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The relationship between parkinsonian rigidity and hypokinesia in the orofacial system: a quantitative analysis.

Muscle rigidity was quantified in the labial muscles of parkinsonian and normal subjects by applying known forces and observing the resultant displacements to determine labial stiffness coefficients. In parallel, labial speech movements were observed from these same subjects to evaluate hypokinesia. Labial rigidity was consistently correlated with decrements in the range of lip movement. EMG from orbicularis oris and mentalis muscles indicated a causal relationship between rigidity and hypokinesia.

Aged↗

Progression of gait disorder and rigidity and risk of death in older persons.

BACKGROUND: Bradykinesia, gait disturbance, rigidity, and tremor are common motor signs in old age. All of these signs are associated with increased morbidity and mortality, but the extent to which they are progressive is unknown. METHODS: Study participants were 787 older Catholic clergy members without clinically diagnosed PD, related conditions, or dementia at baseline. They were evaluated annually for up to 7 years, with >95% follow-up participation by survivors. Evaluations included administration of a modified version of the motor portion of the Unified PD Rating Scale (UPDRS), from which previously established measures of the global UPDRS and four specific motor signs were derived. Scores represent the percent of the total possible UPDRS score obtained. RESULTS: At baseline, the global UPDRS score ranged from 0 to 36.3 (mean +/- SD, 7.3 +/- 6.4). It increased by an average of 0.69 unit per year during follow-up, with more rapid progression in older persons, but there was wide variability with no progression in 21% of subjects and annual increases of up to 8.23 units in the remaining 79%. Of 129 persons who died, 106 had follow-up UPDRS data. In a proportional hazards model, risk of death was associated with both the level of the global UPDRS score at baseline and the annual rate of progression (both p < 0.001). Overall, risk of death in subjects who had some worsening of the global UPDRS score was 2.93 times the rate among those without progression (95% CI, 1.32-6.50). Gait disorder/postural reflex impairment and rigidity worsened, but bradykinesia and tremor did not. Risk of death was associated with worsening of gait/posture but not with the other signs. CONCLUSION: Gait disorder and rigidity, as assessed with the modified UPDRS, are usually progressive in old age. Both the severity of the gait disorder and its rate of progression are strongly associated with risk of death.

Aged↗

Decerebrate rigidity in animals.

Decerebrate rigidity (DR) in animals is caused by a release of spinal neurons from supraspinal inhibition, which results in a caricature of reflex standing and includes tonic neck and labyrinthine reflexes. The reticular formation, cerebellum, vestibular complex, spinal cord, and muscle spindle system and their neurophysiological interaction are critical to DR. Its discovery and investigation were essential to Sherrington's concept of the integrative action of the nervous system. There are two types of DR with different anatomical and physiological bases. Intercollicular decerebration yields rigidity in extensor muscles that results from bilateral destruction of the central tegmental tracts, is abolished by posterior root section, and is due to a facilitation of gamma motoneuron discharge (gamma animal). Anemic decerebration is characterized by excessive extensor rigidity, depends upon the release of tonic labryinthine reflexes from cerebellar inhibition, is independent of posterior root section, and is caused by excessive alpha motoneuron discharge (alpha animal). DR has provided an insight into the mechanisms of posture and standing, but the correlation of laboratory observations and results from animals to humans must be made with caution.

Animals↗

Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report.

Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and developed encephalopathy with cranial nerves signs and long tract spinal cord symptomatology. Brain CT scan and spinal MRI were normal. The CSF showed lymphocytic pleocytosis and no other abnormalities. EMG showed involuntary muscle activity with 2-6 seconds of duration, interval of 30-50 ms and a frequency of 2/second in the left lower limb. Anti-GAD antibodies were detected in the blood. We detected radiological signs of lung cancer during the follow-up, which proved to be an oat cell carcinoma. The patient died two weeks after the diagnosis of the cancer.

Aged↗

[Relationship of decerebrate rigidity maintenance with muscular contraction permanence after spinal cord section in dogs].

The mammalian spinal cord seems to be capable of information retation in simmilar fashion to the cerebral cortex memory storation. The intercollicular transection otherwise, relieving bulbar and mesencephalic areas from cerebral and cerebelar influences produces tetanic contraction on antigravidity muscles due to somatory with stimuli originated from muscle spindle. This phenomena is very suitable to memory retention studies since myotatic reflex is monossinaptic, occurs in this case, on a simple nervous structure (the spinal cord) and can be easily identified and quantitified, through muscular responses. In the present work, decerebrate rigidity effects upon spinal cord before and after spinal section, was studied. Physiological parameters were recorded in order to maintain the experimental condition of the dogs, as close as possible to normal, Our results showed that thirty and forty-five minutes in time difference of decerebrate rigidity influence on spinal motoneurons has little effects in tetany persistance after spinal section. However, ninety minutes period of time between the two transections produces considerable increase in rigidity permanence after spinal cord section. The relationship of our results with differents types of memory and the basic mechanism involved in the response is unknown and will be the subject of future investigations.

Animals↗

[Progressive encephalomyelitis with rigidity. Clinical and electrophysiological aspects].

A 72-year-old man presented with a chronic illness constituted by muscle rigidity affecting his lower limbs, trunk and neck, either spontaneous or triggered by stimuli, together with a spastic paraparesis, manual amyotrophy and pseudobulbar syndrome. The electrophysiologic study showed continuous motor unit activity integrated by normal motor unit potentials. Biochemical and imaging results were normal. These data suggest the diagnosis of idiopathic progressive encephalomyelitis with rigidity. Following administration of gabapentin (2000 mg daily), muscle rigidity and electromyographic continuous motor unit activity were suppressed. Transient drowsiness was the only side effect. The authors have tried to relate these findings to those found in the literature.

Acetates↗

Attention and cognition in bradykinetic-rigid syndromes: an event-related potential study.

Bradykinetic-rigid syndromes are often accompanied by cognitive impairment. Because prominent motor involvement in these disorders may interfere with neuropsychological testing, we used event-related potentials (ERPs) for the assessment of cognition and attention in 41 patients with various bradykinetic-rigid syndromes of less than 5 years duration: idiopathic Parkinson's disease corticobasal degeneration, Steele-Richardson-Olszewski syndrome (SRO), and multiple system atrophy. Patients were compared with matched normals. ERP abnormalities in the auditory "oddball" paradigm were found only in corticobasal degeneration and SRO. ERP abnormalities in selective attention tasks were present in all patient groups, changes in SRO being the most prevalent. Abnormalities in corticobasal degeneration were present under "less-attention-demanding" conditions and suggested involvement of posterior parts of the brain. Multiple system atrophy and idiopathic Parkinson's disease patient groups had minimal ERP abnormalities. However, reaction times in MSA were longer in all paradigms. The results of the study support the view that bradykinetic-rigid syndromes involve some attentional deficits, but also have distinct reaction time and ERP characteristics, which may be helpful in differential diagnosis.

Acoustic Stimulation↗

Synergistic effect of SCH 58261, an adenosine A2A receptor antagonist, and L-DOPA on the reserpine-induced muscle rigidity in rats.

The aim of the present study was to find out whether a blockade of adenosine A2A receptors by the selective antagonist, SCH 58261, potentiates the attenuating effect of L-DOPA, the well-known antiparkinsonian drug, on parkinsonian-like muscle rigidity in rats. Muscle tone was examined using a combined mechano- and electromyographic method, which simultaneously measured muscle resistance of a rat hindfoot to passive extension and flexion in the ankle joint and the electromyographic (EMG) activity of the antagonistic muscles of that joint: gastrocnemius and tibialis anterior. Muscle rigidity was produced by reserpine (5 mg/kg ip) injected in combination with alpha-methyl-p-tyrosine (alpha-MT, 250 mg/kg ip). L-DOPA (25 mg/kg ip) or SCH 58261 (0.1 mg/kg ip) administered separately, slightly influenced the reserpine + alpha-MT-induced muscle rigidity. However, only ankle joint extension was affected significantly while the effect on flexion of the rat hindfoot was not significant. Neither L-DOPA nor SCH 58261 given separately modified the reserpine-enhanced tonic or reflex EMG activities in both muscles examined. However, when L-DOPA (25 mg/kg) was given together with SCH 58261 (0.1 mg/kg), a clear synergistic effect was seen on both examined movements and muscles. The present results show that the blockade of adenosine A2A receptors potentiates the antiparkinsonian effect of L-DOPA. Since such an effect was seen in different animal models of Parkinson's disease (PD), it seems that co-administration of SCH 58261 may allow for the lowering of the doses of L-DOPA in clinical practice, which indicates a potential therapeutic value of this compound in the treatment of PD.

Adenosine A2 Receptor Antagonists↗

Rigid spine syndrome. Report of 4 cases.

Four cases of rigid spine syndrome are reported for the first time in China. They are 2 males and 2 females. One girl has a positive family history. Limitation of flexion of the cervical and dorsolumbar spine, contractures of joints, especially the elbow joints, extreme flattening of the chest and scoliosis are the prominent symptoms of this syndrome. The clinical criteria for this rigid spine syndrome tally with those described by Dubowitz (1965). Symptomatology, diagnosis, differential diagnosis and genetics are discussed. However, the question whether rigid spine syndrome is a single, well defined disease entity remains to be explored.

Adolescent↗

[The relationship between rigidity and strength in the skinned fiber of rabbit skeletal muscle].

In single chemically skinned fibers of the rabbit skeletal muscle in the state of rigour and the calcium activation, the interrelationship between the strength and rigidity, in change of the sarcomeres length from 2.4 to 3.0 microns, of free calcium concentration (pCa = 6.75 +/- 5.5) and of interfilament distance, was studied. The strength-rigidity ratio under these conditions was found to be near to 1. The change in the sarcomeres length was also shown to affect the interrelationship pCa--strength, pCa--rigidity on account of the growth of the cooperation coefficient in the Hill equation.

Animals↗

[Amplitude of the tension developed by glycerinated muscle fibers during rigidity depends on the nature of the structural reorganizations in F-actin induced by formation of the actomyosin complex].

Dependence between the amplitude of tension, developed by glycerinated muscle fibers during rigidity, and the character of structural changes in F-actin, induced by the formation of actomyosin complex, was studied by polarized microfluorimetry and tensiometry. It is shown that during rigidity the anisotropy of intrinsic tryptophan residues as well as of rhodamine phalloidin bound to F-actin, and amplitude of tension depend on pH (6-8) and ionic strength (mu = 0.07 M-0.14 M) of solution. Greater changes in polarized fluorescence and in amplitude of tension were registered during rigidity in solutions with low ionic strength (mu = 0.07 M) and pH 8. It suggested that the amplitude of muscle fibre tension depends on the relative quantity of actin monomers, being in the "switched on" state.

Actins↗

Bilateral intracerebral haemorrhage presenting with supranuclear ophthalmoplegia, bradykinesia and rigidity.

A 70 year old man recovered from a left putamenal haemorrhage in 1984 with a residual mild right hemiparesis. In 1985 he presented following the sudden onset of inability to stand. The clinical findings of supranuclear ophthalmoplegia, bradykinesia and rigidity resembled those of progressive supranuclear palsy. CT scan revealed a recent haemorrhage deep in the right hemisphere white matter in addition to a low density change in the left basal ganglia reflecting the site of previous haemorrhage. The patient's course was uncomplicated and the ophthalmoplegia resolved. Bradykinesia and rigidity persist. This case illustrates an unusual clinical presentation of bilateral intracerebral haemorrhage with supranuclear ophthalmoplegia, bradykinesia and rigidity.

Aged↗

[Rigid spine syndrome and its nosological borders. 2 cases].

The rigid spine syndrome is characterized clinically by rigidity due to deficient extensor muscles of the spine (chiefly cervical) and radiologically, by a normal spinal image. Electromyography demonstrates abnormalities suggestive of a myogenic process, and histology shows alterations of muscular fibres. From the case-records of two male patients aged 14 and 15 years respectively, a relationship could be established between various diseases having in common a rigid spine syndrome. These include congenital muscular dystrophias , sequelae of arthrogryposis, Emery - Dreifuss myopathy and myosclerosis .

Adolescent↗

Fentanyl-O2-N2O rigidity and pulmonary compliance.

Induction of anesthesia with fentanyl has been associated with such extreme rigidity as to cause failure of bag-and-mask ventilation. The etiology of this failure has not been investigated. Therefore, fentanyl-induced rigidity was studied in 5 control patients to establish the dose at which ventilation became impossible and in 5 patients with tracheostomies to investigate changes in pulmonary compliance. Anesthesia was induced with 3 micrograms/kg/min fentanyl to a total of 30 micrograms/kg. In control patients, failure to maintain bag-and-mask ventilation occurred at a mean fentanyl dose of 17 micrograms/kg. In patients with tracheostomies, compliance decreased 16% below baseline levels with fentanyl-100% O2 and an additional 22% with 60% N2O, returning to baseline levels after paralysis. It is concluded that the site of the cause of inability to maintain bag-and-mask ventilation after fentanyl lies above the trachea, specifically at the glottis. Glottic rigidity causes glottic closure and upper airway obstruction.

Adult↗

[Parkinsonian tremor, rigidity and flapping tremor].

Tremor occurring at rest (4-6 Hz) is considered to be one of the most characteristic symptoms in patients with Parkinson's disease. Yet, an early stages of the disease only 50% of the patients manifest resting tremor. Indeed, about 15% of Parkinsonian patients never display tremor at any stage of the disease. If tremor is present, its type is essential for differential diagnosis. Pill-rolling movement, for instance, is a hallmark of Parkinson's disease. Parkinsonian patients often show postural tremor associated with certain postures or movement, whereas flapping tremor is indicative of metabolic (ex: hepatic) encephalopathy. Parkinsonian rigidity is unique muscular rigidity. If rigidity is abolished by L-dopa or dopamine agonists, this provides a reliable sign for diagnosis of Parkinson's disease. Pathophysiology of different types of tremor is briefly discussed.

Adult↗

[A study on truncal rigidity in parkinsonism--evaluation of diagnostic test and electrophysiological study].

Truncal rigidity is regarded as a cause of axial motor disturbances such as difficulty in turning around and in rising from a recumbent position in parkinsonism. However, methodology for clinical evaluation of truncal rigidity has not yet been established. This study aims at developing a suitable bed-side technique to evaluate truncal rigidity in parkinsonism. Forty normal adults and fifty-one patients with Parkinson's disease or juvenile parkinsonism were examined. We tried to evaluate truncal tonus by twisting the subjects' body axis in a supine position. The subjects' axis was passively rotated by the examiner supporting and twisting the subjects' (1) shoulder, (2) axillas, (3) hips, and (4) knees. In comparing these four procedures, the maximal rotation of the axis was obtained easily with twists supporting the knees, that is alternating tilts of the bending knees rightward and leftward in a supine position. By this method of alternating knee tilts, the examiner felt merely a little resistance in twisting the normal subjects. On the other hand, in patients with parkinsonism, a considerable degree of resistance against twists was elicited, and the patients' side-was passively elevated from a bed in accordance with knee tilts. Electrical activities of the truncal muscles (M. erector spinae, M. obliquus abdominis externus and internus) during the alternating knee tilts were studied in ten normal adults and seventeen parkinsonian patients. Continuous electrical activities were present in the axial muscles, particularly at the thoracic level, which were passively stretched by this procedure in parkinsonism but never present in normals.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Clinical and magnetic resonance features of the classic and akinetic-rigid variants of Huntington's disease.

We studied 32 patients with confirmed Huntington's disease (HD); six (mean age, 31.7 years) had the akinetic-rigid form and 26 (mean age, 46.1 years) had the classic hyperkinetic form. Clinical examination included a count of abnormal involuntary movements, motor self-sufficiency evaluation by the Physical Disability Rating Scale, cognitive function assessment by the Mini-Mental State examination, and a verbal fluency test. Magnetic resonance imaging permitted measurement of bicaudate diameter, a sensitive indicator of caudate atrophy in HD. Patients with the akinetic-rigid form of HD were younger and had earlier disease onset than those with the classic form of HD. All patients with akinetic-rigid HD (group 1) had striatal hyperintensity on T2-weighted magnetic resonance images; seven patients with classic HD (group 2) had a similar abnormality. Groups 1 and 2 were in fact similar in all other respects, except that the number of abnormal involuntary movements was greater in group 2. Groups 1 and 2 together had significantly younger age at onset, lower Mini-Mental State Examination score, more severe motor disability, worse verbal fluency test result, and greater bicaudate diameter than the 19 patients with classic HD without magnetic resonance signal abnormality (group 3) and appear to be a uniform population, distinct from group 3. The abnormalities on magnetic resonance images indicated greater striatal damage in groups 1 and 2, which could be the neuroanatomic substrate of their greater motor and cognitive compromise.

Adult↗

Rigid tracheobronchoscopy-induced bacteremia in the pediatric population.

OBJECTIVE: To assess the incidence of bacteremia following rigid tracheobronchoscopy in children to determine whether use of prophylactic antibiotics is warranted in pediatric patients at risk for perioperative endocarditis. DESIGN: Prospective nonrandomized clinical study. SETTING: Specialty care referral center. PATIENTS: Patients younger than 18 years undergoing diagnostic rigid tracheobronchoscopy for airway assessment. Twenty-five patients (14 boys and 11 girls) were enrolled. The mean age was 5.2 years (range, 10 months to 13 years). INTERVENTIONS: Blood samples for culture were obtained intraoperatively at 2 time intervals. The first culture was obtained after the induction of mask anesthesia prior to airway instrumentation; the second, within 5 minutes following the completion of tracheobronchoscopy. Blood cultures were performed under sterile technique and were placed into 20 mL of brain heart infusion broth. All cultures were incubated at 35 degrees C and observed for growth over a 14-day period. RESULTS: There were no documented cases of bacterial growth in blood cultures. All blood cultures, obtained before and after tracheobronchoscopy, were negative for bacterial growth after incubation for 14 days. Two culture bottles yielded contaminant organisms. CONCLUSIONS: Rigid tracheobronchoscopy in the pediatric population is a low-risk procedure for the development of bacteremia. This may bear on present guidelines regarding perioperative antibiotic prophylaxis for endocarditis in the high-risk population.

Adolescent↗