bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prior history of head trauma in Parkinson's disease
Authors: Factor Stewart A. , Weiner William J. 
Published: January 2005

AbstractThe purpose of this study was to evaluate the relationship between head trauma and Parkinson's disease (PD). A questionnaire was completed by 97 PD patients (mean age 68.6 years, duration of disease 6.4 years) and 64 spouses acting as controls (mean age 63 years). Thirty‐one PD patients reported head trauma before onset of PD (mean 32.3 years earlier), whereas 11 controls reported head injury before completing the survey (mean 26 years earlier) (p < 0.05). Twenty PD patients and five controls reported head injury associated with alteration or loss of consciousness (p < 0.05). Injury occurred a mean of 37.7 years before onset of PD and 37.2 years before survey completion in the two groups, respectively. In both of these comparisons we adjusted the frequencies to eliminate sex differences between groups. Comparison of new frequencies showed differences that were not significant. However, a trend toward significance was observed when examining head trauma with alteration of consciousness. Other investigators have reported similar results indicating that previous head trauma may be a risk factor in the etiology of PD. However, recall bias might have an effect on the outcome of these studies and this is discussed. The relationship between head trauma and PD is still tenuous.
Voluntary stimulus‐sensitive jerks and jumps mimicking myoclonus or pathological startle syndromes
Authors: Thompson P. D. , Colebatch J. G. , Brown P. , Rothwell J. C. , Day B. L. , Obeso J. A. , Marsden C. D. 
Published: January 2005

AbstractFive patients who presented with stimulus‐induced jerking as part of an apparent myoclonic or pathological startle syndrome are reported. Neurophysiological observations in these patients suggested the jerks were voluntary in origin. These included (a) variable latencies to the onset of stimulus induced jerks, (b) latencies were greater than that seen in reflex myoclonus of cortical or brainstem origin, and were (c) longer than the fastest voluntary reaction times of normal subjects, (d) variable patterns of muscle recruitment within each jerk and, (e) significant habituation with repeated stimulation. It is argued that these features are consistent with a voluntary origin for the jerks and enable them to be distinguished from the stereotyped electrophysiological characteristics of myoclonus of cortical and brainstem origin. Electrophysiological recordings may help identify patients with this form of psychogenic movement disorder.
“Rubral” tremor induced by a neuroleptic drug
Authors: Friedman Joseph H. 
Published: January 2005

Abstract“Rubral” tremor is a rare movement disorder that occurs typically with midbrain damage. It is defined by its presence at rest, with sustained posture, and with movement. Whether it is a single‐tremor disorder or a combination of two distinct tremors is debated. This report chronicles a severe neuroleptic induced “rubral” tremor in a patient who had had a stable posttraumatic ataxia. The dramatic response to benztropine and bromocriptine is illustrated in the videotape.
Linkage studies in alcohol‐responsive myoclonic dystonia
Authors: Gasser T. , Bereznai B. , Müller B. , Pruszak‐Seel R. , Damrich R. , Deuschl G. , Oertel W. H. 
Published: January 2005

AbstractA large German family with “myoclonic dystonia with lightning jerks responsive to alcohol” was identified. Eleven affected pedigree members and six obligate gene carriers from five generations were identified. A description of one branch of this pedigree was published in 1964. Our examination 30 years after the initial report confirms the clinical syndrome of a nonprogressive movement disorder charcterized by myoclonic jerks affecting the proximal muscles and the muscles of the trunk, accompanied by mild dystonic features in some affected family members. Segregation analysis favors autosomal dominant inheritance with high, but incomplete, penetrance in males and much lower penetrance in females. Linkage analysis was performed using simple sequence repeat polymorphisms (CA repeats) closely associated with or spanning the chromosomal regions containing 15 candidate genes: the gene for early‐onset generalized torsion dystonia, DYTI (chromosome 9q34); the genes for subunits α2, β1, and γ1 (chromosome 4p12–4q13);for α1,α6,β2 and γ2(chromosome 5q31.1–5q31.3); for α4,α5,β3, and γ3 (chromosome 15q11–15q13); for ρ1 and ρ2 (chromosome 6q14–6q21) of the gamma‐aminobutyric acid A receptor; and for the alpha subunit of the glycine receptor (chromosome 5q31). By a combination of pairwise and multipoint linkage analysis, it could be excluded that any of these candidate gene bearing chromosomal regions contain the disease gene in this family. We also excluded major portions of three chromosomal regions syntenic with mouse chromosome 3, which carries the murine beta subunit of the glycine receptor.
Oral and genital pain syndromes in Parkinson's disease
Authors: Ford Blair , Louis Elan D. , Greene Paul , Fahn Stanley 
Published: January 2005

AbstractPainful sensory complaints are known to occur in Parkinson's disease, but painful oral or genital syndromes have not been described. We report seven individuals with presumed idiopathic Parkinson's disease and one with atypical parkinsonism who experienced chronic severe oral or genital pains that appeared to be examples of a primary sensory disturbance related to the underlying parkinsonism. In each case, the pain syndrome overshadowed the other features of the parkinsonism. Five patients experienced oral pain and three patients, all women, had genital pain. No other definable organic cause was detected to explain the symptoms in any case. The genital pain tended to fluctuate in severity with the motor manifestations of the parkinsonism and could be abolished or reduced by levodopa. The recognition of painful oral or genital sensations in patients with parkinsonism should lead to further study regarding the prevalence, neurochemical basis, and treatment for these disabling symptoms.
A new stain for quantitative measurement of sprouting at neuromuscular junctions
Authors: Pestronk Alan , Drachman Daniel B. 
Published: January 2005

AbstractA new combined stain for the simultaneous demonstration of motor nerve terminals and cholinesterase at neuromuscular junctions is described. It employs bromoindoxyl acetate dye‐staining for cholinesterase and silver‐gold impregnation for nerve terminals. The clarity and reliability of the stain permit quantitative measurements of neuromuscular junctions in order to evaluate nerve terminal sprouting as well as other pathological changes. The method is rapid, reproducible, and simple, and it is well suited for the processing of large numbers of frozen sections.
Myasthenia gravis: A personal view of pathogenesis and mechanism, part 2
Authors: Simpson John A. 
Published: January 2005

AbstractA review of our current knowledge of the etiology and pathogenesis of myasthenia gravis is presented, with particular emphasis on the immunological aspects of the disease. Part 1, published in the January/February issue of MUSCLE & NERVE, dealt with the clinical and genetic features of myasthenia gravis which led to the autoimmune theory of the etiology of the disease. Part 2, which appears in this issue, provides a review of the dysfunction of physiology, pharmacology, and structure of the neuromuscular junction in myasthenia gravis, and the part played by the autoimmune process.
Peripheral neuropathy in lead‐intoxicated sickle cell patients
Authors: Imbus Charles E. , Warner Judith , Smith Elaine , Pegelow Charles H. , Allen John P. , Powars Darleen R. 
Published: January 2005

AbstractPeripheral neuropathy and hypertension caused by lead intoxication are reported in two children with sickle cell anemia. One child had generalized weakness in the initial occurrence and distal paralysis during a relapse two years later. The second child had foot and wrist drop. Both had slow peripheral nerve conduction velocities during the episodes. Chelation therapy was successful and resulted in a return of strength (over a period of several months) and a normalization of the blood pressures. Children with sickle cell anemia who are subjected to lead intoxication appear to be predisposed to peripheral nerve damage.
Muscle trophic factor: II. Ontogenic development of activity of a muscle trophic factor in chicken serum
Authors: Kohama Kazuhiro , Ozawa Eijiro 
Published: January 2005

AbstractThe activity of chicken skeletal muscle cell trophic factor was greater in chicken serum than in other tissues examined. The variation of this activity in chicken serum was studied pre‐and post hatching. The activity of the factor in the embryo was high from day 11 to day 13 of incubation and then decreased, reaching a minimum on day 16. It subsequently increased again, reaching a peak around the day of hatching. After hatching, the activity in serum began to decrease on day 4, reaching a minimum at day 18. In the hen, activity did not vary significantly beyond day 18. In the rooster, however, activity increased gradually from about day 50, reached a new maximum by day 110, and was maintained at this level thereafter. The ratio of the average activity of serum from roosters older than 110 days to that of hens of the same age was about 4:3. The time course of activity of the trophic factor closely parallels that of the growth and differentiation of the muscle in vivo.
Early recognition of nerve disorders by near‐nerve recording of sensory action potentials
Authors: Rosenfalck Annelise 
Published: January 2005

AbstractEvaluated herein are the advantages and limitations of recording sensory action potentials with a gross electrode placed near the nerve. This technique has proved to be a sensitive measure of early nerve damage for two reasons: first, electrodes can be positioned in such a way that maximal velocity is determined only along the abnormal stretch of the nerve; and second, small late components originating from demyelinated, remyelinated, or regenerating fibers can be detected.
Acetylcholine receptor antibodies in the diagnosis of human and experimental myasthenia gravis
Authors: Heilbronn Edith , Lefvert Ann‐Kari , Stålberg Erik 
Published: January 2005

AbstractAspects of acetylcholine receptor immunology and circulating receptor antibodies are reviewed with regard to both human and experimental myasthenia gravis. Receptor antibodies that have negligible cross‐reactivity with skeletal muscle receptor can nonetheless cause destruction of the postsynaptic motor endplate area. Antibodies to mammalian skeletal muscle receptor can bind in‐situ receptors. Transfer of myasthenic IgG increases neurophysiologic symptoms in rabbits showing slight sings of experimental myasthenia gravis, suggesting a block of in‐situ receptors. Determination of receptor antibodies using RIA tests and partially purified human skeletal muscle receptor has been evaluated in the diagnosis of myasthenia gravis. Ninety percent of Swedish myasthenic patients in one study were found to have receptor antibodies. A rough correlation has been found between antibody titer and the severity of disease. Immunosuppressive treatment and thymectomy decrease the titer. In patients with thymoma, high antibody titers remain. When taken together, antibody‐titer determination and electrophysiologic tests–particularly single‐fiber electromyography–are very valuable diagnostic tools.
The effect of anticholinesterases on motor unit potentials in myasthenia gravis
Authors: Pinelli Paolo 
Published: January 2005

AbstractIn most patients with myasthenia gravis, the mean duration and amplitude of motor unit potentials sampled in the extensor digitorum communis muscle, as well as the amplitude of the pattern of activity during full effort, increased after application of anticholinesterase. This appears to be a sensitive test for early detection of failure in neuromuscular transmission. In a series of 12 patients, the mean amplitude of motor unit potentials increased 30%–130% in 9, and the mean duration increased 10%–25% in 7. In 3 patients who failed to show an increase in amplitude in the extensor digitorum communis muscle, the amplitude of motor unit potentials increased in a facial or extraocular muscle after intravenous application of edrophonium. This increase in the amplitude or duration of motor unit potentials was even evident in 4 patients who failed to show a decrement in response of the opponens pollicis muscle to repetitive stimuli delivered to the median nerve at 3/sec and 5/sec. In 5 patients, responses evoked by stimuli delivered at 15/sec showed marked facilitation.
Muscle and nerve changes induced by perhexiline maleate in man and mice
Authors: Fardeau M. , Tomé F. M. S. , Simon P. 
Published: January 2005

AbstractPerhexiline maleate therapy may produce a polyneuropathy as well as other side effects in man. The Schwann‐cell origin of the neuropathy is demonstrated by morphologic studies. Ultrastructural studies of muscle and nerve biopsies showed the presence of intracytoplasmic inclusions, primarily in Schwann cells, muscle fibers, and endothelial cells. These inclusions were polymorphic and varied from one cell type to another. They were frequently associated with calcium deposits, and their structure suggested a lysosomal origin. It was possible experimentally to reproduce similar inclusions in mice. Furthermore, numerous tubular aggregates were observed in intoxicated mouse muscle fibers.
Inflammatory myopathy in oculopharyngeal dystrophy
Authors: Bosch E. Peter , Gowans James D. C. , Munsat Theodore 
Published: January 2005

AbstractA 75‐year‐old French‐Canadian woman with familial oculopharyngeal dystrophy demonstrated histopathologic alterations similar to those of idiopathic polymyositis. A second biopsy obtained 15 months later was more consistent with previously reported cases. It is suggested that certain patients with oculopharyngeal dystrophy may pass through an initial phase of secondary muscle inflammation similar to that seen in some other heritable myopathies.
Congenital hypotonia revisited
Authors: Brooke Michael H. , Carroll James E. , Ringel Steven P. 
Published: January 2005

AbstractThe definition of neuromuscular diseases affecting infants has depended on factors as various as the rate of progression of the illness, the clinical picture, and, recently, the morphologic peculiarities in the muscle biopsy. A review of the literature suggests that there are discrepancies in the classification of such illnesses, no matter what system is used. In some instances, a single diagnosis seems to include patients with quite separate illnesses, whereas other patients with seemingly identical diseases have been given different diagnoses.
F waves in patients with hand wasting caused by a cervical rib and band
Authors: Wulff C. H. , Gilliatt R. W. 
Published: January 2005

AbstractIn five patients with unilateral wasting of the hand muscles as a result of a cervical rib and band, F waves were recorded from the hypothenar muscles following stimulation of the ulnar nerve at the wrist. Twelve healthy control subjects were also examined. F‐wave latency was consistently increased in the affected hands of the patients, compared with results from the unaffected and control hands. This increase persisted after removal of the cervical band in four patients and was thought to be mainly due to degeneration of fast‐conducting fibers. In the fifth patient, F‐wave latency decreased after operation, suggesting recovery of a local conduction block in the proximal part of the nerve.
Actomyosin ATPase. II. Fiber typing by histochemical ATPase reaction
Authors: Mabuchi K. , Sréter F. A. 
Published: January 2005

AbstractA new method for fiber typing based on staining for actomyosin Ca, Mg‐ATPase is presented. Inclusion of ethanol in the medium enhanced the differentiation of type 2A and 2B fibers. With this technique, type 1, 2A, and 2B fibers can be distinguished in a single‐step procedure for human biopsy samples.
Element analysis of skeletal muscle in duchenne muscular dystrophy using x‐ray fluorescence spectrometry
Authors: Maunder‐Sewry Caroline A. , Gorodetsky Raphael , Yarom Rena , Dubowitz Victor 
Published: January 2005

AbstractConcentrations of calcium, iron, copper, and zinc were simultaneously measured by x‐ray fluorescence spectrometry in muscle biopsies from 11 normal subjects, 13 patients with Duchenne muscular dystrophy (DMD), and 3 DMD carriers. Calcium and copper levels were significantly elevated in patients with DMD compared to controls, and one presumptive carrier also showed an elevated calcium concentration. Comparisons with morphological data for the same samples showed a significant positive correlation between calcium concentration and the percentage area of type 2 fibers in controls, but no other correlations were found to be significant. These results illustrate the value of x‐ray fluorescence spectrometry and provide further evidence for the involvement of calcium in dystrophic processes.
An experimental morphometric study of neutral lipid accumulation in skeletal muscles
Authors: Odusote Kayode , Karpati George , Carpenter Stirling 
Published: January 2005

AbstractThe neutral lipid content of skeletal muscle fibers was determined by computing the lipid accumulation index (LAI) on transverse cryostat sections stained with Oil Red O. The LAI was defined as: (total area of neutral lipid droplets in a fiber) × 100/(total cross‐sectional area of a fiber). The biceps, diaphragm, and soleus muscles were studied in 3 groups of guinea pigs: normal animals, animals fasted for 48 hours, and animals subjected to muscle denervation and then fasted for 48 hours. and animals, subjected to muscle denervation and then fasted for 48 hours. In normal animals, the highest mean LAI was found in the diaphragm (4.93) in comparison with lower values in the biceps (2.25) and soleus (2.09). After fasting, these values were markedly increased; there was also a concomitant increase in plasma free fatty acid (FFA) concentration. Prior denervation further increased the LAI in biceps and soleus but reduced it in the diaphragm. Type 2A fibers tended to show high lipid accumulation when the plasma FFA concentration was high. Type 2B fibers never accumulated much lipid under any circumstances. Type 1 fibers varied in their response in the different muscles.
Plasma and red blood cell acetylcholinesterase in amyotrophic lateral sclerosis
Authors: Festoff Barry W. , Fernandez Hugo L. 
Published: January 2005

AbstractRed blood cell and plasma cholinesterases were evaluated in control subjects and patients with the major forms of adult, sporadic motor neuron disease. For the purposes of this communication, the patients were considered as having amyotrophic lateral sclerosis (ALS) or its subtypes. Cholinesterase and acetylcholinesterase activities were evaluated and separated by dose response to their respective inhibitors. No kinetic differences were observed comparing red blood cell or plasma enzyme activities using either inhibitor. As found in previous studies, acetylcholinesterase accounted for more than 90% of acetylcholine hydrolysis in red blood cells. The plasma data were more complicated to evaluate, but at least 20% of total activity could be attributed to acetylcholinesterase. When red blood cell acetylcholinesterase activities of patients and controls were compared, no statistically significant difference was found. However, when plasma acetylchlinesterase activity was compared between the 2 groups, a statistically significant increase, almost twice the control value, was found in the ALS patients. These data may ultimately be important in the prognosis of this disease and, conceivably, could aid in understanding its pathogenesis.
Fresh and predegenerate nerve allografts and isografts in trembler mice
Authors: Pollard John D. , McLeod James G. 
Published: January 2005

AbstractIn order to investigate whether Schwann cell or myelin was the principal antigen responsible for nerve graft reiection, fresh nerve grafts and those in which myelin had been previously allowed to degenerate (predegenerate grafts) from both isogeneic BALB/c and allogeneic C57/B1 mice were inserted into trembler BALB/c mice. Schwann cells within nerve allografts from C57/B1 mice were rejected, whether or not the grafts contained myelin. Nerve isografts from normal BALB/c animals produced normally myelinated trembler axons within the grafted segments, and across these segments conduction velocity was restored towards the normal value. It is concluded that Schwann cells, not myelin, constitute the principlal antigen within nerve allografts and it is Schwann‐cell rejection that limits the sucessful use of nerve allografts.
Morphological and biochemical changes in muscle and peripheral nerve in Fabry's disease
Authors: Pellissier J. F. , van Hoof F. , Bourdet‐Bonerandi D. , Monier‐Faugere M. C. , Toga M. 
Published: January 2005

AbstractIn a case of Fabry's disease, microscopic, ultrastructural, and biochemical studies of a muscle biopsy were performed, as well as microscopic, ultrastructural, and morphometric studies of a nerve biopsy. Pleomorphic lipid inclusions were observed in muscle fibers, fibroblasts, and endomysial capillaries. Moreover, the thermolabile isoenzyme A of α‐D‐galactosidase was almost completely absent. In the nerve specimen, polymorphous lysosomes were noted in perineural cells, in fibroblasts, and in endothelial and perithelial cells in association with some nonspecific degenerative changes. The morphometric data revealed a loss of large myelinated fibers, an uncommon finding in Fabry's disease, and a decrease of the average diameter of the unmyelinated fibers, which was related to axonal sprouting. The relationship between the pain attacks and the increased number of the small unmyelinated fibers is discussed.
Late changes in the motor unit after acute poliomyelitis
Authors: Wiechers David O. , Hubbell Susan L. 
Published: January 2005

AbstractPatients who have suffered an acute attack of poliomyelitis may develop a clinically evident progression of weakness later in life. A syndrome known as forme fruste amyotrophic lateral sclerosis occurs in severely affected older polio patients. Neither process is well understood. Ten subjects who had had polio at least 22 years prior to examination and had no complaints of rapid progression of weakness were studied. Single‐fiber recordings were made from the extensor digitorum communis or the tioialis anterior muscle. Fiber densities were increased in all patients. Eight of 10 patients showed marked abnormalities in jitter in more than 50% of the recordings examined. There was a significant association between the percentage of recordings with abnormal jitter and the time since the attack of polio. Blocking was seen in more than 30% of the recordings examined in 6 of 10 patients. There was a significant association between the percentage of recordings demonstrating blocking and both the chronological age and the number of years since the attack of polio. These abnormalities may represent disintegration with aging in the reinnervated motor units.