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[Clinically diagnosed corticobasal degeneration (CBD)].

We reported a 67-year-old male, who suffered from apraxia and amnesia for 2 years and for muscle rigidity of right extremities for a year. Neurological examination revealed dysarthria, dysphagia, marked dystonia of right arm, hyperreflexia of all limbs and ataxic gait. He also had dementia and many other higher cortical dysfunction mostly due to left hemisphere damage. No impairment of eye movement was disclosed. Brain MRI as well as CT showed the significant brain atrophy in the left parieto-occipital region. A degenerative atrophy was suspected by 123I-IMP-SPECT and 18F-FDG-PET. By FDG-PET, the decrease of cerebral blood flow and glucose metabolism was detected not only affected unilateral cerebral cortex including primary motor area but ipsilateral basal ganglia and thalamus. Although, it is difficult to distinguish clinically CBD from atypical case of Alzheimer's disease, we speculated that in early stage of dementia, significant unilateral hypoperfusion and hypometabolism of basal ganglia and thalamus is characteristic of CBD.

Aged↗

[A case of abortive malignant hyperthermia during funnel chest surgery].

We experienced a case of abortive malignant hyperthermia during funnel chest surgery. Although a 5-year-old boy had muscle rigidity after the intravenous injection of succinylcholine chloride, the tracheal intubation was easy. The boy had high body temperature, metabolic acidosis, hyperkalemia and myoglobinuria during nitrous oxide-oxygen-sevoflurane anesthesia. We immediately came to the diagnosis of abortive malignant hyperthermia, gave intravenous injection of dantrolene sodium and started body surface cooling. Postoperative course was uneventful. It is necessary to pay a particular attention to possible malignant hyperthermia in patients with funnel chest surgery.

Anesthesia, General↗

Reflex behavior and programming in Parkinson's disease.

Several aspects of characteristically impaired postural adjustments indicate a defective central programming of the respective EMG patterns in PD. This may be due to deficits in the supraspinal control of spinal interneuronal circuits. This impairment obviously contributes to the difficulty of these patients in performing two motor acts simultaneously. In addition, parkinsonian patients exhibit a reduced sensitivity of polysynaptic reflexes in the leg extensor muscles which correlates with their postural instability. The activity of monosynaptic reflexes is negligible, as in healthy subjects. The impairment of proprioceptive reflex function may be partially compensated for by changes of intrinsic muscle stiffness. Discrepancies in the literature about the behavior of the polysynaptic EMG responses in parkinsonian patients may in part arise from the fact that it depends on both the particular muscle under study and the actual motor task investigated.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗

[Beneficial effect of bromocriptine in a patient with malignant syndrome and hepatic dysfunctions].

A 39 year-old male, diagnosed as meningoencephalitis, was admitted because of the development of malignant syndrome. This syndrome appeared to have resulted from anti-psychotic drugs given to relieve excitement and insomnia. As he had hepatic dysfunctions, we could not administer dantrolene further. Therefore, we gave bromocriptine to ameliorate the symptoms such as muscle rigidity or hemodynamic perturbations. Thereafter, the patient gradually became stable in hemodynamics and in other symptoms. In addition, a further deterioration in hepatic functions did not occur with administration of bromocriptine. The case suggests that in patients with malignant syndrome associated with hepatic dysfunctions, bromocriptine could be a first choice as a pharmacological treatment of the syndrome.

Adult↗

[The effect of a phorbol ester on the aortic wall].

Changes in the muscle tissue stiffness are important and necessary signs of the contractile process. The work is devoted to the study of this parameter of the smooth muscle tissue reaction during the action of phorbol esters, (namely, phorbol 12-myristate 13-acetate (PMA)), synthetic imitators of diacylglycerole. The experiments were carried out on isolated native preparations of aorta thoracic area of guinea pig under isometric conditions. Contraction induced by PMA or by the temperature was not accompanied by the stiffness changes. Potassium contraction (its development, amplitude, relaxation) evoked on the plateau of the PMA-induced contraction was similar in all respects to that in control: the stiffness increased significantly. These findings prove independent development of these two types of contractile processes. At the same time, the stiffness invariability and similar ratios of optical density to mechanical strength changes in the contractions induced by PMA action or by temperature show the likeness of the force generation mechanisms in these mechano-chemical reactions. The results obtained cannot be explained in terms of the generally accepted hypothesis of actomyosin interaction. The conclusion is made that phorbolic esters during the action on the smooth muscle tissue activate the mechanical strength generation by the connective tissue matrix.

Animals↗

[Rhabdomyolysis and acute renal failure caused by haloperidol-decanoate (neuroleptic malignant syndrome)].

A case of rhabomyolysis with attendant severe acute renal failure, arisen in a 59-year-old male treated with haloperidol-decanoate, is presented. The patient has been affected by paranoia schizophrenia since childhood, and he was treated with electroshock and successively with neuroleptics p.o. Four years before our observation, a therapy with haloperidol decanoate (50 mg i.m. monthly) was started. After some time, catatonic like episodes appeared, which got more and more frequent, until they appeared weekly. In occasion of the last of them, he was admitted to our hospital. At the objective examination he presented psychomotory arrest, perspiration, mytacism, severe muscle rigidity, moderate oedems to lower limbs. Laboratory findings showed a pattern consistent with rabdomyolysis and severe renal failure. After that haloperidol decanoate was stopped and rehydration and intensive diuretic therapy was started, the clinical and laboratory pattern went normal, persisting however a light creatinine increase. Probably the rhabdomyolysis was induced by the haloperidol decanoate, and renal failure by secondary severe hyvolemia. This case comes into the so-called neuroleptic malignant syndrome which can rarely arise in patients treated with antipsycotic agents and which causes high mortality, particularly when there are rhabdomyolysis and acute renal failure.

Acute Kidney Injury↗

[Parkinsonism in a patient receiving interferon alpha therapy for chronic hepatitis C].

We report a case of Parkinsonism due to interferon alpha (IFN alpha) therapy for chronic hepatitis C. A 51-year-old female received IFN alpha (Sumipheron@6 x 10(6) IU), three times a week by intramuscular injection. Six months after the initiation of IFN alpha therapy, she noticed tremor of bilateral fingers, and was admitted to our hospital. Neurological examination revealed muscle rigidity, bilateral finger tremor, mild bradykinesia. Tremor was more extreme at posture rather than at rest. She was diagnosed as Parkinsonism, and carbidopa-levodopa therapy was effective. Attention must be paid to Parkinsonism, when IFN alpha is administered.

Chronic Disease↗

[A case of Sjögren's syndrome with a high titer of anticardiolipin antibody that developed as parkinsonism].

A 50-year-old-male consulted us, complaining of gait disturbance. He was diagnosed as having Parkinson syndrome, but had no benefit of drugs for Parkinson disease. On admission he showed symptoms of parkinsonism such as parkinsonian gait, bradykinesia and muscle rigidity, and recurrent aseptic meningitis. Additionally, his condition was complicated by Sjögren's syndrome, based on the preliminary criteria of Sjögren's syndrome established by the European Community. Because his parkinsonism and meningitis were considered to be caused by Sjögren's syndrome, he was administered corticosteroids, which improved his parkinsonism and aseptic meningitis. The brain MR images in our case did not match those typically reported in cases of central nervous system Sjögren's syndrome (CNS-SS). Although the most common abnormalities are small distinct areas of increased signal intensity on T2-weighted images, the MR images in our case revealed periventricular diffuse areas of increased signal intensity on T2-weighted images. Although his parkinsonism and aseptic meningitis are believed to the effects of vasculitis caused by Sjögren's syndrome, it is also possible that his parkinsonism is related to Binswanger's disease resulting from a high titer of anticardiolipin antibody. Our report proposes a possible relationship between anticardiolipin antibody and CNS-SS when MR images reveal diffuse high intensity areas on T2-weighted images.

Antibodies, Anticardiolipin↗

Pathophysiology, assessment, and treatment of Parkinson's disease.

Parkinson's disease (PD) is a slowly progressing neurologic movement disorder affecting nearly 1% of the population over age 65. PD is the fourth most common neurodegenerative disease of patients. Incidence is greater in men with a ratio of 3:2--men to women. Because PD is so complex, diagnosis and treatment are often very challenging. While the cause of PD is unknown, research has concentrated on genetics, exogenous toxins, and endogenous toxins from cellular oxidative reactions. The presenting symptoms of a patient with PD include muscle rigidity, tremors, bradykinesia, and postural instability. Treatment for PD has been primarily pharmacologic: levodopa, carbidopa, anticholinergics, and selegiline (a monoamine oxidase inhibitor to reestablish an equilibrium between dopamine and acetylcholine). surgical procedures (e.g., pallidotomy, thalamotomy, and tissue implants) are a possible choice of treatment for patients who have failed to respond satisfactorily to drugs.

Antiparkinson Agents↗

Neuroleptic malignant syndrome and acute myocardial infarction: case report and review.

The neuroleptic malignant syndrome (NMS) is a potentially life threatening reaction usually observed following administration of dopaminergic antagonists (neuroleptic medications, e.g., phenothiazines, thioxanthenes, and haloperidol). NMS is characterized by mental status changes, muscle rigidity (and movement disorders such as dyskinesias and akathisias), leukocytosis, hyperthermia, and autonomic dysfunction. Because of the variants of this disease, the physician must remain alert to its possibility when confronted with emergency care of patients having received neuroleptics, particularly when hypermetabolic states are etiologically consequential in the development of other disease processes, for example, acute myocardial infarction.

Antipsychotic Agents↗

[Poisoning with a reversible and selective monoaminooxidase inhibitor].

Two cases of poisoning with moclobemide are presented. Moclobemide is a reversible inhibitor of the monoamine oxidase type A (RIMA). Intoxication with moclobemide is according to previously published case stories benign. Both patients described presented severe symptoms, such as convulsions, coma, muscle rigidity and respiratory failure. One of the patients, a 37-year-old woman, also suffered cardiovascular collapse and elevated body temperature (more than 41.9 degrees C), which was treated successfully with dantrolene and norepinephrine. The symptoms match the diagnostic criteria for serotonin syndrome. The possible reasons why the two patients suffered life-threatening complications and the treatment of serotonin syndrome are discussed.

Adult↗

[Cardiac arrhythmia associated with malignant neuroleptic syndrome: description of 2 clinical cases].

Malignant neuroleptic syndrome (alteration of consciousness, muscle rigidity and hyperthermia) is a potentially lethal condition, due also to its life-threatening complications. In particular, hypokinetic and hyperkinetic arrhythmias can be rare and severe early manifestations of this illness, and they deserve a careful approach because of their drug-refractoriness. Arrhythmias associated with the malignant neuroleptic syndrome depend on various mechanisms: neurotransmitter receptor blockades typical of neuroleptic drugs, clustered lipid droplets among the cardiac myofibrils and possible electrolytic disorder due to diaphoresis. The two cases described here presented hypokinetic and hyperkinetic (supraventricular and ventricular) arrhythmias. The arrhythmias, which failed to respond to antiarrhythmic drugs, were temporarily suppressed by DC shock, over-drive pacing and correction of electrolytic imbalance. In case 1, prolonged bromocriptine treatment was required. Complete wash-out of the causative agents resulted in lasting regression of arrhythmias. In conclusion, a correct treatment and a favourable outcome of this syndrome can be achieved only through early diagnosis.

Adult↗

Neuroleptic malignant syndrome related to use of clozapine.

A 19-year-old man presented to a community hospital with a sudden change in level of consciousness, fever, and muscle rigidity. The patient had a history of schizophrenia and was being treated with clozapine. Despite a high index of suspicion for neuroleptic malignant syndrome, definitive care was delayed for more than 24 hours after the patient was transferred to a tertiary care center. This case illustrates the importance of primary care physicians being able to recognize and diagnose this syndrome, particularly as the use of atypical antipsychotic agents increases.

Adult↗

[Evaluation of malignant hyperthermia episodes with the Clinical Grading Scale].

PURPOSE: The Clinical Grading Scale (CGS) was introduced to predict malignant hyperthermia (MH) susceptibility in adverse anaesthetic events. Because many of the clinical symptoms that occur during MH episodes are nonspecific, the CGS was designed as a tool to estimate the qualitative likelihood of MH. The purpose of this study was to compare the results of the CGS with the established in vitro contracture test (IVCT). METHODS: 92 patients with a personal history for MH were tested for MH susceptibility with the IVCT according to the protocol of the European MH Group. All patients were also evaluated with the CGS. Clinical indicators for the CGS are rigidity, muscle breakdown, respiratory acidosis, temperature increase and cardiac involvement. There are additional indicators in case of a family history for MH. For each indicator 3-15 points are added to build a raw score; this raw score corresponds to a MH rank in the CGS that describes the likelihood of MH in the suspected event. The higher the raw score rank, the higher the likelihood of MH and vice versa. RESULTS: From 92 patients, 32 (35%) were diagnosed as MH-susceptible (MHS) with the IVCT, 47 (51%) were MH-normal (MHN), and 13 (14%) were MH-equivocal (MHE). One patient with MH-rank 1 (MH almost never) in the CGS was diagnosed as MHS; on the other hand no patient with MH-rank 6 (MH almost certain) in the CGS was diagnosed as MHN. However, the majority of patients (72%) were assigned to ranks 3 and 4 (MH somewhat less than likely/MH somewhat greater than likely). The qualitative likelihood of MH could therefore not be clearly estimated. CONCLUSION: Our study shows that the MH-rank of the CGS corresponds poorly with the results of the IVCT. In any case the evaluation of an MH suspicious event depends on the availability of data of that event. It is often difficult to obtain sufficient data, especially if the event occurred a long while ago. In these cases the MH rank may underestimate the likelihood of MH susceptibility. On the other hand, overestimation is also possible because some of the scoring indicators depend on the anaesthesiologist's judgement only. At present, the use of the CGS is neither validated nor clinically feasible. The CGS cannot replace IVCT.

Adolescent↗