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A rapidly progressive motor neuron disease associated to a natural killer cells leukaemia
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system play a complex role, either protective or toxic, in ALS
pathogenesis [1–3]. In particular, compelling evidence indicate that
increased blood level of natural killer (NK) and NK-T cells may contribute
to the disease development and progression [2,3].
Here, we report on a patient with an aggressive Motor Neuron
Disease (MND) associated with NK/NK-T cells leukaemia.
1. Case report
A 79-year-old man presented with several months-history of a
progressive atrophy and weakness of the upper limbs, which quickly
spread to the lower limbs. Onset was subtle and apparently occurred in
the month of July (the specific date is not shown for privacy), when the
patient noticed a mild weakness in the proximal muscles of the right
arm. From July through August, the patient's global functioning was
however roughly normal. However, between September and early
October the patient experienced a rapid worsening, with a severe
weakness of the shoulder girdle, of the right arm and in both legs. He
was referred to our ALS Center for evaluation. The neurological examination
documented marked muscle atrophy of the shoulder girdle,
the right arm and both lower limbs, with bilateral foot drop. Deep
tendon reflexes were absent, with the exception of the left arm. Bulbar
functions were normal. Needle EMG showed widespread polyphasic
MUP and fibrillation potentials, with normal sensory and motor conduction
velocities and no conduction blocks. Sensory and motor nerves
amplitudes in the upper limbs were within the normal range. A brain
and cervical MRI was within normal range. Extensive biochemical and
immunological work-up, including CSF, blood cell count, a large antibody
battery which included antibodies to anti-Hu, anti-Ri, anti-Yo, and
anti-gangliosides were negative. Serum creatine phosphokinase level
was mildly increased. A diagnosis of a very rapidly progressing lower
motor neuron disease (MND) was made, with an ALSFRS-R score of 18/
48 and a ΔFS [4] of 10 (Fig. 1A).
Although the clinical features of the patient did not fit with a formal
diagnosis of ALS, a genetic testing for the major ALS related genes
(C9orf72, FUS, TARDBP, SOD1, and Angiogenin) was performed, which
gave negative results. In the middle of November 2016, a follow-up
blood test showed a marked increase of WBC (i.e., 76,050/μL), with a
high prevalence of lymphocytes.
He was then admitted to the Hematology Unit in our Hospital. Blood
cytofluorimetric analysis and a bone marrow biopsy showed a marked
infiltration by immature NK and NK-T cells (Fig. 1B). A new CSF analysis
was performed, which showed increased proteins and 360/μL WBC, of
which about 70% were NK/NK-T cells. A diagnosis of NK/NK-T cell leukaemia
was made. A systemic chemotherapy according to Hyper-CVAD
schema was started (i.e., High Dose Dexamethasone; Hyperfractionated
Cyclophosphamide; Oncovine and Doxorubicine), along with repeated
intrathecal injections with Methotrexate, Ara-C and Dexamethasone.
In the following weeks, the patient showed a transient improvement
in muscle strength and function; a ALSFRS-R evaluation, made in
December 2016, gave a score of 27/48, with a ΔFS dropping to 4.2
(Fig. 1A). Unfortunately, the general health status deteriorated over
time, and in late December, the patient was referred to a Hospice,
where a few weeks later he peacefully passed away.
2. Discussion
We have described an aggressive lower MND, which might be
paraneoplastic, associated with an NK-T leukaemia. A paraneoplastic
association between either lower MND and lymphoma or the upper
predominant MND and breast cancer has been reported [5–7].
The presence of a high number of NK/NK-T cells in the CSF indicates
a meningeal metastasis from the acute leukaemia. These cells
are most probably involved in the lower motor neuron degeneration in
our patient. The clinical diagnosis of MND in this case is supported by
several lines of evidence: a) the onset of atrophy and weakness occurred
in the shoulder girdle, involving first the right shoulder with a rapid
progression to the left shoulder, and then with a proximal-to-distal
spreading to the right arm and then to the lower limbs. This pattern of
spreading is consistent with a primarily MND involvement, rather than
a poly/multi-neuropathy; b) the EMG/ENG pattern was also consistent
with a lower motor neuron disease, as neither abnormal sensory/motor
amplitudes (recorded in the upper limbs) nor conduction velocities/
blocks (recorded in both the upper and lower limbs) could be
detected. It cannot, however, be excluded a contemporary involvement
of the motor neurons cell bodies and the proximal section of the axons (i.e.
the motor roots)
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Dispelling the Myths Behind First-author Citation Counts
Full text linkWe conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
koamabayili/VECTRON-author-checklist: VECTRON author checklist
No full textWe have done our best to complete the author checklist relating to the use of animals in the hut study. Note that the objective for the hut study was to evaluate the IRS treatment applications for residual efficacy against Anopheles mosquitoes, including the local An. coluzzii mosquito population. Cows were only used to attract mosquitoes into the huts and no tests were carried out directly on the cows. The author checklist is intended for use with studies where experiments are carried out on animals, which is why we have had such difficulty in completing this for the hut study, as many of the questions do not relate to how the cows were used
Author-wise bibliometric analysis based on entropy.
No full textAuthor-wise bibliometric analysis based on entropy.</p
Author Under Sail The Imagination of Jack London, 1893-1902
No full textIn Author Under Sail, Jay Williams offers the first complete literary biography of Jack London as a professional writer engaged in the labor of writing. It examines the authorial imagination in London's work, the use of imagination in both his fiction and nonfiction, and the ways he defined imagination in the creative process in his business dealings with his publishers, editors, and agents. In this first volume of a two-volume biography, Williams traverses the years 1893 to 1902, from London's "Story of a Typhoon" to The People of the Abyss. The Jack London who emerges in the pages of Author Under Sail is a writer whose partnership with publishers, most notably his productive alliance with George Brett of Macmillan, was one of the most formative in American literary history. London pioneered many author models during the heyday of realism and naturalism, blurring the boundaries of these popular genres by focusing on absorption and theatricality and the representation of the seen and unseen. London created an impassioned, sincere, and extremely personal realism unlike that of other American writers of the time. Author Under Sail is a literary tour de force that reveals the full range of London as writer, creative citizen, and entrepreneur at the same time it sheds light on the maverick side of machine-age literature.Intro -- Title Page -- Copyright Page -- Dedication -- Contents -- Acknowledgments -- Introduction -- 1. Spirit Truth -- 2. From Absorption to Theatricality and Back Again -- 3. "I Will Build a New Present" -- 4. Sons as Authors -- 5. Fathers as Publishers -- 6. The Daughter as Author -- 7. Lovers as Authors -- 8. At Sea with the Family -- 9. Yellow News, Yellow Stories -- 10. The Return Home -- Notes -- Bibliography -- Index -- About Jay WilliamsIn Author Under Sail, Jay Williams offers the first complete literary biography of Jack London as a professional writer engaged in the labor of writing. It examines the authorial imagination in London's work, the use of imagination in both his fiction and nonfiction, and the ways he defined imagination in the creative process in his business dealings with his publishers, editors, and agents. In this first volume of a two-volume biography, Williams traverses the years 1893 to 1902, from London's "Story of a Typhoon" to The People of the Abyss. The Jack London who emerges in the pages of Author Under Sail is a writer whose partnership with publishers, most notably his productive alliance with George Brett of Macmillan, was one of the most formative in American literary history. London pioneered many author models during the heyday of realism and naturalism, blurring the boundaries of these popular genres by focusing on absorption and theatricality and the representation of the seen and unseen. London created an impassioned, sincere, and extremely personal realism unlike that of other American writers of the time. Author Under Sail is a literary tour de force that reveals the full range of London as writer, creative citizen, and entrepreneur at the same time it sheds light on the maverick side of machine-age literature.Description based on publisher supplied metadata and other sources.Electronic reproduction. Ann Arbor, Michigan : ProQuest Ebook Central, YYYY. Available via World Wide Web. Access may be limited to ProQuest Ebook Central affiliated libraries
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