Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty five patients with motor neuron disease completed a double blind randomised cross over trial of RX77368, a stabilised TRH analogue, iv over 2 hours against saline. Temporary improvement in bulbar symptoms including speech, respiratory parameters, tongue movements and swallowing were seen. Fasciculations increased and spasticity decreased. Change in muscle force with drug was different from placebo but both increase and decrease in force were seen and did not result in detectable changes in function. Side effects were clinically significant in 50% of the patients and cleared within 12 hours. Prolonged rise of thyroxine and an increase in plasma levels of prolactin, thyroid stimulating hormone and growth hormone were seen and followed characteristic patterns.
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PMID:Controlled acute trial of a thyrotrophin releasing hormone analogue (RX77368) in motor neuron disease. 311 76

Sixteen patients with motor neuron disease received RX77368, a TRH analogue, IV, repeatedly over 1-12 weeks (median 2 weeks). Slight to moderate improvement in bulbar function, particularly speech, was reproduced or persisted with repeated infusions in 8 of 12 responders over a median of 18 days (range 14-90) during the period of study. Cramps (5/9) and spasticity (5/8) improved for a median of 14 days (range 7-35) and 7 days (range 2-14) respectively. The highest benefit/side effect ratio was seen with 0.2 mg/kg (0.15 mg/kg in those with severe bulbar palsy) every 3-4 days. Long term studies with this analogue in MND are indicated.
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PMID:Subacute administration of a TRH analogue (RX77368) in motorneuron disease: an open study. 314 18

We studied three patients from two unrelated families with adult hexosaminidase A deficiency. A 30-year-old, non-Jewish proband in the first family had juvenile amyotrophic lateral sclerosis that evolved to mild dementia, ataxia, and axonal (neuronal) motor-sensory peripheral neuropathy. A 36-year-old Jewish proband in the second family had "pure" spinal muscular atrophy. One supposedly healthy brother of the first proband was found to have borderline IQ, mild spasticity, and ataxia but no evidence of motor neuron disease. Marked cerebellar atrophy was detected by head scans in all three patients. In both probands electromyograms were characterized by prominent, complex repetitive discharges in many muscles. Hexosaminidase A activities against the artificial substrate were similar to those reported in infantile Tay-Sachs disease; however, the hexosaminidase A level against GM2 substrates was higher than that found in infantile Tay-Sachs disease. The hexosaminidase A levels of the parents were in the heterozygous range. Motor neuron disease in our patients and in those previously described appears to be part of a multisystem degeneration of the nervous system.
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PMID:Motor neuron disease and adult hexosaminidase A deficiency in two families: evidence for multisystem degeneration. 315 34

During a train of 200 stimuli, F-response frequency, frequency of identical responses, and F-response shape were studied in the ulnar nerve of 17 patients with motor neuron disease (MND) and 16 patients with cervical spondylosis (CS). In MND patients, F-response frequency varied between 5% and 96% with a median of 39% which was significantly lower than controls (p less than 0.001), and showed a significant rank correlation with the M-response amplitude (r = 0.62, p less than 0.004). Identical responses occurred more frequently than in controls (median 30.8%, p less than 0.001). F-response frequency was normal in CS patients (median 76.3%, range 35% to 97%), but the frequency of identical responses (median 6.3%) was higher than normal (p less than 0.01). Reduced F-response frequency in MND was thought to reflect loss of lower motor neurons, while the presence of spasticity was probably the major factor underlying the increased frequency of identical responses in both disorders. F-response shape tended to be simpler in MND and rather more complex in CS patients than controls.
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PMID:F-response frequency in motor neuron disease and cervical spondylosis. 358 84

An attempt has been made to identify and manage patients symptomatic from both cervical and lumbar spinal stenosis. The order of operative intervention was related to the degree of myelopathy and radiculopathy. Patients requiring cervical surgery first had absolute stenosis with a spinal canal equal to or less than 10 mm in anteroposterior diameter. Those requiring lumbar surgery first presented with stenosis and a canal between 11 and 13 mm in depth. In the latter group, patients presented with radiculopathy in their upper and lower extremities. A significant portion (50%) had intermittent neurogenic claudication (INC). Motor and sensory changes were severe in those with absolute as compared to relative stenosis. After cervical laminectomy, myelopathy improved or stabilized, and the subsequent lumbar decompression could be completed with less risk. Cervical cord decompression often resulted in improvement in lumbar symptoms with resolution of pain, spasticity, and sensory deficits of myelopathic origin. However, latent symptoms of INC caused by lumbar stenosis were not affected by cervial decompression and increased in severity. Electrodiagnostic studies were helpful in that somatosensory evoked potentials showed conduction delays in the cervical cord in patients with significant disease. The identification of motor neuron disease and peripheral neuropathies was essential. The surgical management included extensive, multiple level laminectomy, unroofing of the lateral recesses, and foraminotomy. Neurolysis and untethering of the spinal cord was essential. Significant improvement was shown by 90% of these patients.
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PMID:Coexisting cervical and lumbar spinal stenosis: diagnosis and management. 649 58

The recurrent inhibition of alpha-motoneurons by Renshaw cells, brought about by a conditioning reflex discharge, was estimated by measuring the amplitude of a test reflex which involved only those soleus motoneurons which had already fired in response to the conditioning volley. The results obtained in 95 patients with upper motor neuron disease and in 31 normal subjects were compared. At rest, there was no evidence for a decrease in the excitability of Renshaw cells: on the contrary, recurrent inhibition, if it changed at all, was increased in 55 out of 95 patients. In most patients the changes in Renshaw cells excitability which occur during postural or voluntary contractions in normal subjects were not found. We conclude that changes in recurrent inhibition are not responsible for the hyperexcitability of the stretch reflex which characterizes human spasticity. On the other hand, the paralysis of the supraspinal control of Renshaw cells, which normally accompanies voluntary movements, could account in part for two difficulties exhibited by spastic patients: in grading the strength of a muscular contraction, and in regulating reciprocal Ia inhibition according to the requirements of a voluntary movement.
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PMID:Recurrent inhibition of alpha-motoneurons in patients with upper motor neuron lesions. 706 69

In this paper, the autopsy findings of a 78-year-old man mimicking primary lateral sclerosis (PLS) are reported. His clinical symptoms were slowly progressive spasticity, pseudobulbar palsy and character change. He died of sepsis 32 months after protracting the disease. The autopsy revealed severe atrophy of the frontal and temporal lobes. The histological findings were severe neuronal loss with gliosis in the precentral gyrus and left temporal lobe tip, loss of Betz cell, prominent demyelination throughout of the corticospinal tract, axonal swelling in the cerebral peduncule, severe degeneration of the amygdala, mild degeneration of the Ammon horn, normal substantia nigra, a few neuronal cells with central chromatolysis in the facial nerve nucleus and very mild neuronal cell loss in the spinal anterior horn. The anterior horn cell only occasionally demonstrated Bunina body by H & E and cystatin-C stainings, as well as, skein-like inclusion by ubiquitin staining. Thus, this is a case of uncommon amyotrophic lateral sclerosis (ALS) dominantly affecting the upper motor neuron including the motor cortex and temporal limbic system. In analysis of nine cases of putative primary lateral sclerosis in the literature, six cases showed loss of Betz cell in the precentral gyrus, and four cases very mild involvement of the lower motor neuron such as central chromatolysis and eosinophilic inclusion body. Degeneration of the limbic system was observed in two cases. We indicated a possible subgroup with concomitant involvement in the motor cortex and temporal lobe in motor neuron disease dominantly affecting the upper motor neuron.
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PMID:[An autopsied case of dominantly affecting upper motor neuron with atrophy of the frontal and temporal lobes--with special reference to primary lateral sclerosis]. 761 64

This study investigated the potential value of eccentric (ECC) and concentric (CONC) isokinetic testing for quantifying motor deficit in patients with spastic paresis secondary to motor neuron disease. We hypothesized that, at a moderately fast (120 degrees s-1) angular velocity, spastic patients would demonstrate different ECC-CONC torque relationships from healthy controls or patients with non-spastic neuromuscular disorders. Eleven patients with motor neuron disease having clinical evidence of spasticity, and 11 disease-control patients (with non-spastic disorders, e.g. lower motor neuron disease or myopathy) underwent isokinetic testing. One healthy subject was matched to each of the 22 patients. The average torque generated during maximal voluntary ECC and CONC knee flexion (KF) and extension (KE) was measured using an isokinetic dynamometer (Kin-Com). Reliability was established (all ICC > or = 0.97) for patient torque measurements. Relative strength (% of control subject torque) in spastic patients was significantly higher for ECC than for CONC actions in both KF and KE; conversely, in non-spastic disease-control patients relative strength was not affected by the type of muscle action. The ECC/CONC average torque ratios for KE and KF at 120 degrees s-1 were significantly greater in spastic patients than controls, but did not differ from controls in non-spastic patients. In spastic patients the ECC-CONC imbalances were related to ambulatory dysfunction. In four spastic patients followed with serial testing, the disproportion between ECC and CONC voluntary capacity persisted over time.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Eccentric and concentric muscle performance in patients with spastic paresis secondary to motor neuron disease. A preliminary report. 801 93

A spring-loaded device that "breaks" at preset forces was used to assess readings obtained by hand-held dynamometry by three raters with varying experience in the method. Overall accuracy (3%), but not reproducibility or variability, was improved by greater experience. Readings obtained jointly by three raters had 53% greater variability than those obtained by a single rater. Nine muscle groups in 19 patients with motor neuron disease were assessed at 10 sessions (three replications per session) over six days by the experienced rater. Muscle force was expressed relative to that of 22 matched normal controls. The reproducibility was good with a mean % difference of 13.2 and repeatability coefficient of 2.17 kg-force for readings six days apart; the overall correlation coefficient was 0.98. The mean coefficient of variation (CV) of 10 readings was 9.9%. The poorer reproducibility and greater variability seen in clinically weaker muscles may account for differences in patients with bulbar palsy and classical amyotrophic lateral sclerosis; the degree of spasticity had no effect. The rater was estimated to contribute 37% of the total variability when testing patients. The use of a composite score by combining normalised dynamometry readings of eight limb muscles improved mean % difference to 6.7 and mean CV to 5.8%. The reproducibility and variability of hand-held dynamometry readings obtained by a single rater compare well with those of fixed devices. Readings from single raters, irrespective of experience, have similar reproducibility and variability. If, however, multiple raters are used in longitudinal assessments of individual patients, as occurs in clinical trials, the variability of their combined readings should be estimated when calculating the same size required.
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PMID:Accuracy, reproducibility, and variability of hand-held dynamometry in motor neuron disease. 815 81

Amyotrophic lateral sclerosis is an idiopathic, ultimately fatal disease, clinically manifest as progressive weakness and spasticity, associated with the loss of motoneurons. Circumstantial evidence supports a role for autoimmune processes in the progression of this human disorder. Two immune-mediated animal models have been developed in our laboratory for motor neuron loss. Experimental autoimmune motor neuron disease is a lower motor syndrome induced in guinea pigs by the repeated injection of a purified bovine spinal motor neuron antigen. Affected animals demonstrate extremity weakness, associated with electromyographic and morphologic evidence of denervation, a loss of spinal cord motor neurons, high antibody titers against motor neurons, and localization of IgG immunoreactivity to the neuromuscular junction and motor neuron cytoplasm. Experimental autoimmune grey matter disease is a more acute and severe disorder involving both upper and lower motor neurons, induced in guinea pigs by inoculation of a bovine ventral spinal cord homogenate, in which scattered foci of denervation are observed in the motor cortex and ventral spinal cord. Similarities between these diseases and human ALS are reviewed.
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PMID:Experimental immune-mediated motor neuron diseases: models for human ALS. 845 86


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