bluuebunny/crossref_metadata_2025_split huggingface dataset:
Nerve conduction studies in amyotrophic lateral sclerosis
Authors: Cornblath David R. , Kuncl Ralph W. , Mellits E. David , Quaskey Shirley A. , Clawson Lora , Pestronk Alan , Drachman Daniel B. 
Published: January 2005

AbstractNerve conduction studies (NCS) are an integral part of the evaluation of amyotrophic lateral sclerosis (ALS) patients and are useful in distinguishing ALS from disorders that mimic it. The question often arises whether in the presence of severe atrophy and reduction of the compound muscle action potential amplitude, abnormal conduction velocity (CV), distal latency (DL), of F‐wave latency (F) exceeds what can be expected from ALS alone. To determine the limits of abnormality in classic ALS, we prospectively evaluated NCS data from 61 patients who met a strict clinical definition of ALS. We ralated CV, DL, and F to distal evoked amplitude (AMP) in peroneal (n = 63 observations), median (n = 50), and ulnar (n = 52) nerves. In nerves with reduced AMP, CV rarely fell to less than 80% of the lower limit of normal, and DL and F rarely exceeded 1.25 times the upper limit of normal. Utilizing the entire data set and regression analyses, 95% confidence limits for expected values for CV, F, and DL as a function of AMP were calculated. These limits thus derived suggest criteria for NCS abnormalities in ALS and may by useful in differentiating ALS from other illnesses. © 1992 John Wiley & Sons, Inc.
An experimental model of mitochondrial myopathy: Germanium‐induced myopathy and coenzyme Q10 administration
Authors: Wu Chien‐Ming , Matsuoka Taro , Takemitsu Masakazu , Goto Yu‐Ichi , Nonaka Ikuya 
Published: January 2005

AbstractIn skeletal muscles from rats treated with germanium for 23 weeks, there were numerous ragged‐red fibers and cytochrome‐c oxidase (COX)‐deficient fibers. Biochemically, germanium reduced the enzyme activities in the mitochondrial respiratory chain. Rotenone‐sensitive NADH–cytochrome‐c reductase as well as COX activities were markedly reduced, while succinate–cytochrome‐c reductase was less severely, but significantly, affected. The histopathological findings in these muslces were similar to those seen in patients with mitochondrial encephalomyopathy, suggesting that germanium‐induced myopathy may be a useful experimental model. Coenzyme Q10 administration appeared to be ineffective in preventing this experimental myopathy. © 1992 John Wiley & Sons, Inc.
Lower motor neuron dysfunction in patients with multiple sclerosis
Authors: Shefner Jermy M. , Mackin Glenn A. , Dawson David M. 
Published: January 2005

AbstractA patient in whom multiple sclerosis (MS) was ultimately diagnosed presented with a lower motor neuron syndrome involving 1 hand, with EMG evidence of denervation. Twelve other patients were subsequently identified with definite MS and asymmetric hand atrophy. These patients were studied clinically and electrophysiologically. Evidence of chronic and ongoing denervation was noted in the hands of 12 of the 13 patients; in only 3 patients could the EMG abnormalities be accounted for by peripheral nerve lesions. Thus, lesions resulting in lower motor neuron damage may occur in the central nervous system in MS patients. We suggest that demyelination in the region of the ventral root exit zone may account for these findings. © 1992 John Wiley & Sons, Inc.
Acute myopathy and neuropathy in status asthmaticus: Case report and literature review
Authors: Lacomis David , Smith Thomas W. , Chad David A. 
Published: January 2005

AbstractA 38‐year‐old woman developed acute, severe weakness during the treatment of status asthmaticus that included high‐dose intravenous corticosteroids. A muscle biopsy and EMG indicated a myopathy, and nerve conduction studies disclosed a neuropathic component. In association with corticosteroid tapering, the clinical, EMG, and nerve conduction abnormalities resolved. In some patients, intensive treatment of status asthmaticus may cause a reversible, toxic disorder of muscle and nerve. © 1993 John Wiley & Sons, Inc.
Ocular myastyenia gravis: The diagnostic yield of repetitive nerve stimulation and stimulated single fiber EMG of orbicularis oculi muscle and infrared reflection oculography
Authors: Oey P. Liam , Wieneke George H. , Hoogenraad Tjaard U. , Van Huffelen Alexander C. 
Published: January 2005

AbstractFor the diagnosis of ocular myasthenia gravis (ocular MG), testing of the muscles close to the affected ones may be important. The relative importance of several methods: stimulated single fiber EMG (stimulated SFEMG), repetitive nerve stimulation test (RNS) of orbicularis oculi muscle, and infrared reflection oculography (IROG) was investigated. Thirty‐two patients in whom a diagnosis of ocular MG was considered on clinical grounds were admitted to the study. Based on the results of the three neurophysiological tests, the patients could be divided in three groups: a first group with an abnormal stimulated SFEMG, and an abnormal RNS and/or abnormal IROG; a second group with only a slightly abnormal stimulated SFEMG; and a third group with normal tests in all three tests. The clinical diagnosis of ocular MG was made in all 11 patients of the first group; in 86% (6 of 7) of the patients of the second group; and in 7% (1 of 14) of the patients of the third groups. This study demonstrates that the orbicularis oculi muscle is a suitable muscle for stimulated SFEMG in patients with ocular MG, and that the results obtained with this technique showed a better relation with the clinical diagnosis than those of the two other techniques. We also demonstrate that there is no additional value in studying the jitter with different stimulation rates in patients with suspected ocular MG. © 1993 John Wiley & Sons, Inc.
The value of magnetic stimulation in the diagnosis of radiculopathies
Authors: Bischoff Christian , Meyer Bernd‐Ulrich , Machetanz Jochen , Conrad Bastian 
Published: January 2005

AbstractWe studied 42 patients with cervical and lumbar radiculopathies using magnetic stimulation. Prolonged latencies following magnetic stimulation of the brain, the paravertebral spinal nerve, or both, showed a highly significant correlation with muscle weakness in clinical testing (24 of 26 patients with weakness had pathological motor evoked potentials) as well as with spontaneous activity of the target muscles in needle electromyography (24 of 25 subjects). The determination of both the central and the peripheral motor conduction time was found to be essential in patients with radiculopathies, because different patterns of latency changes correlated with different morphological results in computed tomography: in patients diagnosed as having a lateral compression of the nerve root the peripheral nerve latency was delayed; whereas, in patients with more medially localized herniations, a prolonged central motor latency was the most frequent finding. This study posits that combined magnetic stimulation of the brain and nerve root is an effective and painless technique for the noninvasive evaluation of nerve root function. © 1993 John Wiley & Sons, Inc.
Search for HIV proviral DNA and amplified sequences in the muscle biopsies of patients with HIV polymyositis
Authors: Leon‐Monzon Marta , Lamperth Lajos , Dalakas Marinos C. 
Published: January 2005

AbstractWe searched for the presence of human immunodeficiency virus (HIV) in fresh‐frozen muscle biopsy specimens from 10 patients with HIV‐associated polymyositis (HIV‐PM) using (a) 35S‐labeled HIV‐RNA transcript of the virus and in situ hybridization, and (b) polymerase chain reaction and slot‐blot hybridization utilizing primers amplifying sequences from the gag and pol genes of the HIV genome. With in situ hybridization, positive signals were detected in sparse lymphoid cells surrounding the muscle fibers, but not within the muscle fibers, in up to two consecutive sections in 6 of the 10 specimens. By the polymerase chain reaction, amplified HIV‐specific sequences were noted in 2 specimens, but in only 2 of 8 consecutive sections, implying infection of lymphoid cells rather than muscle fibers. Muscle cultures from six specimens failed to show integrated HIV sequences within the myotubes. We conclude that HIV sequences or transcriptional products are not present within the muscle fibers or the cultured myotubes of patients with HIV‐PM. This indicates that: (a) viral replication does not take place within the muscle; (b) integration of HIV proviral genome does not occur within the myonuclei or satellite cells; and (c) HIV‐PM does not seem to be due to a persistent infection of the muscle fiber by the virus. © 1993 John Wiley & Sons, Inc.
Adult acid maltase deficiency
Authors: Barohn Richard J. , McVey April L. , Dimauro Salvatore , Griggs Robert C. , Mendell Jerry R. , Miller Robert G. 
Published: January 2005

AbstractA 30‐year‐old man was referred for neurologic evaluation because of elevated creatine kinase. He had noted symptoms of proximal arm and distal leg weakness for several years, and, on examination, he had weakness in a scapuloperoneal distribution. An electromyogram showed myotonic discharges in the paraspinous muscles, and a muscle biopsy revealed severe vacuolar myopathy. Biochemical analysis of muscle showed acid maltase deficiency. The patient's only brother had childhood‐onset acid maltase deficiency and died of respiratory failure at age 27. Acid maltase deficiency may have heterogeneous presentations within a family, and adult AMD can present as a scapuloperoneal neuromuscular syndrome. © 1993 John Wiley & Sons, Inc.
Variability and interrelationships of surface EMG parameters during local muscle fatigue
Authors: Linssen Wim H. J. P. , Stegeman Dick F. , Joosten Ed M. G. , Notermans Servaas L. H. , van't Hof Martin A. , Binkhorst Rob A. 
Published: January 2005

AbstractThe inter‐and intraindividual variability of the frequency power density spectral and surface EMG amplitude parameters and of the muscle fiber conduction velocity (MFCV) is studied in 26 healthy volunteers during fatiguing isometric ischemic intermittent exercise of the m, biceps brachii at 80% of the maximal voluntary contraction level, with a contraction rate of 30/min. No significant age effects were found. Males were significantly stronger compared with females. The higher initial SEMG amplitude and the strongershift of the frequency power density spectrum (PDS) to lower frequencies appear to be significantly correlated with males. Fatigue induces an almost proportional compression of the SEMG frequency content. The muscle fiber conduction velocity has the highest intraindividual reproducibility (r = 0.81). Despite the definite and strong influence of the MFCV on the PDS, the shift of the PDS can not be explained by a change of MFCV alone.© 1993 John Wiley & Sons, Inc.
Persistent and transient “conduction block” in motor neuron diseases
Authors: Lange Dale J. , Trojaborg Werner , McDonald T. Drake , Blake David M. 
Published: January 2005

AbstractAlthough conduction block indicates dysfunction of peripheral nerve, itmay occur in patients with clinically typical motor neuron disease. There are no universally accepted criteria to identify conduction block, so diagnosis may be difficult. In some peripheral neuropathies, conduction block persists over long periods of time. If conduction block persists in motor neuron disease, then a more reproducible means for identification would be available. We repeatedly studied 9 patients with different forms of motor neuron diseases; conduction block was suspected because of excessive loss of the amplitude of motor evoked responses between distal and proximal stimulation sites. Five showed persistent amplitude loss at intervals between 12 and 36 months. All had focal loss of amplitude and area across a specific segment; all were men; none had definite upper motor neuron signs, 2 had probable and 3 had no upper motor neuron signs; 1 had IgM paraproteinemia, one elevated anti‐GM1 titers; the duration of symptoms spanned 4–13 years. Four patients had transient loss of amplitude that was not reproduced in intervals between 3 and 13 months. None had focal loss of both amplitude and area; 2 were men; all had definite upper motor neuron signs and none had symptoms for more than 3–13 months; and none had immunological abnormalities. Thus, patients with persistent amplitude loss fulfill other criteria for conduction block, have prolonged survival but otherwise have clinical syndromes indistinguishable from ALS, except that definite upper motor neuron signs seem to be exceptional. © 1993 John Wiley & Sons, Inc.
Mechanisms of fatigue in normal intercostal muscle and muscle from patients with myasthenia gravis
Authors: Pagala Murali , Nandakumar N. V. , Venkatachari S. A. T. , Ravindran Kadirimangalam , Amaladevi Bellamakonda , Namba Tatsuji , Grob David 
Published: January 2005

AbstractFatigue mechanisms in normal intercostal muscle and muscle from patients with myasthenia gravis (MG) were evaluated by monitoring the compound muscle action potential (CMAP) and tetanic tension responses to repetitive nerve or muscle stimulation in vitro. When fatigue was induced by nerve stimulation at 30 Hz for 0.5 s every 2.5 s, about half of the original tension decreased after 30 min in normal muscle and 5 min in MG muscle. Analysis of the changes in area of CMAPs and tension indicated that impairment of neuromuscular transmission, muscle membrane excitation, and excitation‐contraction (E‐C) coupling and contractility accounted for 40%, 29%, and 31% of fatigue in normal muscle, and 83%, 0%, and 17% of fatigue in MG muscle. When fatigue was induced by muscle stimulation at 30 Hz, tension declined by a quarter after 30 min in normal muscle, but by a half after 17 min in MG muscle. Impairment of muscle membrane excitation and E‐C coupling and contractility accounted for 58% and 42% of fatigue in normal muscle, and 22% and 78% of fatigue in MG muscle. Thus, fatigue of normal muscle is caused by impairment of at least four processes, and enhanced fatigue of MG muscle is caused by greater impairment of neuromuscular transmission, E‐C coupling, and contractility. © 1993 John Wiley & Sons, Inc.
Antigalactocerebroside antibody increases demyelination in adoptive transfer experimental allergic neuritis
Authors: Hahn Angelika F. , Feasby Thomas E. , Wilkie Lora , Lovgren David 
Published: January 2005

AbstractThere is suggestive but inconclusive evidence for a contribution of T cells and antimyelin antibodies to the pathogenesis of the Guillain–Barré polyneuropathy. We have studied the potential synergism of cellular and humoral immunity in the adoptive transfer model of EAN. EAN was induced in Lewis rats by injecting varying doses of P2 peptide (SP26)‐sensitized T lymphocytes. Disease severity was dose‐dependent. The addition of intravenous GC‐AB to a subclinical dose of SP26‐sensitized T cells resulted in overt clinical disease and markedly enhanced demyelination. Intravenous injection of antibody alone had no effect. We conclude that activated neuritogenic T cells, while entering into peripheral nerves, alter the blood‐nerve barrier, which gives circulating demyelinating antibodies access to the endoneurium. The observations support the concept of a synergistic role of T‐cell autoimmunity and humoral responses in the inflammatory demyelination of Lewis rat EAN. © 1993 John Wiley & Sons, Inc.
Peripheral neuromuscular manifestations in systemic sclerosis (scleroderma)
Authors: Hietaharju Aki , Jääskeläinen Satu , Kalimo Hannu , Hietarinta Marja 
Published: January 2005

AbstractSystemic sclerosis (scleroderma) is thought to be the least likely of the collagen vascular disorders to cause nervous system damage. We evaluated the peripheral neuromuscular manifestations in 32 patients with scleroderma. A clinically defined peripheral nervous system (PNS) lesion was manifest in 5 of 32 patients (16%), including 2 patients with trigeminal neuropathy and single cases of polyneuropathy, brachial plexopathy, and lumbosacral radiculopathy. Neurophysiological studies suggested subclinical PNS involvement in 6 additional patients (3 with distal axonal polyneuropathy, 1 with probable myopathy and superimposed polyneuropathy, 1 with trigeminal neuropathy, and 1 with focal ulnar neuropathy). Even though subjective muscular complaints were numerous (16 patients, 50%), a defined primary muscular disease could be demonstrated only in 5 patients (16%). Our results indicate that peripheral neuropathy in scleroderma is not as uncommon as previously estimated. © 1993 John Wiley & Sons, Inc.
Histochemical and contractile property changes during human muscle development
Authors: Elder Geoffrey C. B. , Kakulas Byron A. 
Published: January 2005

AbstractWhen the histochemical and contractile properties of infant muscles change postnatally, and what influence muscle function has on these changes, were the focus of this study. Contractile properties were measured in the plantaflexor (PF) and dorsiflexors (DF) of 19 newborns and 36 infants aged 5–16 months. Infants were tested between one and four times at monthly intervals. Measurements included maximal twitch tension (Pt), time to peak tension (TPT), and half‐relaxation times (1/2RT). TPT was similar in PF (77 ms) and DF (73 ms) at birth, remained unchanged in DF, and slowed in PF to 110–120 ms between 9 and 12 months. Type I distributions were determined at autopsy in fetal through to adult muscles. Completion of differentiation occurred in soleus at about the age that contractile properties slowed and infants started to use these muscles more. A trend of higher percentages of type I distributions was also noted in children than either newborns or adults in other muscles. The implications of these findings and the clinical use of these methods for evaluating peripheral neuromuscular function is discussed. © 1993 John Wiley & Sons, Inc.
Congenital myasthenic syndromes: I. Deficiency and short open‐time of the acetylcholine receptor
Authors: Engel Andrew G. , Nagel Alexandre , Walls Timothy J. , Harper C. Michel , Waisburg Hector A. 
Published: January 2005

AbstractA 5.5‐year‐old girl had myasthenic symptoms since birth. Tests for antiacetylcholine receptor (AChR) antibodies were negative. To investigate the character of the neuromuscular transmission defect, an intercostal muscle specimen was obtained at age 27 months. Immune deposits were absent from the endplates. On electron microscopy, most postsynaptic regions appeared normal, but the density of AChR on the junctional folds was diffusely reduced. In vitro microelectrode studies revealed that the number of transmitter quanta released by nerve impulse was normal. The amplitude of miniature of endplate potentials and currents was abnormally low. A study of the kinetic properties of AChR by analysis of acetylcholine‐induced current noise demonstrated a significant decrease in mean channel open‐time; the mean channel conductance was normal. The safety margin of neuromuscular transmission in this disorder is likely to be compromised by the deficiency and abnormal kinetic properties of AChR. The findings are unique among those patients with congenital AChR deficiency described to date. © 1993 John Wiley & Sons, Inc.
A statistical approach to fiber diameter distribution in human sural nerve
Authors: Schellens Ronald L. L. A. , van Veen Benno K. , Gabreëls‐Festen Annexe A. W. M. , Notermans Servaas L. H. , van't Hof Martin A. , Stegeman Dick F. 
Published: January 2005

AbstractFifty‐one normal sural nerve biopsies were obtained from 800 diagnostic biopsies. The external diameter distribution of myelinated fibers was described using the sum of two beta probability density functions, describing the thin as well as the thick fiber group. A cross‐sectional study using these distribution functions showed increasing values of the peak of the larger fiber group, the diameter of the thickest fibers, and the separation between the smaller and the larger groups until the beginning of adult life. The transition from a uni‐ into a bimodal histogram occurred gradually between 7 and 13 months. Total transverse fascicular area increased with age, whereas fiber density decreased significantly with age. The number of fibers remained stable over age. The relative proportion of the numbers of fibers in both groups described by one of the beta distributions remained constant over age. This occurred despite a marked decrease in the number of small fibers with a diameter less than, e.g., 6.5 μm. The results indicated an outgrowth of especially the larger myelinated fibers with age. This process continues with decreasing intensity into adult life. © 1993 John Wiley & Sons, Inc.
The cortical silent period and amyotrophic lateral sclerosis
Authors: Prout Alister J. , Eisen Andrew A. 
Published: January 2005

AbstractThe cortical silent period (C‐SP) was elicited by transcranial magnetic stimulation in 25 normal subjects and 19 patients with amyotrophic lateral sclerosis (ALS). The inhibitory (S‐X) period was highly stimulus intensity (Sl)‐dependent (mean r2 = 0.89 for both normals and patients with ALS). The range of the C‐SP (difference between maximum and minimum S‐X intervals) was age‐dependent for normals (r2 = 0.701, P < 0.001) but not patients with ALS. Means, maximums and ranges for the C‐SP were not significantly different between normal and ALS groups and thresholds to cortical stimulation were also comparable. There was a significant, linear, relation between the maximum C‐SP and disease duration of ALS (P = 0.002). The maximum C‐SP was shorter early in the disease. It is hypothesized that the reduced inhibition early in the course of ALS might reflect glutamate‐induced corticomotoneuronal excitotoxicity. © 1994 John Wiley & Sons, Inc.
Postsurgical idiopathic brachial neuritis
Authors: Malamut Richard I. , Marques Wilson , England John D. , Sumner Austin J. 
Published: January 2005

AbstractIdiopathic brachial neuritis (IBN) is a well‐recognized clinical syndrome characterized by brachial pain followed by a patchy amyotrophy of muscles in the shoulder girdle and arm innervated by individual branches of the brachial plexus. Postsurgical IBN has not been widely recognized since Parsonage and Turner's original description in which 10% of patients had antecedent surgery. We present 6 patients who 1–13 days postoperatively developed signs and symptoms which met the clinical and electrophysiologic criteria for IBN. Postsurgical neuralgic amyotrophy is an under‐recognized clinical entity which in most cases is ascribed to brachial plexus stretch injuries occurring during anesthesia. Early recognition of this condition may prevent unnecessary surgical exploration and allow for a more accurate prediction of functional recovery. © 1994 John Wiley & Sons, Inc.