although the visual deficits in patients taking vigabatrin tend to be mild, most patients do not show improvement after they stop taking the drug. Visual field loss resulting from vigabatrin was not reversible. Visual acuity, color vision, and ERG amplitude loss may be reversible in patients with minimal or no field loss.
    Neurology 08/2000; 55(1):40-5. · 8.31 Impact Factor
  • Article: Anomalous optic disc elevation associated with ultrasonographic evidence of increased subarachnoid fluid.
    S R Sadda, C DiBernardo, N R Miller
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    ABSTRACT: The presence of increased subarachnoid fluid around the optic nerve as measured by ultrasound has been shown to be associated with elevated intracranial pressure, as well as a number of other conditions. This finding has proved useful for distinguishing optic disc elevation secondary to papilledema from disc elevation attributable to other causes. This report describes a patient with anomalous optic disc elevation and increased subarachnoid fluid around the optic nerve.
    Journal of Neuro-Ophthalmology 04/2000; 20(1):25-7. · 1.45 Impact Factor
  • Article: Bilateral optic neuropathy associated with diffuse cerebral angiomatosis in Sturge-Weber syndrome.
    S R Sadda, N R Miller, R Tamargo, R Wityk
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    ABSTRACT: Visual loss in patients with encephalotrigeminal angiomatosis or Sturge-Weber syndrome usually results from secondary glaucoma or from damage to the retrogeniculate pathways by the angiomatous lesions. Optic neuropathy has not been reported as a component of this syndrome. This report describes a patient who developed bilateral optic neuropathy with progressive visual loss associated with diffuse cerebral angiomatosis in the setting of Sturge-Weber syndrome.
    Journal of Neuro-Ophthalmology 04/2000; 20(1):28-31. · 1.45 Impact Factor
  • Article: Unilateral central retinal artery occlusion followed by contralateral anterior ischemic optic neuropathy in giant cell arteritis.
    F B Wein, N R Miller
    Retina 02/2000; 20(3):301-3. · 2.81 Impact Factor
  • Article: Vigabatrin: an effective antiepilepsy drug--balancing the risk of visual dysfunction.
    G L Krauss, N R Miller
    Annals of Pharmacotherapy 01/2000; 33(12):1367-8. · 2.13 Impact Factor
  • Article: Visual dysfunction in patients receiving vigabatrin: clinical and electrophysiologic findings.
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    ABSTRACT: Vigabatrin is an antiepileptic drug that, although relatively well tolerated, is associated with visual field constriction and other visual disturbances of unclear origin. We performed a complete neuroophthalmologic examination and electrophysiologic studies on 39 patients receiving vigabatrin and on 11 control patients. Nearly 50% of patients receiving vigabatrin had constricted visual fields compared with control patients. Some of the vigabatrin patients also had reduced visual acuity and abnormal color vision. In addition, most vigabatrin patients had abnormal electroretinographic results, the severity of which correlated strongly with the degree of visual field constriction. Vigabatrin can cause electrophysiologic evidence of retinal dysfunction and clinically detectable disturbances of visual sensory function.
    Neurology 01/2000; 53(9):2082-7. · 8.31 Impact Factor

Institutions

  • 1988–2010
    • Johns Hopkins University
      • Department of Neurology
      Baltimore, MD, USA
  • 1990–2005
    • Johns Hopkins Medicine
      • • Wilmer Eye Institute
      • • Neuro-Ophthalmology Unit
      • • Department of Pathology
      Baltimore, MD, USA