To characterize the clinical phenotype of myelin oligodendrocyte glycoprotein antibody (MOG-IgG) optic neuritis.
Observational case series.
Setting: Multicenter. Patient/Study Population: Subjects ...meeting inclusion criteria: (1) history of optic neuritis; (2) seropositivity (MOG-IgG binding index > 2.5); 87 MOG-IgG-seropositive patients with optic neuritis were included (Mayo Clinic, 76; other medical centers, 11). MOG-IgG was detected using full-length MOG-transfected live HEK293 cells in a clinically validated flow cytometry assay. Main Outcome Measures: Clinical and radiologic characteristics and visual outcomes.
Fifty-seven percent were female and median age at onset was 31 (range 2–79) years. Median number of optic neuritis attacks was 3 (range 1–8), median follow-up 2.9 years (range 0.5–24 years), and annualized relapse rate 0.8. Average visual acuity (VA) at nadir of worst attack was count fingers. Average final VA was 20/30; for 5 patients (6%) it was ≤20/200 in either eye. Optic disc edema and pain each occurred in 86% of patients. Magnetic resonance imaging showed perineural enhancement in 50% and longitudinally extensive involvement in 80%. Twenty-six patients (30%) had recurrent optic neuritis without other neurologic symptoms, 10 (12%) had single optic neuritis, 14 (16%) had chronic relapsing inflammatory optic neuropathy, and 36 (41%) had optic neuritis with other neurologic symptoms (most neuromyelitis optica spectrum disorder–like phenotype or acute disseminated encephalomyelitis). Only 1 patient was diagnosed with MS (MOG-IgG-binding index 2.8; normal range ≤ 2.5). Persistent MOG-IgG seropositivity occurred in 61 of 62 (98%). A total of 61% received long-term immunosuppressant therapy.
Manifestations of MOG-IgG-positive optic neuritis are diverse. Despite recurrent attacks with severe vision loss, the majority of patients have significant recovery and retain functional vision long-term.
In this article, isolated palsies of cranial nerves III, IV, and VI are addressed. After discussion of the pertinent clinical anatomy of cranial nerves III, IV, and VI, the isolated involvement of ...each of these oculomotor nerves is defined. Based on a review of the literature, methods of evaluation and follow-up of patients presenting with diplopia from lesions of these cranial nerves are presented.
To determine if routinely performed computed tomographic (CT) scanning in patients with aneurysmal subarachnoid hemorrhages (aSAHs) is sufficient to identify patients at high risk of vision loss due ...to Terson syndrome (TS).
Consecutive patients with a diagnosis of aSAH admitted to the neurologic intensive care unit of a regional referral hospital over a 3-year period were prospectively evaluated. Head CT scans performed in the emergency department were assessed for the presence of a “crescent sign” (evidence of significant subinternal limiting membrane hemorrhage). Dilated funduscopic examinations were performed by an ophthalmologist, masked to the results of the CT scan, to identify retinal and vitreous hemorrhages consistent with TS. Retinal hemorrhages were categorized according to size—those smaller than 2 mm in diameter were deemed low risk (lrTS) for vision loss and those larger than 2 mm in diameter were deemed high risk (hrTS) for vision loss.
One hundred seventeen patients with aSAH were enrolled in the study. The overall incidence of TS was 24.9% (29 of 117 patients; 12 were bilateral). Compared to patients without TS, those with TS had a higher Fisher Hemorrhage Grade and a lower mean (±standard deviation) GCS score (8.66 ± 4.97 vs 12.09 ± 1.10; P < 0.001). The CT crescent sign was positive in 7 patients (6.0%), 6 (5.1%; 2 were bilateral) of whom were found to have hrTS. Of the 110 patients without a CT crescent sign, 88 (75.1%) patients did not have TS, 21 had lrTS, and 1 patient had hrTS in one eye. The CT crescent sign was highly sensitive (85.7%) and specific (99.1%) for diagnosing hrTS.
The CT crescent sign is a highly sensitive and specific marker for hrTS. CT scanning may replace routine ophthalmologic examinations to identify patients at risk of vision loss due to aSAH.
IEEE 1584 - 2018 documents the standard method for calculating arc flash incident energy. This value is then used to establish one of several safety boundaries around exposed, energized electrical ...conductors and to select protective equipment. While 1584 covers three-phase configurations in detail, it currently excludes single-phase calculations. Prevalence of single-phase AC power distribution inspired curiosity about possible hazards of fire and injury arising from arc flash in such systems. This paper extends work previously published and covers the conclusion of doctoral research into single-phase arc flash in one laboratory configuration. Further experimental work supported the hypothesis that incident energy would be low (less than 1 cal/cm 2 ) for single-phase events below some threshold voltage and for available fault currents up to 22,000 amperes. This threshold voltage appears to be near 434VAC. Repeated test groups at greater than 434V sustained the conclusion that such configurations develop dangerous levels of heat and blast pressure even when the source is single-phase.
An update on eye pain for the neurologist Lee, Andrew G; Al-Zubidi, Nagham; Beaver, Hilary A ...
Neurologic clinics,
05/2014, Letnik:
32, Številka:
2
Journal Article
Recenzirano
Pain in and around the eye with or without an associated headache is a common presenting complaint to the neurologist. Although the main causes for eye pain are easily diagnosed by simple examination ...techniques that are readily available to a neurologist, sometimes the etiology is not as obvious and may require a referral to an ophthalmologist. This article summarizes and updates our prior review in Neurologic Clinics on this topic and includes (1) ocular and orbital disorders that produce eye pain with a normal examination, (2) neurologic syndromes with predominantly ophthalmologic presentations, and (3) ophthalmologic presentations of selected headache syndromes.
In Memoriam: Frank A. Rubino, MD Freeman, William David; Brazis, Paul W; Biller, Jose ...
JAMA neurology,
11/2015, Letnik:
72, Številka:
11
Journal Article
Cervical dystonias have a variable presentation and underlying etiology, but collectively represent the most common form of focal dystonia. There are a number of known genetic forms of dystonia ...(DYT1-27); however the heterogeneity of disease presentation does not always make it easy to categorize the disease by phenotype-genotype comparison.
In this report, we describe a 53-year-old female who presented initially with hand tremor following a total hip arthroplasty. The patient developed a mixed hyperkinetic disorder consisting of chorea, dystonia affecting the upper extremities, dysarthria, and blepharospasm. Whole exome sequencing of the patient revealed a novel heterozygous missense variant (Chr11(GRCh38): g.26525644C > G; NM_031418.2(ANO3): c.702C > G; NP_113606.2. p.C234W) in exon 7 in the ANO3 gene.
ANO3 encodes anoctamin-3, a Ca
-dependent phospholipid scramblase expressed in striatal-neurons, that has been implicated in autosomal dominant craniocervical dystonia (Dystonia-24, DYT24, MIM# 615034). To date, only a handful of cases of DYT-24 have been described in the literature. The complex clinical presentation of the patient described includes hyperkinesias, complex motor movements, and vocal tics, which have not been reported in other patients with DYT24. This report highlights the utility of using clinical whole exome sequencing in patients with complex neurological phenotypes that would not normally fit a classical presentation of a defined genetic disease.
NFPA 70E and IEEE 1584 are defining standards for arc flash hazard analysis. Both assume three-phase faults for calculations since three-phase power distribution is predominant in utility and ...industrial applications; however, arc flash in single-phase systems is excluded. Single-phase faults to neutral or ground or single phase-to-phase faults can occur in a variety of circumstances. Such events may have the potential to produce arc flash and blast that would pose a significant safety concern. This paper describes a recent doctoral research investigation of arc flash in one single-phase configuration. Experiments were sponsored by UL and performed at the Schneider Electric High-Power Laboratory in Cedar Rapids, Iowa. This facility provided a test article; a full suite of voltage, current, and temperature instrumentation; and high-speed video recording. Experimental work revealed incident energy values less than 0.2 cal/cm 2 for 240-volt single-phase arc fault events though there was still significant flash and splatter of molten wire residue. 480 V single-phase events produced an order of magnitude greater heat energy, and at 22 kA sustained arcing until interrupted by the test cell controller. Results suggest that 240 V single-phase panels and equipment common in residential and light commercial applications may be at very low risk of yielding arc flash burn-related injuries. However, 480 V single-phase faults can produce levels of incident heat energy known to cause burns, flash, and blast pressure at available fault currents between 10 and 22 kA.
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