bluuebunny/crossref_metadata_2025 huggingface dataset:
The natural history of a “benign” rib lesion in a patient with a demyelinating polyneuropathy and an unusual variant of poems syndrome
Authors: Simmons Zachary , Wald John , Albers James W. , Feldman Eva L.
Published: January 2005

Patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M‐protein, and skin changes) syndrome exhibit great clinical variability and often have osteosclerotic myeloma. We present a patient with an acquired demyelinating polyneuropathy who was eventually diagnosed as having POEMS syndrome. Her long period of observation permitted documentation of the natural history of a plasmacytoma, including its remarkably slow rate of growth and its transformation from a nonsclerotic to a sclerotic bone lesion. Her clinical and laboratory features emphasize the variability of this syndrome. Biopsy of benignappearing bone lesions should be considered in patients with acquired demyelinating polyneuropathies who do not respond to standard treatment modalities. Serial x‐rays may not distinguish benign lesions from plasmacytomas in this treatable condition. © 1994 John Wiley & Sons, Inc.
AAEM case report #28: Monomelic amyotrophy
Authors: Donofrio Peter D.
Published: January 2005

Monomelic amyotrophy is a rare form of motor neuron disease usually presenting as painless asymmetric weakness and atrophy in the distal upper extremities of young adults. Only rarely are the legs involved and pyramidal findings are uncommon. Monomelic amyotrophy is most often observed in people of Japanese and Indian heritage and affects men almost exclusively. Most cases are sporadic. Laboratory testing is frequently normal or nonspecific except for electrophysiologic studies which typically demonstrate reduced compound muscle action potential amplitudes, fasciculations, and features consistent with acute and chronic denervation in distal upper extremity muscles. Necropsy in 1 patient identified anterior horn cell shrinkage, necrosis, and gliosis in appropriate spinal cord segments. Symptoms and signs often progress for several years before spontaneously arresting. The differential diagnosis for monomelic amyotrophy is broad, including processes which affect the cervical cord, roots, brachial plexus, and individual or multiple nerves in the upper extremity. © 1994 Peter D. Donofrio, M.D. Published by John Wiley & Sons, Inc.
Effects of exercise on muscle activation and metabolism in multiple sclerosis
Authors: Kent‐Braun Jane A. , Sharma Khema R. , Weiner Michael W. , Miller Robert G.
Published: January 2005

We investigated the role of metabolism in muscle fatigue during voluntary exercise in persons with mild multiple sclerosis (MS). Six MS and 8 healthy control subjects performed intermittent, progressive, isometric contractions of the ankle dorsiflexors, during which we measured maximum voluntary force (MVC), inorganic phosphate (Pi), phosphocreatine (PCr), and pH. During exercise. MVC fell sooner in MS, but by the end of exercise the relative decrease in MVC was similar in both groups. In contrast, at the end of exercise Pi/PCr increased to 1.86 ± 0.22 in controls but to only 0.66 ± 0.04 in MS (P < 0.01); likewise, pH was 6.75±0.04 in controls and unchanged (7.06 ± 0.04) in MS (P <0.01). The smaller metabolic change at the same relative exercise intensity suggests a failure of muscle activation that is present even in mild MS. Neurophsyiologic measures of activation indicated some central activation failure and no neuromuscular junction impairment in MS, and suggested that activation failure beyond the muscle membrane(excitation–contraction coupling) may be important in MS. We conclude that metabolic factors do not play a significant role in the development of muscle fatigue during voluntary exercise in mild MS. © 1994 John Wiley & Sons, Inc.
Magnetically induced muscle contraction is caused by motor nerve stimulation and not by direct muscle activation
Authors: Machetanz Jochen , Bischoff Christian , Pichlmeier Reiner , Riescher Hermann , Meyer Bernd‐Ullrich , Sader Astrio , Conrad Bastian
Published: January 2005

When magnetically stimulating peripheral nerves, a local cocontraction of muscle under the coil is observed. We assessed whether this contraction results from: (1) magnetic stimulation of motor nerves, or (2) direct depolarization of the muscle membrane. Wrist extensor muscles of normal subjects were magnetically stimulated with the coil placed directly above the muscle. Neuromuscular transmission was then blocked by atracurium using a technique of local curarization. As a reference, the radial nerve was stimulated electrically. Magnetic and electrical stimuli were applied alternatingly every 10 s. Twitch force of wrist extension was measured isometrically over a period of about 70 min including the phase of complete neuromuscular block. Twitch amplitudes elicited by magnetic and electrical stimuli were equivalent during the whole experiment. These results suggest that muscle cocontraction following magnetic stimulation results from depolarization of terminal motor nerve branches. © 1994 John Wiley & Sons, Inc.
Recording characteristics of the surface EMG electrodes
Authors: Barkhaus Paul E. , Nandedkar Sanjeev D.
Published: January 2005

Routine motor nerve conduction studies are conducted using surface EMG electrodes. Most techniques of estimating the number of motor units (MUs) are based on surface EMG recordings. Therefore, it is important to assess the uptake area of these electrodes. We recorded surface EMG motor unit action potentials (SMUAPs) from the biceps muscle of normal subjects. The SMUAP amplitude fell from 42 μV for the superficially located MUs (i.e., within 10 mm of skin surface) to 11 μV for the deep MUs (i.e., more than 20 mm from the skin surface). We infer that the pickup radius of the surface electrode is less than 20 mm. The implications of the limited uptake area of the surface electrodes to the analysis of compound muscle action potentials, estimation of the number of MUs, and the surface EMG recordings are discussed. © 1994 John Wiley & Sons, Inc.
Passive transfer of seronegative myasthenia gravis to mice
Authors: Burges Judith , Vincent Angela , Molenaar Peter C. , Newsom‐Davis John , Peers Chris , Wray Dennis
Published: January 2005

Muscle weakness in myasthenia gravis is due to autoantibody‐induced loss of functional acetylcholine receptors (AChR). About 15% of myasthenia gravis patients, however, do not have detectable anti‐AChR antibodies. To investigate the effect of their plasma immunoglobulins on neuromuscular transmission, mice were injected with plasma (and in some cases purified immunoglobulin G (IgG)) from 7 “seronegative” myasthenia gravis (SMG) patients, and neuromuscular transmission parameters were examined. When injected for 15 days, all patients' plasma caused reductions in miniature endplate potential amplitudes, while endplate potential quantal content was significantly reduced by plasma from 4 of the 7 patients. There were no changes in ACh‐induced depolarization or single channel properties, and 125l‐α‐bungarotoxin binding studies showed no effect on AChR number, except in 1 case. Purified IgG injected for 3 days had similar effects to plasma injected for 15 days. Our findings confirm that SMG is autoantibody mediated and that there are pathogenic IgG antibodies. SMG appears to be a heterogeneous disorder and the target(s) for the antibodies may be diverse. © 1994 John Wiley & Sons, Inc.
Very efficient myoblast allotransplantation in mice under FK506 immunosuppression
Authors: Kinoshita Ikuo , Vilquin Jean‐Thomas , GuéRette BenoǐT , Asselin Isabelle , Roy Raynald , Tremblay Jacques P.
Published: January 2005

Transgenic CD1 mice expressing β‐galactosidase were used as myoblast donors. The myoblasts were injected in normal or mdx muscles previously irradiated and injected with notexin. Twenty‐eight days after myoblast transplantation, the percentage of muscle fibers β‐galactosidase‐positive was low in mice not immunosuppressed but was high (80%) in those treated with FK506. In mdx mice, muscle fibers expressing β‐galactosidase were also dystrophin positive. Most of the mice not treated with FK506 produced antibodies against the donor myoblasts. These results indicate that FK506 is a very useful immunosuppressive drug for myoblast transplantation in mice. Irradiation and notexin injection used in our experiments are, however, not feasible in humans. Other manipulations capable of increasing the participation of donor myoblasts to regeneration will therefore have to be identified before new clinical trials are attempted. © 1994 John Wiley & Sons, Inc.
The medial calcaneal nerve: Anatomy and nerve conduction technique
Authors: Park Tracy A. , Del Toro David R.
Published: January 2005

We report a new technique for studying conduction in the medial calcaneal nerve (MCN). Dissection of 14 cadaver feet revealed the optimal G1 site to be one third of the way from the apex of the heel to a point midway between the navicular tuberosity and the prominence of the medical malleous. Seventy‐two feet (36 healthy volunteers) were studied using surface stimulation of the tibial nerve 10 cm proximal to the G1 surface electrode. Averaging technique was not required. Reference values (mean ± 2 SD) were determined for MCN onset latency (2.0 ± 0.3 ms), peak latency (2.5 ± 0.3 ms), onset conduction velocity (61 ± 11 m/s), peak conduction velocity (40 ± 5 m/s), baseline‐to‐peak amplitude (18 ± 6 μV), and maximum intrasubject side‐to‐side differences in these values (0.3 ms, 0.3 ms, 15 m/s, 5 m/s, and 17 μV, respectively). This study provides an easily performed, reproducible method for electrophysiologic evaluation of the MCN.© 1995 John Wiley & Sons, Inc.
Extensor digitorum brevis reflex in normals and patients with radiculopathies
Authors: Dillingham Timothy R. , Marin Raul , Belandres Praxedes V. , Chang Audrey
Published: January 2005

This prospective study evaluated the extensor digitorum brevis deep tendon reflex (EDBR) in a normal population and in patients with L‐5 and S‐1 radiculopathies. There were 88 subjects: 53 normals, 17 L‐5, and 18 S‐1 radiculopathy subjects. The clinical EDBR revealed a 91% specificity, with 18% sensitivity for L‐5, and 11% for S‐1. The electrodiagnostic EDBR yielded increased sensitivities of 35% for L‐5 (P = 0.07) and 39% for S‐1 (P = 0.04), with 87% specificity. H‐reflexes showed 50% sensitivity for the S‐1 group (P = 0.0006) and 91% specificity. EDBR latencies were significantly related to age and leg length (r2 = 0.46, P < 0.0001). Age alone explained 26% (P < 0.0001) of the EDBR variability and leg length 20% (P < 0.0001). EDBR clinical and electrodiagnostic reflexes have low sensitivities, high specificities, and do not discriminate L‐5 from S‐1 root involvement.© 1995 John Wiley &Sons, Inc.
Immunopathogenesis and treatment of the guillain‐barré syndrome—part I
Authors: Hartung Hans‐Peter , Toyka Klaus V. , Pollard John D. , Harvey Graham K.
Published: January 2005

The etiology of the Guillain‐Barré syndrome (GBS) still remains elusive. Recent years have witnessed important advances in the delineation of the mechanisms that may operate to produce nerve damage. Evidence gathered from cell biology, immunology, and immunopathology studies in patients with GBS and animals with experimental autoimmune neuritis (EAN) indicate that GBS results from aberrant immune responses against components of peripheral nerve. Autoreactive T lymphocytes specific for the myelin antigens PO and P2 and circulating antibodies to these antigens and various glycoproteins and glycolipids have been indentified but their pathogenic role remains unclear. The multiplicity of these factors and the involvement of several antigen nonspecific proinflammatory mechanisms suggest that a complex interaction of immune pathways results in nerve damage. Data on disturbed humoral immunity with particular emphasis on glycolipid antibodies and on activation of autoreactive T lymphocytes and macrophages will be reviewed. Possible mechanisms underlying initiation of peripheral nerve‐directed immune responses will be discussed with particular emphasis on the recently highlighted association with Campylobacter jejuni infection.© 1995 John Wiley &Sons, Inc.
Interaction of glucocorticoids and activity patterns affect muscle function
Authors: Dodd Stephen L. , Powers Scott K. , Vrabas Ioannis S. , Eason Jane M.
Published: January 2005

The purpose of this study was to determine the effects of glucocorticoids on muscle mass and contractile properties of muscles of similar fiber composition but differing in activity patterns. Rats were divided into two groups and administered prednisolone (5 mg/kg per day) (P;N = 9) or served as controls (C; N = 10) for 10 days. Contractile properties were then determined in the left gastrocnemius‐plantaris‐soleus (GPS) muscle complex and a strip of costal diaphragm (D). An index of fatigue was also determined in both muscle preparations. Whole‐body, GPS, and D weights decreased (P < 0.05) in the P animals (22%, 17%, and 15%, respectively) when compared to C. Specific tension (Po) increased (P < 0.05) in the GPS complex (21%) and decreased in the D (26%). Maximal shortening velocity (Vmax) was not different (P > 0.05) between groups in either the GPS or the D. While the index of fatigue was not different (P > 0.05) between groups in the D, there was a 30% increase (P < 0.05) in the rate of fatigue in the GPS. These data indicate that, although glucocorticoids cause decreased muscle mass in both D and GPS, a change in muscle architecture may prevent a decrease in force‐generating ability in some limb muscles. However, a glucocorticoids do not increase D fatigability as seen in the GPS.© 1995 John Wiley &Sons, Inc.
Charcot–marie–tooth neuropathies: From clinical description to molecular genetics
Authors: Ionasescu Victor V.
Published: January 2005

Ninety‐five families with Charcot‐Marie‐Tooth (CMT) neuropathies were studied clinically, electrophysiologically (MNCVs and EMGs), and by molecular genetics. Fifty‐four families (56.8%) were type 1A mapped at 17p11.2‐p12 and DNA duplication was present in 50 (92.6% of CMT1A families). One family with type 1B (1.1%) mapped at 1q22‐q23 showed a point mutation of the myelin Po gene. Eighteen families (18.9%) were type CMT2 based on electrophysiological studies. Molecular genetics was not yet conclusive. Twenty CMT families were with X‐linked dominant inheritance (CMTX1) (21.1%) mapped at Xq13.1 and connexin 32 (CX32) point mutations were present in 15 families (75%) (five nonsense mutations, eight missense mutations, two deletions). Two CMT families (2.1%) with X‐linked recessive inheritance showed no point mutations of CX32 and their mapping was different from CMTX1, respectively at Xp22.2 for CMTX2 and at Xq26 for CMTX3.© 1995 John Wiley &Sons, Inc.
Phrenic nerve conduction study in normal subjects
Authors: Chen Robert , Collins Stephen , Remtulla Hussein , Parkes Anthony , Bolton Charles F.
Published: January 2005

Phrenic nerve conduction studies were performed in 50 phrenic nerves from 25 normal subjects using a technique modified from previously described methods. The normal ranges for latency, amplitude, negative peak area, and duration were established. The latency correlates with age and the amplitude increases with chest circumference. With our method, the amplitude increases and the duration decreases with lung volume. We found good right‐left agreement and reproducibility. Therefore, the unaffected side can be used as a reference in unilateral phrenic nerve lesions and previous studies can be used for comparison in serial studies. We recommend that phrenic nerve conduction studies be used routinely to diagnose and monitor patients with respiratory involvement from neuromuscular diseases.© 1995 John Wiley &Sons, Inc.
Progression of neuropathy in peripheral arterial disease
Authors: England John D. , Ferguson Michele A. , Hiatt William R. , Regensteiner Judith G.
Published: January 2005

Atherosclerotic peripheral arterial disease (PAD) can cause muscle denervation, but whether it is associated with more severe peripheral nerve disease is not clear. Equally unclear is the effect of exercise training on the neuromuscular aspects of this disease. We performed serial electrophysiologic studies (nerve conduction studies and quantitative electromyography) and muscle strength assessment on 16 patients with moderately severe PAD. Seven of the patients were assigned to a natural history (control) group and 9 to and exercise training group. Over study periods ranging from 3 to 23 months, 6 to 7 patients in the control group and 8 of 9 patients in the exercise training group showed progression of multifocal neuropathic disease in their ischemic legs. For equivalent lengths of time, the degree of neuropathic progression was not significantly different between the control and exercise training group. There was a significant decline in muscle strength for the control group but not for the exercise training group. These results demonstrate that PAD can be associated with the development of a multifocal predominantly motor neuropathy, which is most likely ischemic in etiology. Furthermore, exercise training does not pose an additional risk for the development of neuropathy in patients with moderately severe PAD. © 1995 John Wiley & Sons, Inc.
Failure of anti‐GM1 IgG OR IgM to induce conduction block following intraneural transfer
Authors: Harvey Graham K. , Toyka Klaus V. , Zielasek JÜRgen , Kiefer Reinhart , Simonis Claudia , Hartung Hans‐Peter
Published: January 2005

In order to confirm the reported pathogenicity of human antibodies to monosialoganglioside GM1, immunoglobulin fractions with high anti‐GM1 IgG or IgM titers were prepared from patients with Guillain‐Barré syndrome and multifocal motor neuropathy respectively. These fractions were injected intraneurally into rat tibial nerves with fresh human complement. Neither the anti‐GM1 IgG nor the anti‐GM1 IgM fraction induced significant focal conduction block or slowing compared to a pooled fraction prepared from 5 normal individuals. In contrast, rabbit experimental allergic neuritis serum included as a positive control was highly active. Transverse sections of injected nerve failed to show evidence of demyelination. Staining for human immunoglobulin in cryostat sections showed the presence of injected anti‐GM1 antibody bound to nodes of Ranvier up to 6 days following intraneural transfer. These data fail to confirm previous reports of conduction block from intraneural transfer of anti‐GM1 serum and suggest that such electrophysiological effects may be the result of factors other than or in addition to anti‐GM1 antibodies. © 1995 John Wiley & Sons, Inc.
Quantitative measurement of cutaneous perception in diabetic neuropathy
Authors: Vinik Aaron I. , Suwanwalaikorn Sompongse , Stansberry Kevin B. , Holland Marie T. , McNitt Patricia M. , Colen Lawrence E.
Published: January 2005

To determine the diagnostic value of various cutaneous sensory modalities in diabetic neuropathy, we studied cutaneous perception at the dominant hallux of 113 subjects (32 normal healthy controls and 81 diabetic subjects). The cutaneous sensory perception tests included warm and cold thermal perception, vibration, touch‐pressure sensation, and current perception testing (CPT). The sensitivity of each modality when specificity is held greater than 90% was as follows: warm = 78%, cold = 77%, vibration = 88%, tactile‐pressure = 77%, 5‐Hz CPT = 52%, 250‐Hz CPT = 48%, and 2000‐Hz CPT = 56%. Combination thermal and vibratory gave optimum sensitivity (92–95%) and specificity (77–86%). We conclude that vibratory and thermal testing should be the primary screening tests for diabetic peripheral neuropathy. Other modalities may be of use only in specific situations. © 1995 John Wiley & Sons, Inc.
Homonymous and heteronymous monosynaptic reflexes in biceps brachii
Authors: Miller Thomas A. , Mogyoros Ilona , Burke David
Published: January 2005

Using poststimulus time histograms, it has been reported that stimulation of the median nerve at the elbow produces a monosynaptic EPSP in voluntarily active single motoneurons of the human biceps brachii. The present study was undertaken to: (i) determine whether such stimulation could evoke a reproducible reflex response in biceps brachii; and (ii) establish the optimal conditions for eliciting the reflex under clinical conditions. Twelve normal subjects were studied. No reflex response was recordable when biceps brachii was relaxed. A reflex response with a mean latency of 14.0 ms (±0.96 ms) could be recorded during a background voluntary contraction. The response was small (0.5–4.5% of the maximal M wave) but symmetrical, and could be obtained in all subjects. The responsible afferents appear to be rapidly conducting fibers from forearm flexor muscles and the latencies of the response were consistent with a monosynaptic reflex. Reflex amplitude increased with stimulus intensity and contraction strength. Stimulus rate did not affect amplitude significantly. It is concluded that a reproducible heteronymous monosynaptic reflex can be recorded from the contracting biceps brachii on stimulation of the median nerve at the elbow. Although smaller than the homonymous H reflex evoked by stimulation at Erb's point, it was technically easier to demonstrate that the EMG potential was of reflex origin (rather than part of an M wave). These reflexes should be of value in the assessment of the C‐5/C‐6 segments and the upper trunk of the brachial plexus. © 1995 John Wiley & Sons, Inc.
Amyotrophic lateral sclerosis is a multifactorial disease
Authors: Eisen Andrew
Published: January 2005

Amyotrophic lateral sclerosis (ALS) is probably biphasic. An initial trigger(s) is followed by a terminal cascade coinciding with the onset of neurological deficits. The terminal cascade involves interactive multifactorial pathogenic mechanisms. Aging must play a crucial role leading to multiple defective or degraded gene products accumulating with progressing years. This in turn leads to failure of receptor integrity and resulting excitotoxicity, free radical accumulation, failure of neurotrophism, and possibly immunological disturbances. These events are predated by months or years by a trigger which is also likely to be multifactorial and cumulative. Evidence suggests that environmental factors may be important triggers. Failure of specific glutamate transporters and calcium binding proteins may account for selective vulnerability of the corticomotoneuronal system. It is postulated that in ALS the primary target cell is the corticomotoneuron or the local circuit interneurons which modulate its activity. Glia cells may play an important role in the demise of the corticomotoneuronal cell. The disordered corticomotoneuron induces excessive excitatory transmitter (glutamate?) release at the corticomotoneuronal–spinal‐motoneuronal synapse resulting in the subsequent demise of this neuron. © 1995 John Wiley & Sons, Inc.
Mitochondrial encephalomyopathy with autosomal dominant inheritance: A clinical and genetic entity of mitochondrial diseases
Authors: Kawai Hisaomi , Akaike Masashi , Yokoi Kenji , Nishida Yoshihiko , Kunishige Makoto , Mine Hideki , Saito Shiro
Published: January 2005

We report a Japanese family with chronic progressive external ophthal‐moplegia (CPEO) with autosomal dominant inheritance, and review 54 reported CPEO patients in seven families (including the present family) with autosomal dominant inheritance and mtDNA deletions in the skeletal muscle. Mean age at onset in the CPEO was 26 years, which is older than that in published solitary cases. In addition to blepharoptosis and external ophthalmoplegia, proximal muscle atrophy and weakness were found in 62%, hearing loss in 25%, and ataxia in 17% of the patients. Retinal degeneration was not found, and cardiac involvement was very rare. mtDNA deletions in the muscle were multiple and large scale, and all such deletions were located in the non–D‐loop region. Autosomal dominant CPEO has unique clinical features which differ from those of solitary CPEO, and is associated with multiple large‐scale mtDNA deletions. Thus, autosomal dominant CPEO can be considered a clinical and genetic entity of mitochondrial diseases. © 1995 John Wiley & Sons, Inc.
Detection of HTLV‐I provirus by in situ polymerase chain reaction in mononuclear inflammatory cells in skeletal muscle of viral carriers with polymyositis
Authors: Higuchi I. , Hashimoto K. , Kashio N. , Izumo S. , Inose M. , Izumi K. , Ohkubo R. , Nakagawa M. , Arimura K. , Osame M.
Published: January 2005

We tested for HTLV‐I proviral DNA in skeletal muscle from patients with polymyositis infected with HTLV‐I using the in situ polymerase chain reaction. We found the HTLV‐I provirus in some of the CD4‐positive cells in HTLV‐I‐positive polymyositis cases but not in HTLV‐I‐negative polymyositis ones. We could not detect HTLV‐I within the muscle fibers. We suggest that HTLV‐I‐associated polymyositis is not due to direct, persistent infection of the muscle fiber by the virus, but to a T‐cell‐mediated immunological process triggered by the HTLV‐I‐infected cells. © 1995 John Wiley & Sons, Inc.
Amyloidosis causing a progressive myopathy
Authors: Nadkarni Nitin , Freimer Miriam , Mendell Jerry R.
Published: January 2005

A 62‐year‐old woman developed profound weakness secondary to a progressive myopathy associated with primary systemic amyloidosis. The characteristic apple‐green birefringent amyloid deposits were demonstrated surrounding individual muscle fibers in Congo red stained sections. Electron microscopy demonstrated amyloid filaments in close apposition to muscle fibers exhibiting excessive corrugations of the sarcolemmal membrane. The pathological features of progressive amyloid myopathy associated with primary systemic amyloidosis are distinct from the intracellular amyloid deposits characteristic of sporadic inclusion body myositis and inherited inclusion body myopathy. © 1995 John Wiley & Sons, Inc.
Age‐related differences in regeneration of dystrophic (mdx) and normal muscle in the mouse
Authors: Pastoret Christian , Sebille Alain
Published: January 2005

The mdx mouse, a genetic homologue of human Duchenne muscular dystrophy (DMD), has been attributed with a greater regenerative capacity of its skeletal muscles. Here, we have tested the hypothesis that muscles of mdx mice regenerate better than those of nondystrophic animals. We studied muscle regeneration resulting from a denervation‐devascularization injury (DD) of extensor digitorum longus muscle (EDL) at 3 weeks and 2 months in mdx and wild‐type (C57BL/10) mice. Histological and morphometrical studies of muscle regeneration were made from 3 to 180 days later. When DD was performed in 3‐week‐old C57BL/10 mice, the percentages of nonperipheral nuclei in regenerated fibers decreased progressively over 3 months. This decrease did not occur in animals where DDs were performed at 2months, suggesting that two different populations of muscle precursor cells are mobilized in muscle regenration in mice at these two ages. Moreover, mdx EDL muscle regenerated similarly to the controls for up to 60 postoperative days, as shown by distribution of mean diameters and percentage of nonperipheral nuclei of muscle fibers. After 60 postoperative days, necrosis/regeneration characteristics of mdx muscles recurred, suggesting that mdx‐regenerated muscle fibers remain susceptible to degeneration.© 1995 John Wiley & Sons, Inc.
X‐Linked recessive bulbospinal neuronopathy: Clinical phenotypes and cag repeat size in androgen receptor gene
Authors: Shimada Noriko , Sobue Gen , Doyu Manabu , Yamamoto Koji , Yasuda Takeshi , Mukai Eiichiro , Kachi Teruhiko , Mitsuma Terunori
Published: January 2005

Clinical phenotypes and the CAG repeat size of the androgen receptor gene were assessed in 95 Japanese patients with X‐linked recessive bulbospinal neuronopathy. There was an age‐ and duration‐dependent deterioration of muscle strength, ADL scores, and plasma creatine kinase levels. However, there was no correlation between the presence or absence of gynecomastia or diabetes mellitus and the age at onset or duration of illness. Correlations were present between the CAG repeat size and the age at onset (P < 0.0001) as well as the presence or absence of gynecomastea (P < 0.05). Muscular weakness and ADL scores were also correlated with the CAG repeat size only when they were adjusted by the age at examination not by the duration of illness. These findings suggest that CAG repeat size is one of the determinant factors of disease progression. However, extensive variation in phenotypic severity in patients with the same size of CAG repeat was present even among the siblings, suggesting that other factors than CAG repeat size influence the phenotypic manifestation. The average gain of CAG repeat size expansion was a 1.4 repeat in paternal transmission which was more unstable than that in maternal transmission, but the magnitude of the expansion in paternal transmission was much smaller than is presently known for other diseases in which CAG repeat expansion is the responsible gene defect. © 1995 John Wiley & Sons, Inc.
Facilitation of motor evoked potentials: Timing of jendrassik maneuver effects
Authors: Péréon Yann , Genet Robert , Guihéneuc Pierre
Published: January 2005

Remote voluntary contraction, such as the classical Jendrassik maneuver (JM), is a procedure routinely used to increase the amplitude of tendon reflexes in the lower limb. In 8 healthy subjects we studied the effects of JM on the motor evoked potentials (MEP) recorded from tibialis anterior muscle, produced by transcranial magnetic stimulation (stimulus output of 5–10% over motor threshold). In this study, JM consisted here of a bilateral violent handgrip, preceding magnetic stimulation from 100 to 50 ms (steps of 100 ms). Compared to the control test, latencies remained unchanged. MEP amplitudes were greatly enhanced with a JM test interval from 200 to 400 ms (170% of control amplitude at 300 ms). We also studied 6 patients with severe alterations of MEPs from tibialis anterior muscle. In each case, JM preceding magnetic stimulation (stimulus output 100%) from 300 ms induced reappearance of response or marked enhancement of amplitude, allowing calculation of central conduction time. Such a technique, which is easy to perform, may be useful in clinical practice to calculate central motor conduction time, where it would otherwise be difficult or impossible. © 1995 John Wiley & Sons, Inc.
Diphtheritic neuropathy
Authors: Cléange Alain , Meyrignac Christian , Roualdes Brigitte , Degos Jean‐Denis , Gherardi Romain K.
Published: January 2005

A 29‐year‐old Haitian man had a sore throat with a marked tonsillar exudate followed by the onset of increasingly blurred vision with swallowing difficulties and, then, a diffuse acute demyelinating neuropathy. Cerebrospinal fluid showed moderate pleiocytosis and elevated protein content. Serological testing for diphtheria, but not for tetanus and poliomyelitis, was markedly positive. Other tests including HIV1 and 2, HTLV1 and Lyme disease were negative. Diphtheritic neuropathy should be considered in the differential diagnosis of Guillain–Barré syndrome in nonvaccinated patients. © 1995 John Wiley & Sons, Inc.
Changes in protein, electrolyte, and water metabolism caused by prolonged spreading cortical depression in rats
Authors: Křivánek Jiří
Published: January 2005

Repeated application of 25% KCl on the cerebral cortex for 5 hours led to a decrease of protein content (referred to both dry weight and DNA content). Incorporation of 14C‐leucine into the cortical protein was inhibited by 33%. Six hours after KCl had been removed the normal incorporation rate was found. The potassium and water content of the KCl treated cortex increased. Similar though less marked changes were found in the subcortex, while the values of these substances in the contralateral cortex remained unchanged. The potassium level was found to recover in 24 hours. Much of the change in water content also occurred in 24 hours with recovery to control values in about 13 days.
Effects of micro‐iontophoretic administration of magnesium and calcium on neurones in the central nervous system of cats
Authors: Kato G. , Somjen G. G.
Published: January 2005

Ca2+ and Mg2+ released from five‐barrelled micropipettes by currents of 5 to 80 nA caused a deceleration of the firing rate of neurones discharging spontaneously, or excited by the simulataneous administration of glutamate or acetylcholine, in the cerebral cortex, cuneate nucleus and spinal cord. Excitation of cells in the cuneate nucleus, caused by stimulation of sense organs, was also suppressed by Ca2+ and Mg2+. Synaptic transmission of volleys, evoked by electrical stimulation, from thalamus to cortical cells, from superficial radial nerve to cells in the cuneate nucleus, and from ventral root via recurrent collaterals to Renshaw cells, was partially or completely blocked by Ca2+ and Mg2+ (60 to 200 nA). When given together, the depressant effect of the two ions summed. There was no evidence of enhanced synpatic transmission under the influence of Ca2+. It is concluded that in the central nervous system a small elevation of the concentration of Ca2+ or of Mg2+ has a powerful postsynaptic depressant effect.
Effects of frontal brain electroshock stimulation on eeg activity and memory in rats: Relationship to ECS‐produced retrograde amnesia
Authors: Zornetzer Steven , McGaugh James L.
Published: January 2005

Electroencephalographic changes occurring in rat brain following frontal cortex stimulation in non‐lesioned rats and in rats with bilateral lesions in the region of amygdala were investigated in Experiment I. Seizure duration and EEG spike frequency varied systematically with current level. The amnesic effects of conventional ECS and frontal cortex stimulation were studied in Experiment II. Both treatments were highly effective in producing retrograde amnesia for an inhibitory avoidance response. The findings are interpreted as suggesting that the effectiveness of ECS in producing RA varies with the amount of current reaching the brain.
Effect of guanidine on the squid giant synapse
Authors: Kusano Kiyoshi
Published: January 2005

Effects of guanidine on pre‐ and postsynaptic activities in the untreated or tetrodotoxin‐treated squid giant synapses were examined by externally perfusing with various concentrations (423 mM, 42 mM, 21 mM, and 4.2 mM), or by iontophoretic injection of guanidine into the presynaptic terminal. In 423 mM guanidine (Na‐free), the pre‐ and postsynaptic spikes together with PSP were completely abolished. In concentrations of 42 mM or lower of guanidine media the following changes related to the concentration used were observed: reduction of delayed rectification of both axon membranes without significant alteration of resting membrane resistances; a few millivolts decrease in the resting membrane potentials; small decrease in amplitude of pre‐ and post‐synaptic spikes without marked increase of spike duration; enhancement of synaptic activity as manifested by increases in the amplitude and duration of the PSP. Iontophoretically injected guanidine also reduced delayed rectification of the presynaptic membrane. Input‐output relation was modified in a way similar to externally applied guanidine and an “Off‐PSP” was demonstrated shortly after application of an inside positive presynaptic polarization. Thus, a comparison of the augmentation of synaptic transmission by the extracellular and intracellularly applied guanidine demonstrates that the primary effect is at the presynaptic terminal.
Electrophysiological correlates of the response decrement produced by mechanical stimuli in the protozoan, Stentor coeruleus
Authors: Wood David C.
Published: January 2005

The response pattern of Stentor coeruleus has been studied using repetitive mechanical stimuli which were applied to a limited body surface. The decrement in the probability of response was found to parallel that previously reported when more gross mechanical stimuli were employed. This result justified the employment of such localized stimuli in the determination of electrophysiological correlates of the response decrement.

Electrophysiological records show a decrease in the amplitude of the prepotential and an increase in the latency of the response during the course of repetitive stimulation. After considering possible complications resultant from changes in the amplitude of the diphasic potential and the relation of the stimulus probe to the animal, it was concluded that the potential changes noted do, in fact, indicate a change in receptor function is associated with the response decrement. This result was predicted on the basis of previously reported behavioral data.
Effects of halothane and colchicine on microtubules and electrical activity of rabbit vagus nerves
Authors: Hinkley Robert E. , Green Louis S.
Published: January 2005

Rabbit vagus nerves were treated in vitro with colchicine and halothane, an anesthetic, and the electrical activity monitored. The number of microtubules per square micron of axonal area was measured for each treatment directly from electron micrographs with the aid of a planimeter. Colchicine (10 mM) significantly reduced the number of microtubules per square micron of axonal area from 40 to 16 and had little effect on the electrical activity. Halothane (3 and 10 mM) significantly increased the number of microtubules from 40 to about 55 per square micron of axonal area and blocked the ability of the nerve to propagate impulses within 25 and 10 min, respectively.
Inhibitors of protein synthesis involved in memory disruption: A study of their effects on sympathetic ganglion isolated in vitro
Authors: Paggi Paola , Toschi Giovanni
Published: January 2005

The effect of puromycin, puromycin‐aminonucleoside, cycloheximide and acetoxycycloheximide has been tested on the function of the superior cervical ganglion of rat, by recording the postganglionic response evoked by preganglionic stimulation. All the compounds examined exerted a depressant effect, which was prompt and reversible with puromycin and its aminonucleoside, but slow and irreversible with the glutarimides. A correlation of these effects with inhibition of protein synthesis has been examined. The relevance of these findings for experiments on memory disruption reported in the literature is discussed.
Influx of glutamic acid in peripheral nerve. Energy, ionic, and pH dependence
Authors: Wheeler Darrell D. , Boyarsky L. L.
Published: January 2005

The energy, ionic, and pH dependence of the influx of labelled L‐glutamic acid has been studied in frog sciatic nerve

The addition of 10−2 M D‐glucose to the incubation medium was found to inhibit the total influx of glutamic acid from a 10−6 M solution by about 40%.

The rate of influx was found to be sensitive to the temperature of the bathing medium. Influx was found to increase with temperature in a linear manner from 10°C to 34°C; above 34°C, the rate of influx dropped with increased temperature.

At room temperature, the effect of metabolic inhibitors on total influx ranged from 18% inhibition with aresenate to 62% with DNP. Increasing the incubation temperature to 34°C increased the percentage inhibition, a maximum inhibition of 86% occurring with DNP.

The cardiac glycoside ouabain (10−3 M) slightly inhibited influx (15%).

Reduction of the sodium concentration to 2.3 mM resulted in an 82% decrease in total influx. Omission of potassium or a tenfold increase in potassium concentration had no significant effect on influx. Omission of calcium resulted in a slight stimulation of uptake (16%); a tenfold increase in calcium concentration was slightly inhibitory (15%).

No relation was found between influx and the pH of the incubation medium. Lowering the pH to 5.4 or raising it to 9.4 had no significant effect on influx.

It is concluded that L‐glutamic acid influx in frog sciatic nerve is probably associated with an energy‐dependent process.
Development of rapid axonal flow in the chick embryo
Authors: Bondy Stephan C. , Madsen Carol Jean
Published: January 2005

The development of axonal transport in the optic nerve of the developing chick has been investigated. Following monocular injection of radioactive fucose and proline, the specific activity of protein from paired optic lobes was determined in chick embryos and new‐hatched chicks. In addition, the magnitude of the acid soluble precursor pool within lobes was assayed. The specific activity of protein in the optic lobe contralateral to (and innervated by) the injected eye relative to that of protein within the corresponding ipsilateral lobe (not innervated by the injected eye) was used as an index of axonal flow. In embryonic chicks from 8–12 days of age, four hours after injection of 3H‐fucase into a single eye, this ratio was somewhat above unity. In the 13‐day incubated embryo the ratio rose abruptly to over 10, and remained high throughout the period studied (up to one day after hatch). No similar sharp rise was found for the corresponding ratio for acid soluble radioactivity which remained under 2 in all ages of chicks examined. Parallel experiments with 14C‐proline resulted in a smaller and more gradual development of asymmetry of specific activity of protein from paired optic lobes.

These data suggest that, at the 13th day of incubation, specialized mechanisms develop for the rapid distal transport of glycoprotein synthesized within the retinal ganglion cells to terminations in the optic tectum. This phenomenon follows the major development of cerebral oxidative metabolism, but precedes the appearance of spontaneous or evoked potentials within the chick brain.
Characteristics and a model for fast axoplasmic transport in nerve
Authors: Ochs Sidney
Published: January 2005

Fast axoplasmic transport at a characteristic rate close to 400 mm/day is seen in a variety of different mammalian species. Particulates, proteins, polypeptide, and free amino acids are all carried down the fibers at the same rate. An hypothesis to account for such a heterogeneous transport is based on the sliding filament theory of muscle. In the neuron, a “transport filament” is synthesized in the cell body and to it the various transported species are bound. The transporting filament moves down the nerve by means of cross‐bridges. In analogy to muscle where ATP is required to supply the energy required for the cross‐bridges, fast axoplasmic transport in the nerve fiber also has been found to depend on oxidative metabolism supplying ATP.
Transport of proteins and sulfated mucopolysaccharides in the goldfish visual system
Authors: Elam John S. , Agranoff Bernard W.
Published: January 2005

3H‐Proline, 3H‐asparagine and Na235SO4 have been found to be highly specific markers for axonal transport in the goldfish visual system. The amino acids label both rapidly and slowly transported proteins while the 35S is recovered mainly with the rapidly transported materials, including protein‐bound mucopolysaccharides. These precursors have been used to study the rate of transport, the subcellular distribution and the rate of turnover of transported proteins in the nerve terminals.
Preparative resolution and identification of synaptic components of rat neocortex
Authors: Garey Richard , Harper Jon , Best Jay Boyd , Goodman Alan B.
Published: January 2005

A varity of density gradient centrifugation techniques have been used with varying degrees of success, for the preparative resolution of pinched off nerve endings, i.e. “synaptosomes,” and synaptic components from brain homogenates. The physics of such gradients was critically considered from the standpoint of improving the degree of resolution of particulate classes and preserving morphological integrity. A procedure, using continuous linear Ficoll gradients, is presented which yields improved particulate resolution with good preservation of ultrastructural morphology. Further purification of the membraneous fraction yielded relatively high purity preparations of PSM particulates. The PSM particulates were identified as dissociated postsynaptic membranes by three kinds of electron microscope studies. Broad field electron micrographs, which permit assessment of fraction homogeneity, are presented for all fractions.
Comparison of tertiary and quaternary amine local anesthetics in their ability to depress membrane ionic conductances
Authors: Narahashi Toshio , Frazier Donald T. , Moore John W.
Published: January 2005

Tertiary and quaternary amine local anesthetics have been studied to determine their effect on membrane ionic conductances in squid giant axons. The tertiary compounds 6211 and 6603 (10mM), both lidocaine derivatives, suppressed both peak transient and steady‐state currents from either side of the nerve membrane. The time for the transient current to reach its peak was not appreciably affected. When applied internally, two quaternary compounds, QX‐314 and hemicholinium‐3 (1mM), were effective in suppressing both components of the membrane currents. However, when applied externally, they had no effect on the membrane currents even at a concentration of 10mM. The time to peak current was slightly prolonged when QX‐314 was applied internally. Another quaternary compound QX‐572 suppressed both currents from outside, but was more effective from the inside, 1mM externally being roughly equipotent to 0.1mM internally. The tertiary and quaternary compounds used in the present study block the nerve conduction by essentially the same mechanism as that previously reported for other local anesthetics.
Superfused cerebral tissues in hypoxia: Neurotransmitter and amino acid retention; labile constituents and response to excitation
Authors: Pull I. , Jones D. A. , McIlwain H.
Published: January 2005

Hypoxic conditions have been imposed on incubating superfused neocortical tissues. Their lactate production increased and K and phosphocreatine content fell moderately; under these conditions the release of amino acid and putative neurotransmitters into the superfusing medium was studied. During the hypoxia, total free amino acids increased, the major increase being in the quantity found in the superfusing medium. With [3H] leucine introduced in vitro however, hypoxia caused a significant diminution of efflux from tissue to medium. Electrical stimulation under hypoxia increased the efflux of total amino acid, but not that of leucine. Retention of norepinephrine and serotonin by the tissue, and their release into the superfusing medium during electrical stimulation, were found to persist during hypoxia.
Neural mechanisms underlying behavior in the locust Schistocerca gregaria. II. Integrative activity in metathoracic neurons
Authors: Hoyle Graham , Burrows Malcolm
Published: January 2005

Preliminary studies are reported on the roles of some interneurons and motorneurons in generating simple behavior patterns in the locust Schistocerca gregaria. The neurons were penetrated by intracellular electrodes singly, or in pairs, whilst a variety of reflexes and spontaneous movements was observed. For example: large oscillations of membrane potential occurring at characteristic flight frequency were observed in flight motorneurons after blowing on the head. The pattern of impulses was directly determined by the amplitude and period of the oscillations. Similar oscillations were observed to occur spontaneously in leg motorneurons, where they caused bursts of impulses which closely resembled those occurring during walking.

Both sensory‐integrating and pre‐motor interneurons were encountered, but none with the function of “commanding” motor output. Much of the input to leg motorneurons is already in programmed form.