bluuebunny/crossref_metadata_2025_split huggingface dataset:
The autonomic nervous system in Gilles de la Tourette's syndrome
Authors: Goetz Christopher G. , Shannon Kathleen M. , Carroll V. Susan , Tanner Caroline M. , Weingarten Randall 
Published: January 2005

AbstractTwenty‐three patients with Gilles de la Tourette's syndromes (GTS) underwent nonivasive investigation of autonomic nervous system (ANS) function, as did 23 age‐matched controls. ANS function in GTS patients was no different from that of controls, and patients receiving neuroleptic drugs had the same ANS function as untreated patients. All 23 patients later received clonidine and were retested. The ANS values before administration of clonidine were compared with those while patients were taking clonidine. The only significant change (p <0.01) with clonidine was a reduced resting pulse rate. The combination of clonidine and neuroleptic drugs did not induce significant autonomic changes compared with neuroleptoc therapy alone. These results indicate that the ANS in GTS patients is normal and that the drugs used to abate tics do not produce clinically significant changes in ANS when chronically given. The findings suggest that the pathophysiology and treatment of GTS do not directly involve the nuclei or tracts of autonomic regulation.
Stability of the head: Studies in normal subjects and in patients with labyrinthine disease, head tremor, and dystonia
Authors: Gresty Michael 
Published: January 2005

AbstractThe dynamics of postural control of the head were investigated in normal human subjects and patients with neurological disease. The technique adopted was to measure the head movements provoked by passive, unpredictable oscillations of the trunk in the frequency range 0–6–7 Hz when subjects were required (a) to try to stabilise their head “in space” and (b) to try to make their heads move “en bloc” with the trunk. Head movement responses were characterised by the gain and phase with respect to trunk movement (transfer function) and degree of linear relationship between head and trunk (coherence). The normal transfer function approximated a cascade of two secondorder, underdamped, systems representing the passive inertial, viscous, and elastic properties of the muscle and joints of the head and neck. Stabilisation of the head “in space” produced about 40% reduction in transmission of body movement, was only evident at frequencies <1 Hz and was affected partly by voluntary movements. An alabyrinthine patient could also achieve some spatial stabilisation. The findings indicate a weak role for vestibular‐collic reflexes and emphasise that the primary control of head posture during unpredictable movement is through the tonic visco‐elastic properties of neck muscles that work to stabilise the head on the shoulders. In patients with dystonia of the nekc and essential and cerebellar head tremor, the dystonia could be measured in terms of visco‐elastic constants and damping ratios. The head movement of some tremor patients did not linearly follow the trunk movement, showing that the motion stimulus provoked abnormal phasic muscle activity at frequencies other than those of the tremor. The technique quantifies head control in movement disorders and is sensitive to abnormal function.
Corneal and blink reflexes in Parkinson's disease with “on‐off” fluctuations
Authors: Agostino R. , Berardelli A. , Cruccu G. , Stocchi F. , Manfredi M. 
Published: January 2005

AbstractIn 13 patients with Parkinson's disease and “on‐off” fluctuations and in a control group, the electrically evoked blink (R1 and R2 component) and corneal reflexes (CR), the habituation of the glabellar reflex, and the blink rate were studied. Latency, amplitude, and recovery cycle of R1 was normal. CR latency, but not R2 latency, was significantly shorter in patients than in normal subjects. R2 recovery cycle, but not CR recovery cycle, was facilitated in “off” patients in comparison to normal subjects. CR and R2 duration was longer in “off” than in “on” patients. R2 recovery cycle became similar to that of normal subjects after fluctuation from “off” to “on” period, but CR recovery cycle varied only slightly. The habituation of the glabellar reflex and the blink rate were decreased in “off” patients. These findings indicate that the enhanced excitability of R2 is related to the central dopamine activity levels. The differences between the recovery cycles of CR and R2 may be explained with the smaller number of interneurons subserving CR than R2 circuit. The shorter latency of CR, but not of R2, both in “on” and “off” patients, may suggest that in Parkinson's disease basal ganglia exert a different control of incoming sensory input transmitted via beta and delta fibers.
Tardive myoclonusa
Authors: Little John T. , Jankovic Joseph 
Published: January 2005

AbstractWe describe a 46‐year‐old, schizophrenic woman with late‐onset myoclonus after treatment with antipsychotic drugs. The myoclonic jerking of the neck with synchronous contractions of the face persisted after all the antipsychotic drugs had been discontinued. Tardive myoclonus has not been documented previously.
Analysis of open‐label trials in torsion dystonia using high dosages of anticholinergics and other drugs
Authors: Greene Paul , Shale Heidi , Fahn Stanley 
Published: January 2005

AbstractWe reviewed the records of 358 patients with various forms of focal, segmental, and generalized dystonia who had received pharmacotherapy in a systematic order, beginning with anticholinergics. If no benefit was encountered, we then tested clonazepam, baclofen, and other agents sequentially. In each situation the dosage was gradually increased until benefit or troublesome adverse effects were encountered. In this manner we obtained data on the percentage of patients who showed moderate to marked benefit. Anticholinergics were the most beneficial agent, confirming previous reports. Statistical analysis of this response revealed that benefit from anticholinergics was most likely if treatment was begun within 5 years after the onset of dystonia. From this analysis, it appears that delaying treatment beyond 5 years is likely to result in an unsatisfactory response.
Hemiballism in multiple sclerosis
Authors: Riley David , Lang Anthony E. 
Published: January 2005

AbstractHemiballism has rarely been reported in the setting of multiple sclerosis. Review of clinical descriptions cast some doubt on the accuracy of the diagnosis of the movement disorder in several previous cases. We report a patient with definite multiple sclerosis, with videotape documentation of hemiballism, and demonstration of a plaque in the contralateral subthalamic nucleus on a magnetic resonance image.
The first agonist and antagonist burst in patients with an upper motor neuron syndrome
Authors: Fagioli S. , Berardelli A. , Hallett M. , Accornero N. , Manfredi M. 
Published: January 2005

AbstractRapid elbow flexion movements were studied in patients with an upper motor neuron syndrome following a stroke. The velocity of movements was slower than normal. The initial bursts of electromyographic (EMG) activity in both the agonist and antagonist muscles were prolonged. As in normal subjects, the first agonist burst increased in duration with larger movements, but it generally remained about 40 ms longer than normal. The size of the first agonist burst also increased with larger movements. A fixed linkage between burst duration and level of motor unit recruitment, together with a deficient corticospinal command, could explain the prolonged burst duration with preserved ability to modulate the burst.
Lower body parkinsonism: Evidence for vascular etiology
Authors: Fitzgerald Patricia M. , Jankovic Joseph 
Published: January 2005

AbstractWe studied 10 patients with marked gait difficulty and no or only minimal upper limb involvement, defined here as lower body parkinsonism (LBP). They were compared to a control group of 100 patients with otherwise typical Parkinson's disease (PD). Both groups were of comparable age, but the mean duration of symptoms was significantly shorter in the LBP group (2.6 ± 1.5 years versus 7.5 ± 4.9 years). Gait disturbance was the initial symptom in 90% of LBP patients, as opposed to 7% of controls. Hypertension was present in 70% of LBP patients, and only 22% responded to levodopa. In contrast, only 21% of controls had a history of hypertension, and 96% improved with levodopa. We conclude that these 10 LBP patients constitute a homogenous group, distinct from typical PD. Besides their disproportionate gait disturbance, they are distinguished from PD patients by more rapid progression, higher incidence of hypertension, and a poor response to levodopa. Ischemic etiology for LBP is supported by abnormal neuroimaging studies.
Hemichorea‐hemiballismus associated with acquired immune deficiency syndrome and cerebral toxoplasmosis
Authors: Sanchez‐Ramos Juan R. , Factor Stewart A. , Weiner William J. , Marquez Jose 
Published: January 2005

AbstractA young woman had hemichorea‐hemiballismus subsequently found to be secondary to a cerebral toxoplasmosis infection complicating human immunodeficiency virus infection. This patient had the sixth reported case of acquired immune deficiency syndrome (AIDS) with hemichorea‐hemiballismus, and each has been secondary to cerebral toxoplasmosis. The presence of hemichorea‐hemiballismus in a young patient should suggest a diagnosis of AIDS and in particular the diagnosis of secondary cerebral toxoplasmosis. Other movement disorders that occur in AIDS are discussed.
Spasms of amputation stumps: A report of 2 cases
Authors: Marion M. H. , Gledhill R. F. , Thompson P. D. 
Published: January 2005

AbstractThe occurrence of involuntary movements of stumps follwing amputation is described in two patients. Although recognised for over 100 years, this phenomenon has received little attention in the modern literature. The condition appears to represent a variant within the spectrum of movement disorders induced by injury to the peripheral nervous system.
Complex hyperkinesia during recovery from left temporoparietal cortical infarction
Authors: Ferbert A. , Rickert H. B. , Biniek R. , Brückmann H. 
Published: January 2005

AbstractA 51‐year‐old man with preexistant lacunar infarctions in the basal ganglia and pons developed complex involuntary movements of his right arm 1 day after a purely cortical‐subcortical ischemic infarction in the territory of the left middle cerebral artery sparing the basal ganglia. The movements were stereotyped and repetitive, affecting hand and fingers or proximal arm muscles alternatively or together. Although hyperkinesia after basal ganglia lesion has been described many times in the literature, it has not yet been reported after cortical‐subcortical ischemic infarctions sparing the basal ganglia.
Dystonic tics in patients with Tourette's syndrome
Authors: Jankovic Joseph , Stone Lael 
Published: January 2005

AbstractDystonic tics, in contrast to clonic tics, are relatively slow and temporarily sustained twisting, pulling, or squeezing movements producing briefly maintained abnormal postures. Because dystonic tics often present diagnostic difficulties, we studied these motor tics in 156 patients with Tourette's syndrome (TS). In addition to clonic motor and vocal/phonic tics, seen in all patients, 89 (57%) of them exhibited one or more dystonic tics. The most common dystonic tics were oculogyric deviations (43 patients), blepharospasm (23), and dystonic neck movements (11). Blinking (110) and facial twitching (86) were the most common clonic tics. Except for possibly a greater familial occurrence, a higher frequency of previous head trauma, and associated attention deficit disorder, the group of patients with dystonic tics did not significantly differ in any of the major clinical variables from those TS patients without dystonic tics. We conclude that dystonic tics are typical motor manifestations of TS and that patients with these motor tics are not different from those with more typical clonic tics.
Muscle relaxation in Parkinson's disease: A reaction time study
Authors: Grasso M. , Mazzini L. , Schieppati M. 
Published: January 2005

AbstractWe tested the hypothesis that the relaxation reaction time in Parkinson's disease (PD) is delayed, as a sign of disorder in the control of voluntary motoneuron derecruitment. We compared, in the triceps brachii muscle, the reaction times (RTs) of the onset (O‐RT) of electromyographic (EMG) activity during initation of a contraction with the RTs of the termination of EMG tonic activity during full relaxation (R‐RTs). Fourteen patients with idiopathic PD and 10 normal controls were examined. Mean R‐RTs for all controls were 30 ms shorter than mean O‐RTs. Mean R‐RTs for all patients were ∼70 ms longer than mean O‐RTs. In two untreated patients levodopa therapy improved both O‐RT and R‐RT, but the difference between the two was unchanged. There was no correlation between EMG level and R‐RT or between peak force and O‐RT in either controls or patients. O‐RT and R‐RT were correlated with the bradykinesia score. In some patients, bursts of late activity were recorded after the R‐RT; the duration of this activity was correlated with the duration and staging of the disease and with bradykinesia and rigidity scores. The reversed latency of onset and termination of muscle contraction in PD suggests an abnormality in the inhibitory spinal mechanisms, possibly stemming from a defect in the pathways descending to the spinal cord.
Autosomal dominant “spheroid body myopathy”
Authors: Goebel Hans H. , Muller Jans , Gillen H. William , Merritt A. Donald 
Published: January 2005

AbstractA slowly progressive autosomal dominant neuromuscular disease–termed spheroid body myopathy–is described in four successive generations and documented by muscle biopsies in five patients of two generations. With an onset in adolescence, the disease proceeds to some motor incapacitation, but life span is apparently not shortened. The salient morphologic feature is the presence of spheroid bodies, chiefly occurring in type 1 myofibers. Ultrastructurally, these spheroid bodies are composed of fine filaments but are devoid of organelles; in some cases they resemble cytoplasmic bodies. “Smearing in the l‐band” is a frequent and early finding. At a later age, signs of denervation are also present, both clinically and in muscle biopsies. The clinical and morphologic features justify the designation of this neuromuscular condition as a distinct entity.
F‐wave conduction velocity in the deep peroneal nerve: Charcot‐marie‐tooth disease and dystrophia myotonica
Authors: Panayiotopoulos Chrysostomos P. 
Published: January 2005

AbstractThe F‐wave has been used to estimate the motor nerve conduction velocity (MNCV) along the proximal segment (spinal cord to knee) of the axons of the deep peroneal nerve in patients with Charcot‐Marie‐Tooth disease and those with dystrophia myotonica. A new, modified method has been applied to estimate proximal MNCV in patients in whom F‐waves or M‐responses cannot be obtained from the small muscles of the foot. Terminal latencies and MNCV along the distal nerve segment (knee to ankle) have also been estimated using conventional techniques. The results have been compared with those obtained for control subjects. Proximal MNCV was severely slowed in every patient with Charcot‐Marie‐Tooth disease; the degrees of proximal and distal MNCV decreases were related. In patients with dystrophia myotonica, distal and proximal MNCVs were significantly reduced in comparison with control subjects; the MNCV slowing was not related to the degree of muscle atrophy. This is consistent with the hypothesis that the nerves and muscles are independently affected in dystrophia myotonica. It is concluded that the F‐wave MNCV technique is as useful as, and may be more sensitive than, the conventional MNCV method.
Neonatal experimental autoimmune myasthenia gravis
Authors: Sanders Donald B. , Cobb Edith E. , Winfield John B. 
Published: January 2005

AbstractNeonatal rats born of and nursed by mothers immunized with Torpedo acetylcholine receptor protein developed a defect of neuromuscular transmission as indicated by reduced miniature endplate potential amplitudes. It is likely that antibodies to the Torpedo receptor protein were passively transferred to the neonates in the milk. With the exception of the route of transfer, this neonatal from of experimental autoimmune myasthenia gravis appears to be similar to its human counterpart, and thus can serve as an experimental model.
Acute muscle necrosis after chronic overdosage of phenformin and fenfluramine
Authors: Palmucci L. , Bertolotto A. , Schiffer D. 
Published: January 2005

AbstractA case of acute muscle necrosis with probable myoglobinuria is presented. The patient had been taking excessive doses of phenformin and fenfluramine, and a toxic etiology is suggested. Muscle necrosis is tentatively attributed either to the combined effect of these drugs on some underlying biochemical abnormality of muscle or to a defect of oxygen utilization secondary to myoglobin alterations.
Thenar hypoplasia with associated radiologic abnormalities
Authors: Cavanagh N. P. C. , Yates D. A. H. , Sutcliffe J. 
Published: January 2005

AbstractWe report on five patients who presented with unilateral or bilateral hypoplasia of the thenar eminence. X rays of the affected limbs showed varying degrees of malformation and underdevelopment of the thumb phalanges and first metacarpal bone, and of the carpal bones at the base of the thumb, indicating a developmental abnormality. Our electrodiagnostic findings were compatible with this interpretation. We suggest that this condition is part of the clinical spectrum of radial club hand (radial dysplasia) and that when seen in adults, its recognition is important in the differential diagnosis of carpal tunnel syndrome.
Actomyosin ATPase. I. Quantitative measurement of activity in cryostat sections
Authors: Mabuchi K. , Sréter F. A. 
Published: January 2005

AbstractA method for the quantitative study of the actomyosin ATPase activity (Ca, MG‐ATPase) in thin sections cut in a cryostat is presented. This method is based on the liberation of 32P from [γ32P]ATP or 45Ca phosphate precipitation. The advantage of this method lies in the requirement for only a small muscle sample and the availability of serially cut sections for other assays including Ca uptake by sarcoplasmic reticulum and histochemical tests for oxidative and glycolytic enzymes. The actomyosin ATPase activity for various types of muscles determined by this method showed the same sequence found in isolated protein, that is, fast‐twitch skeletal > slow‐twitch skeletal > cardiac. The Ca, Mg‐ATPase of cryostat sections showed Ca sensitivity. The fact that the sections retained Ca sensitivity at 37°C, in contrast to myofibrils, which have been reported to lose Ca sensitivity at this temperature, indicates that the structural integrity of the contractile and regulatory apparatus is preserved to a higher degree in sections than in isolated myofibrils.
Microanalysis of perineural calcification in diabetic nephropathy
Authors: Kalimo Hannu , Mäki Jouko , Paetau Anders , Haltia Matti 
Published: January 2005

AbstractThree different microanalytical methods were used to identify calcium deposits in the sciatic nerve perineurium of a 33‐year‐old woman who died after developing nephropathy, retinopathy, and neuropathy as complications of diabetes. Scanning electron microscopic x‐ray microanalysis (SEM‐XMA) enabled localization of calcium and phosphorus elements to the perineurium in whole nerve cross sections. With SEM‐XMA and scanning‐transmission electron microscopic x‐ray microanalysis (STEM‐XMA) of the minute crystallites in the perineurium, the approximate relative amounts of calcium and phosphorus in the deposits were found to correspond to those in calcium hydroxyapatite. Finally, the crystallites were specifically identified by selected area electron diffraction, and were found to be composed mainly of calcium hydroxyapatite.
Neuron specific in vitro cytotoxicity of sera from patients with amyotrophic lateral sclerosis
Authors: Roisen Fred J. , Bartfeld Harry , Donnenfeld Hyman , Baxter Janet 
Published: January 2005

AbstractSera from patients with amyotrophic lateral sclerosis (ALS) were assayed for a neuron‐specific cytotoxic effect on long‐term organized cultures of neonatal mouse anterior horn segments. Blind studies show that ALS sera when incorporated into the culture media have a greater degree of antineuronal toxicity than sera from patients with other neurological diseases or from family members of ALS patients.
Regeneration of peripheral nerve through a polyglactin tube
Authors: Molander Hårkan , Olsson Yngve , Engkvist Ove , Bowald Staffan , Eriksson Ingvar 
Published: January 2005

AbstractPolyglactin 910, a resorbable synthetic material, was used as a mesh‐tube to bridge defects (7 to 9 mm in length) in sectioned rabbit tibial nerve. After absorption of the mesh a new nerve sheath was formed which enclosed numerous minute fascicles of regenerating axons. The polyglactin tube influenced the direction taken by the regenerating axons and guided them into the distal segment. The tube also reduced the formation of neuromas and the growth of scar tissue from surrounding structures.
Exercise effects on recovery of muscle acetylcholinesterase from reduced neuromuscular activity
Authors: Gardiner Phillip F. , Lapointe Mario , Gravel Diane 
Published: January 2005

AbstractIn order to investigate the effects of reduced and subsequently increased neuromuscular activity on muscle acetylcholinesterase (AChE), rats had one hindlimb immobilized with plaster casts for 4 weeks and were killed either at the end of immobilization (group I), after 4 weeks of resumed normal activity following cast removal (group R), or after 4 weeks of resumed activity supplemented with a daily treadmill‐walking task (group E). Immobilization resulted in a decrease in adductor longus muscle weight to 66.4% of control; total muscle end‐plate‐associated AChE was decreased to 51.4%. Total muscle ACh hydrolysis was not significantly affected. Mild daily exercise during recovery increased total muscle end‐plate AChE to control levels after 4 weeks, while in group R the corresponding level was significantly lower (84.4%). Decreased neuromuscular activity has different effects on end‐plate AChE and non‐end‐plate AChE. Mild endurance‐type overload during recovery from immobilization can accelerate recovery of end‐plate AChE activity to normal.
Chronic denervation of rat diaphragm: Selective maintenance of adult fast myosin heavy chains
Authors: Carraro Ugo , Libera Luciano Dalla , Catani Claudia , Danieli‐Betto Daniela 
Published: January 2005

AbstractAfter long‐term denervation in mixed rat muscles there is a selective loss of slow myosin. The bidimensional electrophoretic pattern of light chains and the results of preliminary studies on heavy chains have left open the question of whether the nature of the residual fast‐like myosin is of the immature or adult type. We have further investigated chronically denervated myosin by (1) electrophoresis in nondissociating conditions; (2) acidic electrophoresis of the heavy chains; and (3) proteolytic mapping of the heavy chains. These techniques clearly distinguish adult myosins from those present in immature muscles. Using these criteria, myosin from the chronically denervated diaphragm is of an adult type, even though the presence of trace amounts of embryonic myosin cannot be excluded. The contractile properties also indicate that chronically denervated hemidiaphragm is more similar to an adult fast muscle than to an immature muscle. The selective maintenance after long‐term denervation of fast myosin in adult muscle provides good evidence of the independence of the genetic expression of myosin from a direct neural chemotrophic control.