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Visual Evoked-potential latency prolongation in MS: correlation with cognitive performance on a computerized testing battery [Meeting Abstract]

Gudesblatt, M.; Zarif, M.; Balcer, L.; Bumstead, B.; Fafard, L.; Torres, V.; Florence, A.; Galetta, S.; Doniger, G.
ISI:000209137300171
ISSN: 1352-4585
CID: 5342522

Should most patients with optic neuritis be tested for neuromyelitis optica antibodies and should this affect their treatment?

Galetta, Steven L; Cornblath, Wayne T
PMID: 21107126
ISSN: 1070-8022
CID: 174717

The neuro-ophthalmology of multiple sclerosis

Frohman, Teresa C; Graves, Jennifer; Balcer, Laura J; Galetta, Steven L; Frohman, Elliot M
Multiple sclerosis (MS) is the quintessential neurologic disorder from which to understand the principles of afferent and efferent neuro-ophthalmology. Perhaps with the exception of stroke, no other disorder is associated with nearly every sign and symptom of abnormalities targeting the visual system and the ocular motor apparatus. This focused review will underscore the most common syndromes and their derivative signs and symptoms that affect vision as a consequence of MS.
PMID: 22810602
ISSN: 1080-2371
CID: 174656

Photo essay. MRI and positron emission tomography findings in Heidenhain variant Creutzfeldt-Jakob disease [Case Report]

Prasad, Sashank; Lee, Edward B; Woo, John H; Alavi, Abass; Galetta, Steven L
The typical presentation of Heidenhain variant Creutzfeldt-Jakob disease (CJD) is a rapidly progressive visual loss in the setting of a relatively normal ophthalmologic examination. At presentation, patients with this uniformly fatal illness frequently demonstrate only minor cortical abnormalities on MRI. Here, we document the clinical presentation and imaging results of a patient with Heidenhain variant CJD in whom abnormalities on positron emission tomographic imaging were more evident than changes on MRI. These changes were present in striate cortex and visual association areas, providing clinical-anatomical correlation with our patient's visual deficits. Nuclear imaging provides a considerably more sensitive measure of neural dysfunction early in the course of this disease.
PMID: 20581692
ISSN: 1070-8022
CID: 174719

Eye movement abnormalities in multiple sclerosis

Prasad, Sashank; Galetta, Steven L
Patients with multiple sclerosis commonly describe visual symptoms that result from several eye movement abnormalities that occur from disruption of critical pathways in the brainstem, cerebellum, and cerebral hemispheres. These abnormalities include internuclear ophthalmoplegia, ocular motor palsy, ocular misalignment, pathologic nystagmus, impaired saccades, saccadic intrusions, and impaired pursuit. Detailed knowledge of these problems and their neuroanatomic localization will aid the physician by guiding diagnosis and therapeutic decision making.
PMID: 20637994
ISSN: 0733-8619
CID: 174718

Longitudinal study of vision and retinal nerve fiber layer thickness in multiple sclerosis

Talman, Lauren S; Bisker, Esther R; Sackel, David J; Long, David A Jr; Galetta, Kristin M; Ratchford, John N; Lile, Deacon J; Farrell, Sheena K; Loguidice, Michael J; Remington, Gina; Conger, Amy; Frohman, Teresa C; Jacobs, Dina A; Markowitz, Clyde E; Cutter, Gary R; Ying, Gui-Shuang; Dai, Yang; Maguire, Maureen G; Galetta, Steven L; Frohman, Elliot M; Calabresi, Peter A; Balcer, Laura J
OBJECTIVE: Cross-sectional studies of optical coherence tomography (OCT) show that retinal nerve fiber layer (RNFL) thickness is reduced in multiple sclerosis (MS) and correlates with visual function. We determined how longitudinal changes in RNFL thickness relate to visual loss. We also examined patterns of RNFL thinning over time in MS eyes with and without a prior history of acute optic neuritis (ON). METHODS: Patients underwent OCT measurement of RNFL thickness at baseline and at 6-month intervals during a mean follow-up of 18 months at 3 centers. Low-contrast letter acuity (2.5%, 1.25% contrast) and visual acuity (VA) were assessed. RESULTS: Among 299 patients (593 eyes) with >or=6 months follow-up, eyes with visual loss showed greater RNFL thinning compared to eyes with stable vision (low-contrast acuity, 2.5%: p < 0.001; VA: p = 0.005). RNFL thinning increased over time, with average losses of 2.9microm at 2 to 3 years and 6.1microm at 3 to 4.5 years (p < 0.001 vs 0.5-1-year follow-up interval). These patterns were observed for eyes with or without prior history of ON. Proportions of eyes with RNFL loss greater than test-retest variability (>or=6.6microm) increased from 11% at 0 to 1 year to 44% at 3 to 4.5 years (p < 0.001). INTERPRETATION: Progressive RNFL thinning occurs as a function of time in some patients with MS, even in the absence of ON, and is associated with clinically significant visual loss. These findings are consistent with subclinical axonal loss in the anterior visual pathway in MS, and support the use of OCT and low-contrast acuity as methods to evaluate the effectiveness of putative neuroprotection protocols.
PMCID:2901775
PMID: 20517936
ISSN: 0364-5134
CID: 174658

Combined optic neuropathy and myelopathy secondary to copper deficiency [Case Report]

Pineles, Stacy L; Wilson, Christina A; Balcer, Laura J; Slater, Robert; Galetta, Steven L
We report two patients, both with a history of gastric surgery, who presented with progressive optic neuropathy and myelopathy. The patients' symptoms were initially attributed to vitamin B12 deficiency and/or neuromyelitis optica; however, after the neurologic deficits continued to progress with the use of conventional treatments, further evaluation was initiated, and a severe copper deficiency was revealed. Copper deficiency is a rare cause of progressive optic neuropathy and myelopathy and should be considered in the differential diagnosis. It is crucial to elicit a history of gastric surgery or other risk factors for hypocupremia in those patients undergoing an evaluation for subacute or chronically progressive optic neuropathy or myelopathy.
PMID: 20451943
ISSN: 0039-6257
CID: 174660

Natalizumab plus interferon beta-1a reduces lesion formation in relapsing multiple sclerosis

Radue, Ernst-Wilhelm; Stuart, William H; Calabresi, Peter A; Confavreux, Christian; Galetta, Steven L; Rudick, Richard A; Lublin, Fred D; Weinstock-Guttman, Bianca; Wynn, Daniel R; Fisher, Elizabeth; Papadopoulou, Athina; Lynn, Frances; Panzara, Michael A; Sandrock, Alfred W
The SENTINEL study showed that the addition of natalizumab improved outcomes for patients with relapsing multiple sclerosis (MS) who had experienced disease activity while receiving interferon beta-1a (IFNbeta-1a) alone. Previously unreported secondary and tertiary magnetic resonance imaging (MRI) measures are presented here. Patients received natalizumab 300 mg (n=589) or placebo (n=582) intravenously every 4 weeks plus IFNbeta-1a 30 microg intramuscularly once weekly. Annual MRI scans allowed comparison of a range of MRI end points versus baseline. Over 2 years, 67% of patients receiving natalizumab plus IFNbeta-1a remained free of new or enlarging T2-lesions compared with 30% of patients receiving IFNbeta-1a alone. The mean change from baseline in T2 lesion volume over 2 years decreased in patients receiving natalizumab plus IFNbeta-1a and increased in those receiving IFNbeta-1a alone (-277.5mm(3) versus 525.6mm(3); p<0.001). Compared with IFNbeta-1a alone, add-on natalizumab therapy resulted in a smaller increase in mean T1-hypointense lesion volume after 2 years (1821.3mm(3) versus 2210.5mm(3); p<0.001), a smaller mean number of new T1-hypointense lesions over 2 years (2.3 versus 4.1; p<0.001), and a slower rate of brain atrophy during the second year of therapy (-0.31% versus -0.40%; p=0.020). Natalizumab add-on therapy reduced gadolinium-enhancing, T1-hypointense, and T2 MRI lesion activity and slowed brain atrophy progression in patients with relapsing MS who experienced disease activity despite treatment with IFNbeta-1a alone.
PMID: 20236661
ISSN: 0022-510x
CID: 174721

Freedom from disease activity in multiple sclerosis

Havrdova, Eva; Galetta, Steven; Stefoski, Dusan; Comi, Giancarlo
BACKGROUND: Multiple sclerosis (MS) shares many pathologic features with other immune-mediated inflammatory diseases, such as rheumatoid arthritis, Crohn disease, and psoriasis. The development of effective biologic agents for rheumatoid arthritis has resulted in a treatment paradigm shift such that disease remission is now an explicit goal. EXPERT CLINICAL OPINION: The traditional immunomodulatory disease-modifying therapies for MS (interferon beta and glatiramer acetate) delay disease progression and reduce activity on brain MRI to varying degrees; however, they have not been demonstrated to induce disease remission. Therefore, the concept of disease remission or freedom from disease activity in MS has received little attention from the neurology community. We discuss some potential definitions of disease remission in MS and whether freedom from disease activity can become an increasingly useful measure of therapeutic response. FUTURE DIRECTIONS: Future research should be directed at determining the long-term significance of freedom from disease activity during a short-term clinical trial in relapsing-remitting MS.
PMID: 20421571
ISSN: 0028-3878
CID: 174720

Education research: a new system for reducing patient nonattendance in residents' clinic

Price, Raymond S; Balcer, Laura J; Galetta, Steven L
BACKGROUND: Patient nonattendance in neurology and other subspecialty clinics is closely linked to longer waiting times for appointments. We developed a new scheduling system for residents' clinic that reduced average waiting times from >4 months to < or =3 weeks. The purpose of this study was to compare nonattendance for clinics scheduled using the new model (termed "rapid access") vs those scheduled using the traditional system. METHODS: In the rapid access system, nonestablished (new) patients are scheduled on a first-come, first-served basis for appointments that must occur within 2 weeks of their telephone request. Nonattendance for new patient appointments (cancellations plus no-shows) was compared for patients scheduled under the traditional vs the rapid access scheduling systems. Nonattendance was compared for periods of 6, 12, and 18 months following change in scheduling system using the chi2 test and logistic regression. RESULTS: Compared to the traditional scheduling system, the rapid access system was associated with a 50% reduction in nonattendance over 18 months (64% [812/1,261 scheduled visits] vs 31% [326/1,059 scheduled visits], p < 0.0001). In logistic regression models, appointment waiting time was a major factor in the relation between rapid access scheduling and nonattendance. Demographics, diagnoses, and likelihood of scheduling follow-up visits were similar between the 2 systems. CONCLUSIONS: A new scheduling system that minimizes waiting times for new patient appointments has been effective in substantially reducing nonattendance in our neurology residents' clinic. This rapid access system should be considered for implementation and will likely enhance the outpatient educational experience for trainees in neurology.
PMCID:2839191
PMID: 20211902
ISSN: 0028-3878
CID: 174663