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Article Title |
Author(s) |
Type (Status) |
Available Online |
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Mutation spectrum and phenotypic manifestation in FSHD Greek patients
DOI: 10.1016/j.nmd.2011.11.001
Abstract: Facioscapulohumeral Muscular Dystrophy (FSHD) is a genetic myopathy with a remarkable intra- and inter-familial clinical heterogeneity. This study reports the clinical and genetic analysis o...
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P. Sakellariou,
K. Kekou,
H. Fryssira,
C. Sofocleous,
P. Manta,
A. Panousopoulou,
K. Gounaris,
E. Kanavakis
et al.
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Full-length article
(In Press Corrected Proof)
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22 February 2012 |
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A case of myelopathy, myopathy, peripheral neuropathy and subcortical grey matter degeneration associated with recessive compound heterozygous POLG1 mutations
DOI: 10.1016/j.nmd.2011.10.017
Abstract: This 54year old woman presented with symptoms of sensory ataxic neuropathy, with cerebellar features. She developed further weakness, visual disturbances with diplopia, dysarthria and dyspha...
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P. McKelvie,
R. Marotta,
D.R. Thorburn,
J. Chin,
S. Punchihewa,
S. Collins
et al.
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Short communication
(In Press Corrected Proof)
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22 February 2012 |
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Sun, Surf and Science
DOI: 10.1016/j.nmd.2012.01.007
Algarve, the ultimate sunshine haven: deep blue sea and azure skies, vivid green golf courses; sweet nemesis for ex-pat Brits and a serious challenge to the activities of the World Muscle Society’s Tr...
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Jane Miller,
Susan Brown
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Conference
(In Press Corrected Proof)
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17 February 2012 |
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Experimental validation of in silico predicted KCNA1, KCNA2, KCNA6 and KCNQ2 genes for association studies of peripheral nerve hyperexcitability syndrome in Jack Russell Terriers
DOI: 10.1016/j.nmd.2012.01.008
Abstract: KCNA1, KCNA2, KCNA6 and KCNQ2 are associated with peripheral nerve hyperexcitability in humans. In order to determine if these genes are also involved in Jack Russell Terriers with a similar...
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Mario Van Poucke,
An E. Vanhaesebrouck,
Luc J. Peelman,
Luc Van Ham
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Full-length article
(In Press Corrected Proof)
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16 February 2012 |
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Fetal acetylcholine receptor inactivation syndrome and maternal myasthenia gravis: A case report
DOI: 10.1016/j.nmd.2012.01.002
Abstract: Fetal acetylcholine receptor inactivation syndrome is a rare condition occurring in newborns of myasthenic mothers, characterized by bulbar and facial weakness after recovery from the genera...
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Adele D’Amico,
Enrico Bertini,
Flaviana Bianco,
Patrizia Papacci,
Leslie Jacobson,
Angela Vincent,
Eugenio Mercuri
et al.
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Short communication
(In Press Corrected Proof)
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10 February 2012 |
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Paroxysmal neuromyotonia: A new sporadic channelopathy
DOI: 10.1016/j.nmd.2012.01.004
Abstract: Neuromyotonia is a heterogeneous group of genetic and autoimmune channelopathies resulting in hyperexcitability of peripheral nerves. We report an unusual case of neuromyotonia, which to our...
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Teeratorn Pulkes,
Charungthai Dejthevaporn,
Metha Apiwattanakul,
Chutima Papsing,
Michael G. Hanna
et al.
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Short communication
(In Press Corrected Proof)
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06 February 2012 |
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Two common mutations (p.Gln832X and c.663+1G>C) account for about a third of the DYSF mutations in Korean patients with dysferlinopathy
DOI: 10.1016/j.nmd.2011.12.007
Abstract: Dysferlinopathy refers to autosomal recessive muscular dystrophies caused by mutations in dysferlin gene (DYSF). It includes two major distinct disorders, Miyoshi myopathy and limb-girdle mu...
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Young-Eun Park,
Hyang-Sook Kim,
Chang-Hoon Lee,
Tai-Seung Nam,
Young-Chul Choi,
Dae-Seong Kim
et al.
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Short communication
(In Press Corrected Proof)
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01 February 2012 |
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Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains
DOI: 10.1016/j.nmd.2011.10.011
Abstract: The genetic defect of mdx mice resembles that of Duchenne muscular dystrophy, although their functional performance and life expectancy is nearly normal. By contrast, mice lacking utrophin a...
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Maaike van Putten,
Darshan Kumar,
Margriet Hulsker,
Willem M.H. Hoogaars,
Jaap J. Plomp,
Annemarieke van Opstal,
Maarten van Iterson,
Peter Admiraal,
Gert-Jan B. van Ommen,
Peter A.C. ‘t Hoen,
Annemieke Aartsma-Rus
et al.
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Full-length article
(In Press Corrected Proof)
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30 January 2012 |
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Cerebral and muscle MRI abnormalities in myotonic dystrophy
DOI: 10.1016/j.nmd.2012.01.003
Abstract: Pathophysiological mechanisms underlying the clinically devastating CNS features of myotonic dystrophy (DM) remain more enigmatic and controversial than do the muscle abnormalities of this c...
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Daniel T. Franc,
Ryan L. Muetzel,
Paul R. Robinson,
Craig P. Rodriguez,
Joline C. Dalton,
Cameron E. Naughton,
Bryon A. Mueller,
Jeffrey R. Wozniak,
Kelvin O. Lim,
John W. Day
et al.
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Full-length article
(In Press Corrected Proof)
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30 January 2012 |
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When tubules aggregate
DOI: 10.1016/j.nmd.2011.12.006
Within muscle fibres, tubules appear as microtubules, transverse tubules, and different components of the sarcotubular system, the latter consisting of longitudinal tubules and lateral or terminal sac...
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Hans H. Goebel
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Discussion
(In Press Corrected Proof)
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27 January 2012 |
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Cortical heterotopia in LGMD2I
DOI: 10.1016/j.nmd.2011.11.005
A 40-year-old man, with a history of one generalized non-febrile seizure at the age of 4years, presented with slowly progressive proximal muscle weakness in the four limbs since the age of 12years and...
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Dimitri Renard,
Carla Fernandez,
Celine Bouchet-Seraphin,
Pierre Labauge
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Short communication
(In Press Corrected Proof)
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24 January 2012 |
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Cardiac dysfunction and pathology in the dystrophin and utrophin-deficient mouse during development of dilated cardiomyopathy
DOI: 10.1016/j.nmd.2011.07.003
Abstract: Cardiac involvement in Duchenne muscular dystrophy is asymptomatic until function is severely affected. Little is known about its evolution, and few animal models are available to study pote...
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Ju Lan Chun,
Robert O’Brien,
Suzanne E. Berry
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Full-length article
(In Press Corrected Proof)
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24 January 2012 |
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Necklace fibers as histopathological marker in a patient with severe form of X-linked myotubular myopathy
DOI: 10.1016/j.nmd.2011.12.005
Abstract: X-linked myotubular myopathy due to mutations in the MTM1 gene is classically characterized by a severe neonatal phenotype and a typical muscle biopsy presenting globular and centrally locat...
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Juliana Gurgel-Giannetti,
Edmar Zanoteli,
Eralda Luiza de Castro Concentino,
Osorio Abath Neto,
João Bosco Pesquero,
Umbertina Conti Reed,
Mariz Vainzof
et al.
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Short communication
(In Press Corrected Proof)
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24 January 2012 |
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Exome sequencing identifies KIAA1377 and C5orf42 as susceptibility genes for monomelic amyotrophy
DOI: 10.1016/j.nmd.2011.11.006
Abstract: Precise topographic localization, predominance in males mostly of Asian origin, and existence of some familial cases suggest a genetic background for monomelic amyotrophy. To identify suscep...
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Young-Min Lim,
InSong Koh,
Young-Mi Park,
Jae-Jung Kim,
Dae-Seong Kim,
Hyo-Jin Kim,
Kyu-Heum Baik,
Hye-Yeon Choi,
Gap-Seok Yang,
Eva Also-Rallo,
Eduardo F. Tizzano,
Josep Gamez,
Kiejung Park,
Han-Wook Yoo,
Jong-Keuk Lee,
Kwang-Kuk Kim
et al.
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Full-length article
(In Press Corrected Proof)
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20 January 2012 |
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Rippling muscle disease and facioscapulohumeral dystrophy-like phenotype in a patient carrying a heterozygous CAV3 T78M mutation and a D4Z4 partial deletion: Further evidence for “double trouble” overlapping syndromes
DOI: 10.1016/j.nmd.2011.12.001
Abstract: We report the first case of a heterozygous T78M mutation in the caveolin-3 gene (CAV3) associated with rippling muscle disease and proximal myopathy. The patient displayed also bilateral win...
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Giulia Ricci,
Isabella Scionti,
Greta Alì,
Leda Volpi,
Virna Zampa,
Marina Fanin,
Corrado Angelini,
Luisa Politano,
Rossella Tupler,
Gabriele Siciliano
et al.
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Full-length article
(In Press Corrected Proof)
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16 January 2012 |
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182nd ENMC International Workshop: RYR1-related myopathies, 15–17th April 2011, Naarden, The Netherlands
DOI: 10.1016/j.nmd.2011.12.003
Twenty clinicians and basic scientists from 10 countries convened for the 182nd ENMC sponsored Workshop on RYR1-related myopathies from the 15th to the 17th of April 2011 in Naarden, The Netherlands. ...
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Heinz Jungbluth,
James J. Dowling,
Ana Ferreiro,
Francesco Muntoni
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Full-length article
(In Press Corrected Proof)
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09 January 2012 |
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186th ENMC International Workshop: Congenital myasthenic syndromes 24–26 June 2011, Naarden, The Netherlands
DOI: 10.1016/j.nmd.2011.12.004
The ENMC hosted a group of 22 participants including parents, clinicians and scientists involved in the care or research of congenital myasthenic syndromes (CMS) patients. These represented different ...
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Amina Chaouch,
David Beeson,
Daniel Hantaï,
Hanns Lochmüller
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Full-length article
(In Press Corrected Proof)
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09 January 2012 |
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Assessment of cardiac function in three mouse dystrophinopathies by magnetic resonance imaging
DOI: 10.1016/j.nmd.2011.10.025
Abstract: Lack of dystrophin results in skeletal muscle dystrophy and dilated cardiomyopathy in humans and animal models. To achieve a basic understanding of the natural development of cardiomyopathy ...
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Ingrid E.C. Verhaart,
Rianne J.M. van Duijn,
Brigit den Adel,
Arno A.W. Roest,
Jan J.G.M. Verschuuren,
Annemieke Aartsma-Rus,
Louise van der Weerd
et al.
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Full-length article
(In Press Corrected Proof)
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03 January 2012 |
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N-Acetylcysteine treatment of dystrophic mdx mice results in protein thiol modifications and inhibition of exercise induced myofibre necrosis
DOI: 10.1016/j.nmd.2011.11.007
Abstract: Oxidative stress is implicated as a factor that increases necrosis of skeletal muscles in Duchenne Muscular Dystrophy (DMD) and the dystrophic mdx mouse. Consequently, drugs that minimize ox...
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Jessica R. Terrill,
Hannah G. Radley-Crabb,
Miranda D. Grounds,
Peter G. Arthur
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Full-length article
(In Press Corrected Proof)
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29 December 2011 |
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A Becker myotonia patient with compound heterozygosity for CLCN1 mutations and Prinzmetal angina pectoris
DOI: 10.1016/j.nmd.2011.10.024
Abstract: Becker myotonia is a recessive muscle disease with prevalence of >1:50,000. It is caused by markedly reduced function of the chloride channel encoded by CLCN1. We describe a Polish patien...
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Daniel Zielonka,
Karin Jurkat-Rott,
Paweł Stachowiak,
Anna Bryl,
Jerzy T. Marcinkowski,
Frank Lehmann-Horn
et al.
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Short communication
(In Press Corrected Proof)
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26 December 2011 |
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A missense mutation in the skeletal muscle chloride channel 1 (CLCN1) as candidate causal mutation for congenital myotonia in a New Forest pony
DOI: 10.1016/j.nmd.2011.10.001
Abstract: A 7-month-old New Forest foal presented for episodes of recumbency and stiffness with myotonic discharges on electromyography. The observed phenotype resembled congenital myotonia caused by ...
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Inge D. Wijnberg,
Marta Owczarek-Lipska,
Roberta Sacchetto,
Francesco Mascarello,
Francesco Pascoli,
Walter Grünberg,
Johannes H. van der Kolk,
Cord Drögemüller
et al.
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Short communication
(In Press Corrected Proof)
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26 December 2011 |
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Acid phosphatase-positive globular inclusions is a good diagnostic marker for two patients with adult-onset Pompe disease lacking disease specific pathology
DOI: 10.1016/j.nmd.2011.11.003
Abstract: Diagnosis of adult-onset Pompe disease is sometimes challenging because of its clinical similarities to muscular dystrophy and the paucity of disease-specific vacuolated fibers in the skelet...
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Rie S. Tsuburaya,
Kazunari Monma,
Yasushi Oya,
Takahiro Nakayama,
Tokiko Fukuda,
Hideo Sugie,
Yukiko K. Hayashi,
Ikuya Nonaka,
Ichizo Nishino
et al.
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Short communication
(In Press Corrected Proof)
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23 December 2011 |
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Multiple exon skipping strategies to by-pass dystrophin mutations
DOI: 10.1016/j.nmd.2011.10.007
Abstract: Manipulation of dystrophin pre-mRNA processing offers the potential to overcome mutations in the dystrophin gene that would otherwise lead to Duchenne muscular dystrophy. Dystrophin mutation...
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Carl F. Adkin,
Penelope L. Meloni,
Susan Fletcher,
Abbie M. Adams,
Francesco Muntoni,
Brenda Wong,
Steve D. Wilton
et al.
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Full-length article
(In Press Corrected Proof)
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19 December 2011 |
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Best practice guidelines on genetic diagnostics of Facioscapulohumeral muscular dystrophy: Workshop 9th June 2010, LUMC, Leiden, The Netherlands
DOI: 10.1016/j.nmd.2011.09.004
During the 171st European Neuromuscular Centre international workshop Standards of care and management of facioscapulohumeral muscular dystrophy (FSHD) in January 2010 , it was concluded that there wa...
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Richard J.L.F. Lemmers,
Suzanne O’Shea,
George W. Padberg,
Peter W. Lunt,
Silvère M. van der Maarel
et al.
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Full-length article
(In Press Corrected Proof)
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19 December 2011 |
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Focal myositis – A neurogenic phenomenon?
DOI: 10.1016/j.nmd.2011.10.023
Abstract: We report four cases of focal myositis. The patients, three men and one woman, had painful muscle hypertrophy, affecting four different sites. MRI confirmed the muscle enlargement and oedema...
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Hanne Marie Bøe Lunde,
Geir Olve Skeie,
Anne K. Bertelsen,
Bjørn Karlsen,
Hrvoje Miletic,
Sigurd Lindal,
Nils J. Brautaset,
Laurence A. Bindoff
et al.
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Short communication
(In Press Corrected Proof)
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12 December 2011 |
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Tubular aggregates in skeletal muscle: Just a special type of protein aggregates?
DOI: 10.1016/j.nmd.2011.10.005
Abstract: Tubular aggregates are inclusions, usually found in type II muscle fibers and in males, consisting of regular arrays of tubules derived from the sarcoplasmic reticulum. Tubular aggregates ar...
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Stefano Schiaffino
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Short communication
(In Press Corrected Proof)
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12 December 2011 |
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X-linked myotubular myopathy due to a complex rearrangement involving a duplication of MTM1 exon 10
DOI: 10.1016/j.nmd.2011.11.004
Abstract: X-linked myotubular myopathy is a predominantly severe congenital myopathy with central nuclei on muscle biopsy due to mutations in the MTM1 gene encoding myotubularin. We report a boy with ...
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N. Trump,
T. Cullup,
J.B.G.M. Verheij,
A. Manzur,
F. Muntoni,
S. Abbs,
H. Jungbluth
et al.
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Short communication
(In Press Corrected Proof)
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12 December 2011 |
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Density, calibre and ramification of muscle capillaries are altered in a mouse model of severe spinal muscular atrophy
DOI: 10.1016/j.nmd.2011.10.021
Abstract: Spinal muscular atrophy (SMA) is traditionally described and characterised as a disease of the neuromuscular system. Recently, the vascular system has been implicated in SMA pathogenesis, bu...
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E. Somers,
Z. Stencel,
T.M. Wishart,
T.H. Gillingwater,
S.H. Parson
et al.
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Full-length article
(In Press Corrected Proof)
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09 December 2011 |
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5th Annual Dysferlin Conference 11–14 July 2011, Chicago, Illinois, USA
DOI: 10.1016/j.nmd.2011.10.014
The fifth Annual Dysferlin Conference, sponsored and organized by the Jain Foundation, was held from July 11 to 14, 2011 in Chicago, Illinois. Participants included 34 speakers, 31 poster presenters, ...
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Douglas E. Albrecht,
Laura E. Rufibach,
Bradley A. Williams,
Nilah Monnier,
Esther Hwang,
Plavi Mittal
et al.
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Full-length article
(In Press Corrected Proof)
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09 December 2011 |
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Correlation between muscle involvement, phenotype and D4Z4 fragment size in facioscapulohumeral muscular dystrophy
DOI: 10.1016/j.nmd.2011.10.018
Abstract: This study aimed to evaluate muscle involvement pattern and correlate the lesions on muscle imaging with clinical features and D4Z4 fragment size in 24 patients with facioscapulohumeral musc...
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Chien-Hua Wang,
Mana Leung,
Wen-Chen Liang,
Tysh-Jyi Hsieh,
Tai-Heng Chen,
Yuh-Jyh Jong
et al.
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Full-length article
(In Press Corrected Proof)
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09 December 2011 |
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High cardiovascular morbidity and mortality in myofibrillar myopathies due to DES gene mutations: a 10-year longitudinal study
DOI: 10.1016/j.nmd.2011.10.019
Abstract: To determine incidence and type of major cardiac adverse events in patients with mutated desmin (DES) gene, we retrospectively reviewed baseline medical information, and examined the long-te...
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Karim Wahbi,
Anthony Béhin,
Philippe Charron,
Murielle Dunand,
Pascale Richard,
Christophe Meune,
Patrick Vicart,
Pascal Laforêt,
Tanya Stojkovic,
Henri Marc Bécane,
Thierry Kuntzer,
Denis Duboc
et al.
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Full-length article
(In Press Corrected Proof)
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07 December 2011 |
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Sleep breathing disorders in 40 Italian patients with Myotonic dystrophy type 1
DOI: 10.1016/j.nmd.2011.08.010
Abstract: The aim of this study was to estimate the prevalence and nature of sleep breathing disorders in Myotonic dystrophy type 1 (DM1). We wanted to determine whether there is a relationship betwee...
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Alessandro Pincherle,
Vincenzo Patruno,
Paola Raimondi,
Sabrina Moretti,
Ambra Dominese,
Filippo Martinelli-Boneschi,
Maria Barbara Pasanisi,
Eleonora Canioni,
Franco Salerno,
Francesco Deleo,
Roberto Spreafico,
Renato Mantegazza,
Flavio Villani,
Lucia Morandi
et al.
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Full-length article
(In Press Corrected Proof)
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05 December 2011 |
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Quantitative muscle ultrasound is a promising longitudinal follow-up tool in Duchenne muscular dystrophy
DOI: 10.1016/j.nmd.2011.10.020
Abstract: Responsive outcome measures are needed to follow the disease status of Duchenne muscular dystrophy (DMD) patients, as new therapeutic approaches become available for affected boys. Quantitat...
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Merel Jansen,
Nens van Alfen,
Maria W.G. Nijhuis van der Sanden,
Johannes P. van Dijk,
Sigrid Pillen,
Imelda J.M. de Groot
et al.
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Full-length article
(In Press Corrected Proof)
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02 December 2011 |
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Clinical features and new molecular findings in muscle phosphofructokinase deficiency (GSD type VII)
DOI: 10.1016/j.nmd.2011.10.022
Abstract: Muscle phosphofructokinase (PFKM) deficiency, a rare disorder of glycogen metabolism also known as glycogen storage disease type VII (GSDVII), is characterized by exercise intolerance, myalg...
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Olimpia Musumeci,
Claudio Bruno,
Tiziana Mongini,
Carmelo Rodolico,
M’hammed Aguennouz,
Emanuele Barca,
Angela Amati,
Denise Cassandrini,
Luigi Serlenga,
Giuseppe Vita,
Antonio Toscano
et al.
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Full-length article
(In Press Corrected Proof)
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02 December 2011 |
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185th ENMC International Workshop: Stem/precursor cells as a therapeutic strategy for muscular dystrophies 3–5 June 2011, Naarden, The Netherlands
DOI: 10.1016/j.nmd.2011.09.008
The 185th ENMC Workshop on stem/precursor cells as a therapeutic strategy for muscular dystrophies was held at Naarden, The Netherlands, on 3–5 June 2011. Sixteen participants from seven countries (Un...
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Ketan Patel,
Jennifer Morgan
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Full-length article
(In Press Corrected Proof)
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30 November 2011 |
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Myasthenic tongue
DOI: 10.1016/j.nmd.2011.10.006
In September 2010, a 55year-old male patient presented with fixed severe facial weakness, nasal speech, dysarthria and marked tongue atrophy with central furrowing, but good muscle bulk of the lateral...
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Vasiliki Zouvelou,
Michael Rentzos,
Panagiotis Toulas,
Ioannis Evdokimidis
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Short communication
(In Press Corrected Proof)
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28 November 2011 |
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A rapid immunohistochemical test to distinguish congenital myotonic dystrophy from X-linked myotubular myopathy
DOI: 10.1016/j.nmd.2011.10.002
Abstract: Severe forms of myotubular myopathy (MTM) and congenital myotonic dystrophy type 1 (CDM), both present as floppy infants with hypotonia, respiratory failure and bulbar insufficiency. Muscle ...
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Caroline A. Sewry,
Ros C.M. Quinlivan,
Waney Squier,
Glenn E. Morris,
Ian Holt
et al.
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Full-length article
(In Press Corrected Proof)
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23 November 2011 |
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Myopathy in a woman and her daughter associated with a novel splice site MTM1 mutation
DOI: 10.1016/j.nmd.2011.10.010
Abstract: We have investigated a woman and her daughter with an early onset, slowly progressive myopathy. Muscle biopsy showed in both cases severe atrophy with marked fatty replacement. Frequent fibe...
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Carola Hedberg,
Christopher Lindberg,
Gyöngyvér Máthé,
Ali-Reza Moslemi,
Anders Oldfors
et al.
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Full-length article
(In Press Corrected Proof)
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21 November 2011 |
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Screening for mutations in Spanish families with myotonia. Functional analysis of novel mutations in CLCN1 gene
DOI: 10.1016/j.nmd.2011.10.013
Abstract: Myotonia congenita is an inherited muscle disorder caused by mutations in the CLCN1 gene, a voltage-gated chloride channel of skeletal muscle. We have studied 48 families with myotonia, 32 o...
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María J. Mazón,
Francisco Barros,
Pilar De la Peña,
Juan F. Quesada,
Adela Escudero,
Ana M. Cobo,
Samuel I. Pascual-Pascual,
Eduardo Gutiérrez-Rivas,
Encarna Guillén,
Javier Arpa,
Pilar Eraso,
Francisco Portillo,
Jesús Molano
et al.
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Full-length article
(In Press Corrected Proof)
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17 November 2011 |
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SETX gene mutation in a family diagnosed autosomal dominant proximal spinal muscular atrophy
DOI: 10.1016/j.nmd.2011.09.006
Abstract: Autosomal dominant proximal spinal muscular atrophy (ADSMA) is a rare disorder with unknown gene defects in the majority of families. Here we describe a family where the diagnosis of juvenil...
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Sabine Rudnik-Schöneborn,
Larissa Arning,
Jörg T. Epplen,
Klaus Zerres
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Short communication
(In Press Corrected Proof)
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16 November 2011 |
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Long-term follow-up of patients with congenital myasthenic syndrome caused by COLQ mutations
DOI: 10.1016/j.nmd.2011.09.002
Abstract: Congenital myasthenic syndromes (CMS) are clinically and genetically heterogeneous inherited disorders characterized by impaired neuromuscular transmission. Mutations in the acetylcholineste...
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I. Wargon,
P. Richard,
T. Kuntzer,
D. Sternberg,
S. Nafissi,
K. Gaudon,
A. Lebail,
S. Bauche,
D. Hantaï,
E. Fournier,
B. Eymard,
T. Stojkovic
et al.
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Full-length article
(In Press Corrected Proof)
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16 November 2011 |
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Three cases of myasthenia gravis from one family with variations in clinical features and serum antibodies
DOI: 10.1016/j.nmd.2011.10.004
Abstract: Myasthenia gravis, an autoimmune disorder affecting neuromuscular transmission, is mainly sporadic while familial cases are very rare. Usually familial myasthenia gravis cases have uniform c...
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Yuping Chen,
Wei Wang,
Dongning Wei,
Li Yang
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Short communication
(In Press Corrected Proof)
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15 November 2011 |
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Transgenic inactivation of murine myostatin does not decrease the severity of disease in a model of Spinal Muscular Atrophy
DOI: 10.1016/j.nmd.2011.10.012
Abstract: Spinal Muscular Atrophy (SMA) is a devastating neurodegenerative disease and is a leading genetic cause of infantile death. SMA is caused by the homozygous loss of Survival Motor Neuron-1 (S...
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Hansjörg Rindt,
Desire M. Buckley,
Spencer M. Vale,
Megan Krogman,
Ferrill F. Rose,
Michael L. Garcia,
Christian L. Lorson
et al.
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Full-length article
(In Press Corrected Proof)
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14 November 2011 |
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A critical Smn threshold in mice dictates onset of an intermediate spinal muscular atrophy phenotype associated with a distinct neuromuscular junction pathology
DOI: 10.1016/j.nmd.2011.09.007
Abstract: Spinal muscular atrophy (SMA) is caused by mutations/deletions within the SMN1 gene and characterized by loss of lower motor neurons and skeletal muscle atrophy. SMA is clinically heterogene...
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Mélissa Bowerman,
Lyndsay M. Murray,
Ariane Beauvais,
Bruno Pinheiro,
Rashmi Kothary
et al.
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Full-length article
(In Press Corrected Proof)
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09 November 2011 |
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Blood glutathione decrease in subjects carrying lamin A/C gene mutations is an early marker of cardiac involvement
DOI: 10.1016/j.nmd.2011.09.005
Abstract: Dominant inherited Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B are due to mutations in the LMNA gene encoding lamin A/C and present similar life-threatening ...
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Christophe Meune,
Lara Khouzami,
Karim Wahbi,
Philippe Caramelle,
Valérie Decostre,
Gisèle Bonne,
Françoise Pecker
et al.
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Full-length article
(In Press Corrected Proof)
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09 November 2011 |