bluuebunny/crossref_metadata_2025 huggingface dataset:
A multi‐institutional study of immunohistochemical investigation for the roles of cyclin D1 and E‐cadherin in superficial squamous cell carcinoma of the esophagus*
Authors: Shiozaki Hitoshi , Doki Yuichiro , Yamana Hideaki , Isono Kaichi
Published: October 2004

Background and Objectives

Aiming to clarify and possibly extend indications for minimally invasive treatment, we characterized superficial esophageal cancers (SEC) with respect to biologic properties regulating malignant potential.

Methods

Surgical specimens obtained at eight cancer institutes from 222 Japanese patients with SEC (all squamous cell carcinomas) were investigated immunohistochemically for expression of cyclin D1 and E‐cadherin.

Results

Perturbations of cyclin D1 (overexpression) and E‐cadherin (reduced expression) were observed in 37.6% (68 of 181) and 39.9% (71 of 178) of SEC patients. E‐cadherin expression was more frequently reduced in cancers that invaded the submucosal layer, while cyclin D1 overexpression was constant, irrespective of depth of invasion. Overexpression of cyclin D1 was more frequent in poorly differentiated squamous cell carcinomas, while E‐cadherin did not vary according to histologic differentiation. Lymph node metastasis, the only independent postoperative prognostic factor in these patients, occurred in only 4.8% of mucosal cancers (2 of 42), but in 51.1% of submucosal cancers (92 of 180). However, neither cyclin D1 nor E‐cadherin status affected lymph node metastasis.

Conclusion

Both E‐cadherin and cyclin D1 play important roles in esophageal carcinogenesis, but neither can be used to identify patients who do not require lymph node dissection and might be treated by endoscopic mucosal resection. J. Surg. Oncol. 2002;79:166–173. © 2002 Wiley–Liss, Inc.
Multiple‐Well, multiple‐path unimolecular reaction systems. II. 2‐methylhexyl free radicals
Authors: Barker John R. , Ortiz Nicolas F.
Published: October 2004

Vibrationally excited 2‐methylhexyl radicals formed by shock wave activation or by chemical activation can isomerize by multiple pathways to form any of six stable isomers, can fragment by multiple CH and CC bond fission pathways, and can be collisionally stabilized. Master equation simulations of chemical activation and of shock wave activation are used to explore the generic behavior of this complicated coupled system. Selecting the argon pressure in chemical activation systems that produce the 2‐methyl‐1‐hexyl radical isomer (1) can control the yield of specific isomers. Shock heating of 1 also shows a highly regular sequence of isomer formation. This regular behavior is because the first isomerization steps are faster than subsequent steps. Other radical isomers, such as 2‐methyl‐3‐hexyl (3), do not show such regular behavior, because the first isomerization step is slower than subsequent steps. Incubation and unimolecular rate‐constant fall‐off are observed in the shock wave simulations. The unimolecular rate‐constant fall‐off for the coupled system produces low‐pressure limiting rate constants proportional to [M]n, where n can be greater than unity. The fact that n can be greater than unity is a natural feature of multichannel coupled unimolecular reaction systems, but detection of the effect in experiments may be very demanding. © 2001 John Wiley & Sons, Inc. Int J Chem Kinet 33: 246–261, 2001
Effect of diode laser irradiation on the survival rate of gingival fibroblast cell cultures
Authors: Kreisler M. , Daubländer M. , Willershausen‐Zönnchen B. , d'Hoedt B.
Published: October 2004

Background and Objective

The present study is part of a basic research program investigating the cellular effects of the GaAlAs‐diode laser with a wavelength of 810 nm on human periodontal tissues. The aim of the investigation was to evaluate the effects of the laser treatment on the survival rate of human gingival fibroblasts (HGF) in monolayer cell culture at different power settings and durations.

Study Design/Materials and Methods

HGF were obtained from a human gingival connective tissue explant. Cells were cultured on 24‐well tissue culture plates. One hundred and thirty multi well cell cultures were laser treated. The variable parameters were power output (0.5–2.5 W) and exposure time (60–240 seconds per well). The cultures were analyzed by means of trypan blue staining, and the cell numbers counted under a light microscope. Photographs before and after irradiation were taken and evaluated.

Results

Depending on different settings, the laser irradiation caused significant (P < 0.05, t‐test) reductions of cell numbers. Exposure time was more relevant to this phenomenon than the power output. Linear regression analysis revealed no unambiguous correlation between power output and cell death, when exposure time was kept constant.

Conclusions

When used for periodontal pocket decontamination, the laser beam may cause damage to collateral periodontal tissues, if the power setting and the duration of the treatment are not adequate. Further investigation, especially with regard to the effects on hard tissue and microorganisms, are needed to give a secure recommendation for irradiation parameters at pocket decontamination. Lasers Surg. Med. 39:445–450, 2001. © 2001 Wiley‐Liss, Inc.
Doubly curved aperture antenna shape prediction with the use of network‐based predictors
Authors: Punhani Amitesh , Washington Gregory , Theunissen Wilhelmus H.
Published: October 2004

The main objective of this work is to predict the shape of an antenna subreflector that produces a desired radiation far‐field pattern by utilizing artificial intelligence and other methodologies. In this study the size of the radiation beam is kept constant while it is steered throughout the domain. Three different methodologies or constructs are used to develop this model: Neural networks, batch least squares, and recursive least squares. The accuracy of a method is measured by the sum‐squared error of the training examples. During training the variables inside of the constructs are varied, so that the predicted aperture shape matches the actual shape. The networks predicted the antenna reflector shape at an average accuracy of over 97%, the maximum being 99.78%. © 2002 Wiley Periodicals, Inc. Microwave Opt Technol Lett 33: 156–163, 2002; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/mop.10263
Electromagnetic properties measurements of ferromagnetic materials using microstrip
Authors: Hinojosa J. , Cottevieille D. , Le Mehaute A. , Faucon L.
Published: October 2004

A broadband technique for the simultaneous measurement of the complex permittivity and permeability of film‐shaped ferromagnetic materials is presented. Complex permittivity and permeability are computed from S‐parameter measurements under a constant exterior magnetic field of microstrip transmission lines used as cells. Moreover, a simple and fast technique that allows to the measurement of magnetic materials with a low permeability is presented. The measurement bench uses a vector network analyzer, a high‐quality on‐microstrip test fixture, and an electromagnet. Measurements in the 0.05–20 GHz range show the validity of these methods. © 2002 Wiley Periodicals, Inc. Microwave Opt Technol Lett 33: 176–180, 2002; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/mop.10269
Extension of transmission‐line model for longitudinal‐slot dielectric‐filled waveguide
Authors: Chainon S. , Himdi M.
Published: October 2004

This Letter presents a well‐suited model for the analysis of longitudinal‐slot dielectric‐filled waveguide antennas by extending the transmission‐line model used for printed slot antennas. The found slot admittance is calculated on the waveguide plane thanks to a transformation ratio that corresponds to the current discontinuity, taking into account the permittivity of the antenna. The results obtained with the use of this method are first compared to a commercial software based on the FDTD method, for two dielectrics, with different slot offsets, in the millimeter‐wave band. Second, the method is compared with a measurement. © 2002 Wiley Periodicals, Inc. Microwave Opt Technol Lett 33: 180–183, 2002; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/mop.10270
Analysis of current‐crowding effects in RF‐MEMS spiral inductors by simple equivalent circuits
Authors: Yaojiang Zhang , Haibo Long , Zhenghe Feng
Published: October 2004

The metal loss of multiturn spiral inductors is known to increase dramatically under current‐crowding effects at high frequencies. An equivalent circuit is derived to simulate this current crowding, including skin and proximity effects. An efficient formula is derived to calculate self‐ and mutual coupling of currents in irregular hexahedrons. Possible approaches for the design of high‐Q RF‐MEMS spiral inductors are discussed. © 2002 Wiley Periodicals, Inc. Microwave Opt Technol Lett 33: 218–221, 2002; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/mop.10280
Dual‐mode bandpass filters using triangular ring resonators with coupling stubs
Authors: Weng Min Hung , Hung Cheng‐Yuan , Chen Wu‐Nan
Published: October 2004

A novel dual‐mode bandpass filter using a microstrip triangular ring resonator with coupling stubs has been designed and tested. The basic configuration of this novel dual‐mode filter is a triangular ring resonator. Quasi‐L‐shaped I/O arms used as coupling stubs for enhanced coupling are nonorthogonally and symmetrically separated on each corner of the triangular resonator, and a perturbation element is introduced within the resonator at a symmetrical location. The effects of the lengths of the coupling stubs and the width of the perturbation element are studied. New filters having narrower or wider bandwidth and transmission zeros are presented. The proposed filter responses are in good agreement with the simulations and experiments. © 2004 Wiley Periodicals, Inc. Microwave Opt Technol Lett 43: 512–515, 2004; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/mop.20519
Analysis of TP53 germline mutations in pediatric tumor patients using DNA microarray‐based sequencing technology
Authors: Schaefer Karl‐Ludwig , Wai Daniel , Poremba Christopher , Diallo Raihanatou , Boecker Werner , Dockhorn‐Dworniczak Barbara
Published: October 2004

Background

Whereas in sporadic human malignancies mutations of the TP53 tumor‐suppressor gene occur in cancers of almost every organ and histologic subtype, patients with an inborn TP53 defect are at high risk to develop, in particular, soft tissue and bone sarcomas, brain tumors, leukaemias, adrenocortical tumors, and breast cancer. To demonstrate the usefulness of microarray technology applied to TP53 sequencing in pediatric tumors, we investigated young patients suffering from tumors typical of the Li‐Fraumeni context who were, therefore, candidates for harboring inborn defects in tumor‐suppressor genes.

Procedure

Six individuals were studied, including typical Li‐Fraumeni patients as well as patients without any family history of cancer. DNA samples were independently analyzed for TP53 mutations by GeneChip® and standard automated laser fluorescence (ALF®) sequencing technology.

Results

The tumor and corresponding constitutional DNA samples clearly showed identical mutations which were confirmed by ALF sequencing. All coding exons (exons 2–11) of TP53 were analyzed simultaneously. The entire sequencing procedure and data analysis was carried out within 24 hr.

Conclusions

The GeneChip® TP53‐sequencing assay may be feasible for routine molecular genetic diagnostics in determining the TP53 status of childhood‐tumor patients and in enabling a disease management based on the genetic background of the individual. Med. Pediatr. Oncol. 2002;38:247–253. © 2002 Wiley‐Liss, Inc.
Predictive value of clinical evaluation in the follow‐up of children with a brain tumor
Authors: de Graaf Nanko , Hew Joffre M. , Fock Johanna M. , Kamps Willem A. , de Graaf Siebold S.N.
Published: October 2004

Background

During follow‐up of children with a brain tumor, traditionally surveillance‐imaging studies are done in addition to clinical evaluations. The purpose of this study was to determine the role of clinical evaluations by a multidisciplinary team for the detection of recurrent tumor.

Procedure

We retrospectively assessed the predictive value of clinical evaluation, using subsequent neuroimaging as the gold standard. Ninety‐eight children with a newly diagnosed primary brain tumor were included in the study. In these patients, 393 imaging studies were done, 75 because of clinical suspicion of recurrence, and 318 for routine surveillance only.

Results

In 28 of these 75 imaging studies on clinical indications, a radiologic diagnosis of recurrence was made. Only 9 out of 318 routine surveillance imaging studies resulted in a nunexpected diagnosis of recurrence. Thus, the overall positive predictive value of clinical evaluation was 37%; the overall negative predictive value was 97%. The negative predictive values for specific brain tumors varied from 91% (optical glioma) to 99% (primitive neuroectodermal tumors).

Conclusions

An accurate multidisciplinary clinical evaluation has a very high negative predictive value. Consequently, the added value of surveillance imaging studies is limited. The role of such studies during the follow‐up of children with brain tumors should be reconsidered. Med. Pediatr. Oncol. 2002;38:254–257. © 2002 Wiley‐Liss, Inc.
Opsoclonus‐myoclonus‐ataxia syndrome in neuroblastoma: Histopathologic features–A report from the children's cancer group†
Authors: Cooper Robert , Khakoo Yasmin , Matthay Katherine K. , Lukens John N. , Seeger Robert C. , Stram Daniel O. , Gerbing Robert B. , Nakagawa Atsuko , Shimada Hiroyuki
Published: October 2004

Background

Opsoclonus‐myoclonus‐ataxia (OMA) is a paraneoplastic syndrome that occurs in about 2–3% of all cases of neuroblastoma. The histopathologic characteristics of neuroblastoma tumors associated with this syndrome were evaluated in a series of cases and controls.

Procedure

Pathology slides from a total of 54 neuroblastoma tumors were reviewed blindly. They included 13 tumors associated with opsoclonus‐myoclonus and 41 age‐ and stage‐matched controls. All tumors were classified into either the favorable (FH) or unfavorable histology (UH) group according to the International Neuroblastoma Pathology Classification (the Shimada system). Grade of lymphocytic infiltration was evaluated and presence or absence of lymphoid follicles was recorded in the individual tumor tissues.

Results

Twelve of 13 cases with opsoclonus–myoclonus were in the FH group. Twelve of 13 cases had diffuse (found in every section prepared from the multiple portions of the primary tumor) and extensive (occupying more than 50% of a single of multiple microscopic fields with × 100 magnification) lymphocytic infiltration with lymphoid follicles. Of the 41 control cases (27 FH and 14 UH tumors), 18 had focal areas of lymphocytic infiltration and six showed lymphoid follicles, but none had diffuse or extensive infiltration in their primary tumors.

Conclusions

Diffuse and extensive lymphocytic infiltration with lymphoid follicles is a characteristic histologic feature of neuroblastic tumors with opsoclonus–myoclonus. This observation suggests an immune‐mediated mechanism for this rare paraneoplastic syndrome. Med. Pediatr. Oncol. 36:623–629, 2001. © 2001 Wiley‐Liss, Inc.
Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood
Authors: Vujanić Gordan M. , Sandstedt Bengt , Harms Dieter , Kelsey Anna , Leuschner Ivo , de Kraker Jan ,   on behalf of the SIOP Nephroblastoma Scientific Committee
Published: October 2004

The previous International Society of Paediatric Oncology (SIOP) trials and studies recognized three prognostic groups of renal tumors of childhood: low risk, intermediate risk, and high risk tumors, which were further defined in the SIOP (Stockholm) Working Classification of Renal Tumors of Childhood (1994). The results of the latest SIOP Trials and Studies showed that certain histological features which remain after preoperative chemotherapy, such as blastema, are of prognostic significance while others are not. Therefore, in the next SIOP Trials and Study a revised classification of renal tumors will be followed. It still recognizes the three tumor risk groups with different types in each of them, but for treatment purposes, only three major types of nephroblastoma need to be recognized: completely necrotic (low risk tumor), blastemal (high risk tumor), and others (intermediate risk tumors). Patients will be treated according to tumor histology and stage. Trials which include preoperative chemotherapy have shown that the presence of necrotic tumor or chemotherapy induced changes in the renal sinus or perirenal fat can be ignored for distinguishing between stage I and II, but if present at resection margins or lymph nodes, it should be regarded as stage III. Prognostic significance of all histological component of Wilms tumors will be studied prospectively in the new trial. Med Pediatr Oncol 2002;38:79–82. © 2002 Wiley‐Liss, Inc.
Supratentorial embryonal tumors in children under 5 years of age: An SFOP study of treatment with postoperative chemotherapy alone
Authors: Marec‐Berard P. , Jouvet A. , Thiesse P. , Kalifa C. , Doz F. , Frappaz D.
Published: October 2004

Background

To determine the effectiveness of multiagent chemotherapy as sole post‐operative treatment of supratentorial central nervous system (CNS) embryonal tumors in young children.

Procedure

The data of 25 children under 5 years of age diagnosed with supratentorial embryonal tumors (17 primitive neuroectodermal tumors, four pinealoblastomas, and four medulloepitheliomas) treated exclusively by postoperative chemotherapy (CT) between 1990 and 1997 were reviewed.

Results

Fifteen tumors were hemispheric and 10 were deeply seated. Four children presented with disseminated leptomeningeal disease. Total resection was performed in nine patients, subtotal in 9, partial in 3, and a diagnostic biopsy only in 2. Two children did not undergo surgery. Twenty‐four children relapsed with a median time of 5.5 months. The median overall survival was 12 months, and the 2‐, and 5‐ year survivals were 30 and 14y, respectively. The 2‐ year disease‐free survival was 4%. There was a significantly worse prognosis in patients undergoing incomplete resection and in the group with deeply situated tumors. Four relapses were treated by second surgery followed by high‐dose chemotherapy and radiotherapy. Two of them remain in CR2, and all these children are free of late sequelae.

Conclusions

CT alone failed to maintain disease‐free survival in most of the children, although, disease progression was delayed to some extent. Children under 5 years with supratentorial embryonal tumors should undergo total surgical resection if possible. Med Pediatr Oncol 2002;38:83–90. © 2002 Wiley‐Liss, Inc.
A randomised study of prophylactic G‐CSF following MRC UKALL XI intensification regimen in childhood ALL and T‐NHL
Authors: Little M.A. , Morland B. , Chisholm J. , Hole A. , Shankar A. , Devine T. , Easlea D. , Meyer L.C. , Pinkerton C.R.
Published: October 2004

Background

Despite the current widespread use of prophylactic G‐CSF in children with solid tumours and leukaemia, its effectiveness has not been clearly demonstrated. This randomised study evaluates the role of G‐CSF given after a 5‐day intensification block in children with acute lymphoblastic leukaemia (ALL).

Procedure

Forty‐six children with ALL or T‐Cell non‐Hodgkins lymphoma (NHL) treated on MRC ALL 97, UKALL XI or UKCCSG 9504 NHL protocols were randomised to receive granulocyte colony‐stimulating factor following either the first or the second block of intensive chemotherapy in a cross‐over study to determine if the prophylactic administration of G‐CSF could reduce the rate of readmission to hospital for management of febrile neutropenia.

Results

There was a statistically significant difference in the rate of hospital admission in the group receiving prophylaxis, with 34 of 46 being admitted, compared to 42 of 46 patients in the control arm (74 vs. 91%; P = 0.0386). There were no differences found in duration of hospital admission, haematological toxicity, neutrophil recovery or duration of supportive care between the two groups. There was no demonstrable cost benefit derived from the prophylactic administration of G‐CSF.

Conclusions

This study shows that the prophylactic administration of G‐CSF following intensification chemotherapy for childhood ALL and T‐NHL produces a significant reduction in the rate of readmission to hospital for the management of febrile neutropenia. Med Pediatr Oncol 2002;38:98–103. © 2002 Wiley‐Liss, Inc.
Familial amyotrophic lateral sclerosis
Authors: Hand Collette K. , Rouleau Guy A.
Published: October 2004

The increasing complexity of the pathways implicated in the pathogenesis of familial amyotrophic lateral sclerosis (ALS) has stimulated intensive research in many directions. Genetic analysis of familial ALS has yielded six loci and one disease gene (SOD1), initially suggesting a role for free radicals in the disease process, although the mechanisms through which the mutant exerts toxicity and results in selective motor neuron death remain uncertain. Numerous studies have focused on structural elements of the affected cell, emphasizing the role of neurofilaments and peripherin and their functional disruption in disease. Other topics examined include cellular homeostasis of copper and calcium, particularly in the context of oxidative stress and the processes of protein aggregation, glutamate excitotoxicity, and apoptosis. It has become evident that there is considerable interplay between these mechanisms and, as the role of each is established, a common picture may emerge, enabling the development of more targeted therapies. This study discusses the main areas of investigation and reviews the findings. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 135–159, 2002 DOI 10.1002/mus.10000
Intermuscle differences in activation
Authors: Behm D.G. , Whittle J. , Button D. , Power K.
Published: October 2004

The objective of this study was to investigate differences within individual subjects in the ability to activate the quadriceps, plantar flexors (PF), dorsiflexors (DF), and elbow flexors (EF) during isometric contractions. Twelve male subjects performed submaximal and maximal voluntary isometric contractions, and maximal tetanic contractions were also induced by electrical stimulation. The interpolated twitch technique was used to gauge the extent of muscle inactivation or inability to produce maximum force. Measurements included torque output, absolute and relative rate of force development (RFD), and percentage of muscle inactivation. The quadriceps exceeded all other muscle groups in voluntary and tetanic torque output, voluntary absolute RFD, and absolute and relative tetanic RFD. The quadriceps also exceeded the PF and DF in voluntary relative RFD and had greater muscle inactivation (15.5%) than the EF (5.0%), PF (5.0%), and DF (1.3%). Although the higher RFD may suggest a higher percentage of type II fibers in the quadriceps, their higher threshold of recruitment leads to greater difficulty in fully activating the quadriceps. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 236–243, 2002 DOI 10.1002/mus.10008
Cosegregation of the mitochondrial DNA A1555G and G4309A mutations results in deafness and mitochondrial myopathy
Authors: Campos Y. , García A. , López A. , Jiménez S. , Rubio J.C. , Del Hoyo P. , Bustos F. , Martín M.A. , Cabello A. , Ricoy J.R. , Arenas J.
Published: October 2004

We report a patient with progressive external ophthalmoplegia (PEO), exercise intolerance, and deafness after aminoglycoside exposure, harboring two pathogenic mutations in her mtDNA: an A1555G in the 12S rRNA gene and a G4309A in the tRNAIle gene. Muscle histochemistry showed abundant ragged‐red fibers, and biochemistry revealed normal respiratory chain function. The A1555G mutation was homoplasmic in blood from the proband and from all maternal relatives. The G4309A mutation was abundant in the proband′s muscle, less abundant in her blood, still less abundant in the mother′s blood, and absent in blood from other maternal relatives. Family members were asymptomatic. Our data suggest that the former mutation resulted in aminoglycoside‐induced deafness and the latter caused PEO plus exercise intolerance. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 185–188, 2002 DOI 10.1002/mus.10012
Effects of leukemia inhibitory factor on rat skeletal muscles are modulated by clenbuterol
Authors: Gregorevic Paul , Williams David A. , Lynch Gordon S.
Published: October 2004

Leukemia inhibitory factor (LIF) is implicated in skeletal muscle regeneration, but the effect of exogenous LIF on uninjured muscles is not known. We tested the hypothesis that LIF administration would stimulate muscle hypertrophy, with an increased effect during clenbuterol‐induced fiber remodeling. Rats received daily injections of either saline or LIF, and either regular or clenbuterol‐supplemented drinking water for 4 weeks. In the slow‐twitch soleus muscles of LIF‐treated rats, specific force (sPo) and muscle fiber size were increased by ∼13% and ∼26%, respectively, compared to saline‐treated rats. In the soleus muscles of rats receiving LIF and clenbuterol, compared to rats receiving clenbuterol alone, maximum isometric tension (Po) was ∼19% greater. LIF alone did not affect the properties of fast‐twitch extensor digitorum longus (EDL) muscles, but in rats receiving LIF and clenbuterol, compared to clenbuterol alone, EDL fiber size and muscle mass were increased by ∼20% and ∼10%, respectively. The hypertrophic effects of exogenous LIF on uninjured skeletal muscles indicate that LIF may have application in the treatment of conditions characterized by muscle wasting. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 194–201, 2002 DOI 10.1002/mus.10015
Bent spine syndrome: Computed tomographic study and isokinetic evaluation
Authors: Laroche Michel , Ricq Geraldine , Delisle Marie‐Bernadette , Campech Michel , Marque Philippe
Published: October 2004

Primary acquired lumbar kyphosis of the adult, or bent spine syndrome, is probably secondary to late‐onset muscle dystrophy preferentially involving the paravertebral muscles. In order to better define the extent of this myopathy, we compared spinal computerized tomography scans and muscle force, power, and fatigability, assessed by isokinetic tests, of the spinal erector and pelvic and shoulder girdle muscles in 23 patients with primary acquired lumbar kyphosis (20 women and 3 men, mean age 72.8 years) and 15 matched control subjects. Patients had decreased spinal and shoulder muscle density. Force, power, and work done were reduced in their paravertebral muscles, whereas in the scapular and gluteus medius muscles only work done after a series of 20 rapid movements showed a decrease. Primary acquired lumbar kyphosis in the adult may be considered as a late‐onset girdle myopathy with subclinical involvement of the muscles of the pelvic and shoulder girdles. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 189–193, 2002 DOI 10.1002/mus.10017
Acquisition and expression of proximal and distal upper limb stimulus–response curves to transcranial magnetic stimulation
Authors: Ray Julian , McNamara Brian , Boniface Simon
Published: October 2004

Motor cortex stimulus–response (S/R) curves are an indication of cortical excitability and are of relevance to topographical mapping. The aims of this study were to compare two different methods of collecting data to construct a S/R curve for transcranial magnetic stimulation (TMS) in the upper limbs, to identify reliable summary statistics for the S/R curve, and to determine whether S/R curves predicted motor threshold. Motor evoked potentials (MEP) were obtained from biceps brachii (BB) and abductor pollicis brevis (APB) muscles at rest with a circular coil centered at the vertex. Motor threshold was determined using a validated protocol. MEPs were obtained with 1% increments in intensity or from the average of five trials at 5% increments. The S/R relationships were fitted to linear, S‐shaped, and Boltzmann functions. A linear function determined from the average of five trials accurately summarized our data (r2 from 0.6 to 0.9, P < 0.05, n = 8, for right APB and from 0.6 to 0.9, P < 0.05, n = 8 for right BB). The X‐axis intercept of the line determined using these methods fell between the upper and lower limits of motor threshold in all eight subjects. We propose that MEP values obtained at intervals of 5% averaged over five trials, fitted to a linear function provides a practical means of assessing the S/R characteristics of TMS for proximal and distal upper limb muscles. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 202–206, 2002 DOI 10.1002/mus.10020
Concomitant dermatomyositis and myasthenia gravis presenting with respiratory insufficiency
Authors: Van De Warrenburg Bart P.C. , Hengstman Gerald J.D. , Vos Pieter E. , Boerman Rudolph H. , Ter Laak Henk J. , Van Engelen Baziel G.M.
Published: October 2004

We report a 28‐year‐old woman with a history of chronic immune‐mediated hepatitis, in whom the simultaneous manifestation of dermatomyositis and myasthenia gravis resulted in severe neck extensor weakness and subacute respiratory insufficiency, followed by proximal muscle weakness and external ophthalmoplegia. Radiological signs of a thymoma were absent. The distinguishing clinical, electrophysiological, and biopsy findings are discussed. We suggest that an underlying immunoregulatory disorder was present, explaining the occurrence of three rare immune‐mediated diseases in one patient. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 293–296, 2002 DOI 10.1002/mus.10022
Mycophenolate mofetil in the therapy of polymyositis associated with a polyautoimmune syndrome
Authors: Schneider C. , Gold R. , Schäfers M. , Toyka K.V.
Published: October 2004

Mycophenolate mofetil 1.5 g daily (30 mg/kg body weight) was given to a patient with ankylosing spondylitis, ulcerative colitis, and severe refractory polymyositis after conventional treatment regimes had failed. No severe side effects occurred. Considerable improvement of clinical symptoms and electromyographic findings were seen within 6 months after the initiation of mycophenolate mofetil, allowing for tapering and discontinuation of methylprednisolone. Mycophenolate mofetil may be considered as an useful alternative in the treatment of polymyositis when standard therapeutic regimens fail. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 286–288, 2002 DOI 10.1002/mus.10026
Electrical stimulation factors in potentiation of human quadriceps femoris
Authors: Binder‐Macleod Stuart A. , Dean Jesse C. , Ding Jun
Published: October 2004

Potentiation is the enhancement of force seen after repetitive activation of skeletal muscle. The frequency and duration of stimulation, total number of pulses delivered to the muscle, and the peak forces or force‐time integrals produced by the stimulation all have been suggested to affect the degree of potentiation. The purpose of this study was to determine the effect of the electrical stimulation characteristics on the development of post‐activation potentiation. Eleven subjects were tested with five potentiating trains, including 12‐pulse 100‐HZ, 31‐HZ, 14‐HZ, and 5‐HZ, and 6‐pulse 14‐HZ trains. The potentiating trains differed in stimulation frequency, train duration, and total number of pulses. They also produced different peak forces and force time integrals from the activated muscles. Our results showed that the 12‐pulse 5‐HZ train produced about 50% less potentiation than the other four potentiating trains. At stimulation frequencies of 14 HZ or higher, the total number of pulses delivered to the muscle was the primary factor in potentiation development. Furthermore, peak force and force‐time integral had no effects on the rate or amount of potentiation. These results should help clinicians and researchers to design protocols that control for the effects of muscle potentiation. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 271–279, 2002 DOI 10.1002/mus.10027
Acceleration by MS‐818 of early muscle regeneration and enhanced muscle recovery after surgical transection
Authors: Sugiyama Noriyuki , Yoshimura Atsushi , Fujitsuka Chie , Iwata Hisashi , Awaya Akira , Mori Satoru , Yoshizato Hideo , Fujitsuka Noriaki
Published: October 2004

The synthesized pyrimidine compound MS‐818 has neurotrophic effects in several kinds of neuronal cells, but its effect with respect to muscle cells remains unknown. We therefore examined the effects of MS‐818 on regeneration for 12 weeks in a wounded area (damaged and gap areas) of cut muscle in adult rats. The right semitendinosus muscles of reated and control groups were severed and sutured at the belly and the left semitendinosus muscles were left intact. MS‐818 was administered intraperitoneally to the treated group at a dose of 5 mg/kg once daily. Control rats received an equal volume of physiological saline. A reference group underwent no surgical procedure. MS‐818 significantly increased the maximal isometric twitch tension (Tmax) compared to control and reference rats after week 4 (approximately 1.4‐fold control value; 0.6‐fold reference value). Northern blotting showed that MS‐818 enhanced myogenin mRNA expression to about 1.5‐fold above the control level at 2, 4, and 7 days after surgery. Immunohistochemical and histochemical studies showed significant enhancement in the treated group since myogenic cells expressed desmin and were positive for neonatal myosin, and the fiber diameters and numbers of premature myofibers and end plates were increased when compared with those in the control group. These results show that MS‐818 accelerated the proliferation and differentiation of activated satellite cells and the fusion of myotubes to form immature myofibers. At week 12, Tmax, fiber diameter, and number of end plates in the treatment group recovered 60, 85, and more than 100%, respectively, compared to the reference group. The mechanism of MS‐818 effects on the accelerated regeneration of cut muscle is discussed. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 218–229, 2002 DOI 10.1002/mus.10028
Analysis of motor pathway involvement in konzo using transcranial electrical and magnetic stimulation
Authors: Tshala‐Katumbay Desire , Eeg‐Olofsson Karin Edebol , Kazadi‐Kayembe Theodore , Tylleskär Thorkild , Fällmar Peo
Published: October 2004

To elucidate the involvement of motor pathways in konzo, 21 konzo subjects (mean age 22 years) underwent transcranial electrical stimulation (TES) in 1998. Fourteen konzo subjects (mean age 21 years) underwent transcranial magnetic stimulation (TMS) in 2000. Three subjects underwent both TES and TMS. Motor evoked potentials (MEPs) were recorded in the abductor pollicis brevis (APB) muscle with TES, and in the abductor digiti minimi (ADM) and tibialis anterior (TA) muscles with TMS. APB‐MEPs were normal in 2 of 21 subjects and absent in 9; central conduction time (CCT) was prolonged in 10. Resting ADM‐MEPs were absent in 9 of 14 subjects with clinically preserved upper limbs. Among these nine, seven subjects responded after facilitation. Most subjects (13 of 14) failed to show TA‐MEPs. Of the subjects who underwent both types of stimulation, one had normal TES‐MEP but abnormal ADM‐MEP with TMS. These findings suggest involvement of both corticomotoneurons and motor descending pathways in konzo. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 230–235, 2002 DOI 10.1002/mus.10029
Effects of voluntary activity on the excitability of motor axons in the peroneal nerve
Authors: Kuwabara Satoshi , Cappelen‐Smith Cecilia , Lin Cindy S.‐Y. , Mogyoros Ilona , Burke David
Published: October 2004

To investigate whether there are inter‐nerve differences in the extent and pattern of axonal excitability changes produced by voluntary contractions of tibialis anterior (TA) and abductor pollicis brevis (APB), threshold tracking was used to measure axonal excitability parameters [threshold, supernormality and strength‐duration time constant (τSD)] of peroneal and median motor axons in 11 healthy subjects. Maximal contractions for 1 min resulted in an increase in threshold, an increase in supernormality, a decrease in τSD and an increase in latency, all of which indicate axonal hyperpolarization. The increase in threshold was less in peroneal axons (18 ± 4%) than median axons (37 ± 6%, mean ± SEM, P < 0.001), and was accompanied by smaller absolute changes in latency, supernormality, and τSD. Peroneal axons had less supernormality at rest but a greater change in supernormality for the change in threshold. There were major contraction‐induced changes in the compound muscle action potential of TA but not that of APB. Voluntary contractions depress axonal excitability, but the changes are quantitatively different for motor axons innervating different muscles. There are three clinical implications. First, weakness and fatigue due to activity‐dependent conduction block may vary for different muscles, independent of disease severity, and therapeutic strategies to overcome activity‐dependent conduction block may not be equally effective for different muscles. Second, in motor control studies using the H reflex to document motoneuron excitability, a constant stimulus will not produce a constant neural volley if the stimulated axons have been activated by, for example, a voluntary contraction. Third, TA is probably not optimal for testing for activity‐dependent conduction block. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 176–184, 2002 DOI 10.1002/mus.10030
Assessment of contractile and noncontractile components in human skeletal muscle by magnetic resonance imaging
Authors: Holmbäck Anna Maria , Askaner Krister , Holtås Stig , Downham David , Lexell Jan
Published: October 2004

A magnetic resonance imaging (MRI) technique for the assessment of contractile and noncontractile components of human skeletal muscle is described, and the inter‐rater and intra‐rater test–retest reliability for repeated measurements from the same MR image are examined. Twenty cross‐sectional MR images from the right lower leg were obtained from 30 healthy young men and women (mean age 24.1 years, SD 3.3). The anatomical cross‐sectional area (aCSA; cm2), the cross‐sectional area of noncontractile components (Noncon; cm2), the contractile cross‐sectional area (cCSA = aCSA minus Noncon; cm2), and the relative amount of Noncon (%), of the ankle dorsiflexor muscle compartment were determined for each slice using a computer‐based image analysis system. Reliability for repeated measurements of the slice with the largest aCSA for the 30 subjects was analyzed by two raters on two different occasions. Inter‐rater reliability on both occasions, assessed by the intraclass correlation coefficient (ICC), was excellent for cCSA (ICC3.1 = 0.99) and Noncon (ICC3.1 > 0.82). Intra‐rater (between occasions) reliability was excellent for the two raters for measurements of cCSA (ICC1.1 = 0.99) and Noncon (ICC1.1 > 0.94). Bland and Altman analyses did not identify any clinically relevant bias in the measurements. Method errors were acceptable: within subjects coefficients of variation (CV) was less than 1.8% for cCSA and less than 16.3% for Noncon. It is concluded that repeated measurements of contractile and noncontractile components of the ankle dorsiflexor muscle compartment, obtained from the same MR image, are highly reliable. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 251–258, 2002 DOI 10.1002/mus.10031
Rapidly progressive amyloid polyneuropathy associated with a novel variant transthyretin serine 25
Authors: Yazaki Masahide , Yamashita Taro , Kincaid John C. , Scott John R. , Auger Raymond G. , Dyck Peter J. , Benson Merrill D.
Published: October 2004

We report a 52‐year‐old woman with a novel transthyretin (TTR) variant serine replacing alanine at residue 25 [Ala25Ser (Serine 25)], who showed a unique clinical picture with a relatively acute onset neuropathy within a few days of an influenza vaccination, progressing to a severe degree within 2 years. Sural nerve biopsy revealed amyloid deposition in the endoneurium. Sequencing of the proband's DNA revealed a G to T transversion at the first position of codon 25 of TTR gene. DNA analysis of this family showed the same mutation in her older sister and a niece, but her parents did not have the mutation. Haplotype analysis revealed the mutation to be clearly linked to haplotype III allele inherited from the proband's father. These results indicate this novel Serine 25 mutation originated in the paternal germline mosaicism. It is possible that the vaccination had an influence on the unique clinical picture, but this remains uncertain. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 244–250, 2002 DOI 10.1002/mus.10032
Spasticity causes a fundamental rearrangement of muscle–joint interaction
Authors: Lieber Richard L. , Fridén Jan
Published: October 2004

Sarcomere length was measured in flexor carpi ulnaris (FCU) muscles from patients with severely spastic wrist flexion contractures (n = 6), as well as from patients with radial nerve injury and a normally innervated FCU (n = 12). Spastic FCU muscles had extremely long sarcomere lengths with the wrist fully flexed (3.48 ± 0.44 μm) compared to the FCU muscles of patients with radial nerve injury (2.41 ± 0.31 μm). In three of the patients with spastic wrist flexion contractures, the slope of the FCU sarcomere length–joint angle relationship was measured and found to be, essentially, normal (0.017 ± 0.005 μm/°, n = 3) suggesting that serial sarcomere number (and therefore muscle fiber length) was unchanged in spite of the dramatic absolute sarcomere length change. These results indicate that spasticity results in a major alteration of normal muscle–joint anatomical relationships that has not previously been recognized to our knowledge. We hypothesize that the results are explained either by the inability of muscle fibers to add serial sarcomeres in response to growth, or the selective loss of FCU muscle length secondary to the central nervous system lesion. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 265–270, 2002 DOI 10.1002/mus.10036
Titin and nebulin content in human skeletal muscle following eccentric resistance exercise
Authors: Trappe Todd A. , Carrithers John A. , White Faber , Lambert Charles P. , Evans William J. , Dennis Richard A.
Published: October 2004

We measured titin and nebulin content in muscle biopsies from the vastus lateralis before and 24 h after one bout of high‐intensity eccentric knee extensor resistance exercise in seven men (26 ± 3 years). Titin and nebulin content were significantly (P < 0.05) reduced after exercise by 30 and 15%, respectively. These results suggest that the structural components of the myofibrillar apparatus are degraded following high‐intensity eccentric resistance exercise in humans. Loss of these proteins may have important implications for the mechanisms regulating the adaptive response of skeletal muscle to resistance exercise. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 289–292, 2002 DOI 10.1002/mus. 10037
Magnetic stimulation of the central and peripheral nervous systems
Authors: Weber Markus , Eisen Andrew A. ,   AAEM
Published: October 2004

Since 1985, when the technique of transcranial magnetic stimulation (TMS) was first developed, a wide range of applications in healthy and diseased subjects has been described. Comprehension of the physiological basis of motor control and cortical function has been improved. Modifications of the basic technique of measuring central motor conduction time (CMCT) have included measurement of the cortical silent period, paired stimulation in a conditioning test paradigm, repetitive transcranial magnetic stimulation (rTMS), and peristimulus time histograms (PSTH). These methods allow dissection of central motor excitatory versus inhibitory interplay on the cortical motor neuron and its presynaptic connections at the spinal cord, and have proven to be powerful investigational techniques. TMS can be used to assess upper and lower motor neuron dysfunction, monitor the effects of many pharmacological agents, predict stroke outcome, document the plasticity of the motor system, and assess its maturation and the effects of aging, as well as perform intraoperative monitoring. The recent use of rTMS in the treatment of depression and movement disorders is novel, and opens the way for other potential therapeutic applications. © 2002 American Association of Electrodiagnostic Medicine. Muscle Nerve 25: 160–175, 2002 DOI 10.1002/mus.10038
Unilateral cranial and phrenic nerve involvement in axonal Guillain–Barré syndrome
Authors: Sakakibara Yumi , Mori Masahiro , Kuwabara Satoshi , Katayama Kaoru , Hattori Takamichi , Koga Michiaki , Yuki Nobuhiro
Published: October 2004

A 49‐year‐old woman developed acute left facial, hypoglossal, and phrenic nerve palsies, as well as dysphagia and weakness in the neck and arms. Electrophysiologic studies showed an acute motor axonal neuropathy. Serum anti‐GM1 IgG antibody was positive. Intavenous immunoglobulin treatment resulted in good clinical recovery. The present report indicates that the cranial and phrenic nerves may be affected unilaterally in Guillain–Barré syndrome, and that there is clinical variability in the axonal subtype of this syndrome. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 297–299, 2002 DOI 10.1002/mus.10041
Congenital inflammatory myopathy: A demonstrative case and proposed diagnostic classification
Authors: McNeil Stephen M. , Woulfe John , Ross Catherine , Tarnopolsky Mark A.
Published: October 2004

There have been few reports of congenital inflammatory myopathy in the literature, and most of these have been associated with congenital muscular dystrophy. We review the literature and present a case with electromyographic and muscle biopsy evidence of congenital inflammatory myopathy with onset in the perinatal period and no evidence of a congenital muscular dystrophy. There was evidence of subjective improvement following corticosteroid administration (∼ mg/kg per day) with a concomitant normalization of the serum creatine kinase activity. Of particular interest in the case was the history of maternal infection, suggesting a possible postinfectious molecular mimicry as the etiology of the muscle inflammation. This case suggests that a rare form of congenital inflammatory myopathy does exist that is not associated with a congenital muscular dystrophy. A preliminary classification scheme is proposed to separate true congenital cases from those acquired after birth and those cases associated with congenital dystrophy. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 259–264, 2002 DOI 10.1002/mus.10043
Psychometric evaluation of a new handicap scale in immune‐mediated polyneuropathies
Authors: Merkies Ingemar S.J. , Schmitz Paul I.M. , Van Der Meché Frans G.A. , Samijn Johnny P.A. , Van Doorn Pieter A.
Published: October 2004

A new handicap measure, the Rotterdam nine‐item handicap scale, was developed and its validity, reliability, and responsiveness evaluated in patients with immune‐mediated polyneuropathies. We evaluated 113 stable patients, of whom 83 had Guillain–Barré syndrome (GBS), 22 had chronic inflammatory demyelinating polyneuropathy (CIDP), and 8 had a gammopathy‐related polyneuropathy. We also studied 20 patients with recently diagnosed GBS (n = 7) or CIDP (n = 13) and changing clinical conditions (longitudinal group). Significant discriminatory validity and correlation with the Rankin scale were demonstrated for the Rotterdam nine‐item handicap scale (stable group: Spearman's test, r = −.76 to −.78; longitudinal group: intraclass correlation coefficient, r = .83; P < .0001). Also, good reliability (r = .89–.98; P < .0001) and high responsiveness values (standardized response mean values > .8) were obtained for the Rotterdam nine‐item handicap scale. In contrast to the Rankin scale, the Rotterdam scale not only provided information regarding mobility but also highlighted physical independence, occupation, and social integration. These results illustrate the clinical usefulness of the Rotterdam nine‐item handicap scale under these conditions. © 2002 Wiley Periodicals, Inc. Muscle Nerve 25: 000–000, 2002