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NEUROLOGY 2007;68:540-541
© 2007 American Academy of Neurology

February 20 Highlights

APOE {varepsilon}4: Risk factor for impaired verbal learning in MS

Koutsis et al. investigated the effect of APOE {varepsilon}4 on different cognitive domains in patients with MS. They found that {varepsilon}4 carriers had a sixfold increase in the relative risk of impairment in verbal learning vs noncarriers.

see page 546

Preventable adverse events in stroke


Figure 13
Figure. Event analysis.

Holloway et al. found that adverse events were spontaneously reported in approximately one in eight patients admitted with ischemic stroke or intracerebral hemorrhage over a 3.5-year period. About 46% of reported events were considered preventable and involved all aspects of care from acute thrombolytic treatment to end-of-life management.

see page 550

The editorial by Rizzo and Glick notes that eliminating medication errors and minimizing falls and other adverse events in stroke (and by example in other major categories of neurologic disease) will require general and specific measures, including comprehensive institutional efforts, systems changes, and multilevel collaborations among stroke teams, other neurologists, all caregivers, systems managers, and patients. For now, documentation of preventable adverse events in patients with stroke is a wake-up call for those of us who may implement acute interventions, such as thrombolysis.

see page 542

Lipid levels and risk of ischemic stroke

In a prospective cohort study of 27,937 women, Kurth et al. found significant associations between total cholesterol, low-density lipoprotein cholesterol, the total cholesterol to high-density lipoprotein cholesterol ratio, and non-high-density lipoprotein cholesterol with increased risk of ischemic stroke.

see page 556

Differential expression in facioscapulohumeral dystrophy (FSHD)

Osborne et al. found no differential expression in facioscapulohumeral dystrophy muscle of genes proximal to the causal deletion on 4q35. They did, however, find differential expression of vascular genes suggesting a link between the muscular dystrophy and the retinal vasculopathy seen in FSHD.

see page 569

Typical phenotype in FSHD family with extended proximal deletion

Deak et al. characterized an FSHD family transmitting an extended proximal deletion encompassing the p13E-11 probe binding site and genes FRG2 and DUX4c. The deletion spans approximately 75 kb, the largest yet described. Despite the unusual genotype, all family members have typical FSHD phenotype.

see page 578

The editorial by S.A. Greenberg and Padberg about these two articles notes that the genetic disturbance present in almost all people with FSHD is of a peculiar nature. It consists of an uncommon deletion of a region at the distal end of chromosome 4q combined with the presence of one of two common sequence variants distal to this deleted region. Both are necessary. Furthermore, unlike most other genetic disorders, the mutant FSHD deleted region does not include any known transcribed sequences. These two articles advance our understanding of FSHD. One provides compelling data pertaining to a new hypothesis for the disease mechanism, and insight into the relationship between gene expression and non-muscle systemic disease in FSHD, and the other adds to defining the minimal genetic requirement for the disease.

see page 544

Serum estradiol and risk of stroke in elderly men

Abbott et al. examined the relationship between serum estradiol and stroke in elderly men. Estradiol was measured in 2,197 elderly men from 1991 to 1993 with follow-up for stroke to 1998. Findings suggest that elevated estradiol is associated with incident stroke in elderly men.

see page 563

Neuromyelitis optica: Secondary progression is rare


Figure 23
Figure. Actuarial survival analysis conversion to a secondary progressive clinical course.

Wingerchuk et al. determined that only two of 96 (2.1%) neuromyelitis optica patients developed a secondary progressive course. This rate was lower than predicted from MS natural history data (21 expected cases; p = 0.00002), supporting the hypothesis that relapses and progression are dissociated in CNS demyelinating diseases.

see page 603

Patterns of swallowing in parkinsonian syndromes

Alfonsi et al. used an electrophysiological method to identify swallowing abnormalities and dysphagia severity in parkinsonian syndromes. They found that differences between Parkinson disease, and multiple system atrophy or progressive supranuclear palsy were present early in the course of the disease.

see page 583

Lacunar infarct vs deep hemorrhage: Who gets which?

Labovitz et al. used a population-based stroke incidence survey to compare determinants of lacunar infarction vs deep intracerebral hemorrhage. In multivariate analysis, cases with lacunar infarct were significantly older, more likely to have diabetes, and had higher cholesterol than cases with deep intracerebral hemorrhage.

see page 606

Anti-MuSK vs anti-AChR positive and seronegative myasthenia gravis

Deymeer et al. compared 32 anti-MuSK positive and 33 seronegative vs 161 consecutive anti-AChR positive patients with myasthenia gravis. The anti-MuSK positive group had a higher frequency of respiratory crises. Their outcome was not different from that of the anti-AChR-positive patients; however, the seronegative patients had better outcome than the other two groups.

see page 609

Outcome of chronic daily headache in adolescents

Wang et al. followed up 122 community-based adolescents with chronic daily headache (CDH) for two years. Most subjects still had frequent headaches although CDH incidence declined to 25%. Migraine diagnosis gained prominence as headache frequencies decreased. Medication overuse and major depression predicted poor outcomes.

see page 591

Referral bias and its effect on survival in ALS

Sorenson et al. studied the effect of referral bias on survival of ALS subjects in a tertiary care setting. The study identified a significant bias when comparing referred patients to a population based sample. They conclude this bias should be considered when assessing the apparent impact of self-selected treatments in the ALS population.

see page 600


Related articles in Neurology:

Medical errors after stroke: Preventable or inevitable?
Matthew Rizzo and Thomas H. Glick
Neurology 2007 68: 542-543. [Full Text]  

Pushing the genetic frontier with facioscapulohumeral muscular dystrophy
Steven A. Greenberg and George W. Padberg
Neurology 2007 68: 544-545. [Full Text]  

APOE {varepsilon}4 is associated with impaired verbal learning in patients with MS
G. Koutsis, M. Panas, E. Giogkaraki, C. Potagas, G. Karadima, C. Sfagos, and D. Vassilopoulos
Neurology 2007 68: 546-549. [Abstract] [Full Text]  

The safety of hospital stroke care
Robert G. Holloway, Deborah Tuttle, Tracy Baird, and W. Keith Skelton
Neurology 2007 68: 550-555. [Abstract] [Full Text]  

Lipid levels and the risk of ischemic stroke in women
T. Kurth, B. M. Everett, J. E. Buring, C. S. Kase, P. M. Ridker, and J. M. Gaziano
Neurology 2007 68: 556-562. [Abstract] [Full Text]  

Serum estradiol and risk of stroke in elderly men
R. D. Abbott, L. J. Launer, B. L. Rodriguez, G. W. Ross, P.W.F. Wilson, K. H. Masaki, D. Strozyk, J. D. Curb, K. Yano, J. S. Popper, and H. Petrovitch
Neurology 2007 68: 563-568. [Abstract] [Full Text]  

Expression profile of FSHD supports a link between retinal vasculopathy and muscular dystrophy
R. J. Osborne, S. Welle, S. L. Venance, C. A. Thornton, and R. Tawil
Neurology 2007 68: 569-577. [Abstract] [Full Text]  

Genotype-phenotype study in an FSHD family with a proximal deletion encompassing p13E-11 and D4Z4
K. L. Deak, R. J.L.F Lemmers, J. M. Stajich, R. Klooster, R. Tawil, R. R. Frants, M. C. Speer, S. M. van der Maarel, and J. R. Gilbert
Neurology 2007 68: 578-582. [Abstract] [Full Text]  

Electrophysiologic patterns of oral-pharyngeal swallowing in parkinsonian syndromes
E. Alfonsi, M. Versino, I. M. Merlo, C. Pacchetti, E. Martignoni, G. Bertino, A. Moglia, C. Tassorelli, and G. Nappi
Neurology 2007 68: 583-589. [Abstract] [Full Text]  

Outcomes and predictors of chronic daily headache in adolescents: A 2-year longitudinal study
Shuu-Jiun Wang, Jong-Ling Fuh, Shiang-Ru Lu, and Kai-Dih Juang
Neurology 2007 68: 591-596. [Abstract] [Full Text]  

Effect of referral bias on assessing survival in ALS
Eric J. Sorenson, Jayawant Mandrekar, Brian Crum, and J. Clarke Stevens
Neurology 2007 68: 600-602. [Abstract] [Full Text]  

A secondary progressive clinical course is uncommon in neuromyelitis optica
D. M. Wingerchuk, S. J. Pittock, C. F. Lucchinetti, V. A. Lennon, and B. G. Weinshenker
Neurology 2007 68: 603-605. [Abstract] [Full Text]  

Lacunar infarct or deep intracerebral hemorrhage: Who gets which? The Northern Manhattan Study
Daniel L. Labovitz, Bernadette Boden-Albala, W. Allen Hauser, and Ralph L. Sacco
Neurology 2007 68: 606-608. [Abstract] [Full Text]  

Clinical comparison of anti-MuSK- vs anti-AChR-positive and seronegative myasthenia gravis
F. Deymeer, O. Gungor-Tuncer, V. Yilmaz, Y. Parman, P. Serdaroglu, C. Ozdemir, A. Vincent, and G. Saruhan-Direskeneli
Neurology 2007 68: 609-611. [Abstract] [Full Text]  




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