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Rigidity decreases resting tremor intensity in Parkinson's disease: A [(123)I]beta-CIT SPECT study in early, nonmedicated patients.

Tremor is one of the clinical hallmarks of Parkinson's disease (PD). Although it is accepted that other classic symptoms of PD such as rigidity and bradykinesia result from a degeneration of the nigrostriatal system and subsequent reduction in striatal dopamine, the pathophysiology of resting tremor remains unclear. The majority of recent single photon emission computed tomography (SPECT) and positron emission tomography (PET) studies, using various radioligands, demonstrated significant correlation between striatal radioligand bindings and the degree of parkinsonian symptoms such as rigidity and bradykinesia, but not tremor. We investigate the relationship between the degeneration of the nigrostriatal pathway and the appearance of resting tremor, taking into account the possible interference of rigidity with the resting tremor. Thirty early and drug-naïve PD patients were examined. Tremor and rigidity of the arms were assessed using UPDRS, and the power of tremor was estimated using spectral analysis of tremor peaks. [(123)I]beta-CIT SPECT was used to assess degeneration of the dopaminergic system in PD patients. A comparison between asymmetry indices showed that in terms of both tremor and rigidity, the most affected arm corresponded significantly with the contralateral striatum, having the largest reduction in radioligand binding. Furthermore, tremor power accounted for a significant part of variance in the contralateral striatum, suggesting a relationship between this PD symptom and the degeneration of the dopaminergic system. Further, the degree of tremor was reduced with increasing rigidity. However, correcting for the influence of rigidity, the significant contribution of tremor in the variance in the contralateral striatal [(123)I]beta-CIT binding disappeared. When the confounding influence of rigidity is taken into account, no significant direct relationship between dopaminergic degeneration and the degree of tremor could be found. Other pathophysiological mechanisms should be similarly investigated in order to further our understanding of the origin of resting tremor in PD.

Adult↗

Neuroleptic malignant syndrome complicated by disseminated intravascular coagulation.

The neuroleptic malignant syndrome (NMS), clinically manifested by hyperpyrexia, mental status changes, muscular rigidity, and autonomic dysfunction, is an idiosyncratic reaction to major neuroleptic pharmacologic agents. The reported causes of morbidity and mortality include respiratory insufficiency, cardiovascular collapse, arrhythmias, and renal failure. Coagulopathy does not seem to be a prominent component of the syndrome. We observed a case of NMS in which disseminated intravascular coagulation was a prominent feature.

Adult↗

Niacin improved rigidity and bradykinesia in a Parkinson's disease patient but also caused unacceptable nightmares and skin rash--a case report.

A patient with Parkinson's disease taking levodopa/carbidopa, selegiline, buproprion, aspirin and niacin had decreased rigidity and bradykinesia when his niacin dose was steadily escalated for treatment of high triglycerides, but ultimately the patient could not tolerate niacin because of severe nightmares and skin rash. If further research can reproduce this patient's initial beneficial experience while avoiding the adverse effects, niacin could be a useful adjunctive agent for Parkinson's disease, either population-wide or in a pharmacogenomically defined set of responders.

Aged↗

Problems in intramedullary nailing of femoral fractures.

Intramedullary nailing, an improved method for treatment of fractures of the shaft of the femur, is still in a transitional stage. Experience in 35 cases indicates that supplementary fixation as safeguard against sepparation or overriding often is advisable, particularly in the presence of considerable fragmentation. Wire loops, cross screws and, in some cases, a Collins band modified in such a way as to avoid occlusion of blood supply, have been used for this purpose. For strength and rigidity, the rod used should be as great in diameter as the medullary space can accommodate. A method of preoperative determination of the size of rod to use has been devised. As the intramedullary rod may bend or break, it is advisable that patients be cautioned against full weight bearing on the injured leg too soon after operation.

Dental Occlusion↗

Randomized trial of pallidotomy versus medical therapy for Parkinson's disease.

Thirty-six patients with Parkinson's disease (PD) were randomized to either medical therapy (N = 18) or unilateral GPi pallidotomy (N = 18). The primary outcome variable was the change in total Unified Parkinson's Disease Rating Scale (UPDRS) score at 6 months. Secondary outcome variables included subscores and individual parkinsonian symptoms as determined from the UPDRS. At the six month follow-up, patients receiving pallidotomy had a statistically significant reduction (32% decrease) in the total UPDRS score compared to those randomized to medical therapy (5% increase). Following surgery, patients' showed improvement in all the cardinal motor signs of PD including tremor, rigidity, bradykinesia, gait and balance. Drug-induced dyskinesias were also markedly improved. Although the greatest improvement occurred on the side contralateral to the lesion, significant ipsilateral improvement was also observed for bradykinesia, rigidity and drug-induced dyskinesias. A total of twenty patients have been followed for 2 years to assess the effect of time on clinical outcome. These patients have shown sustained improvement in the total UPDRS (p < 0.0001), "off" motor (p < 0.0001) and complications of therapy subscores (p < 0.0001). Sustained improvement was also seen for tremor, rigidity, bradykinesia, percent on time and drug-induced dyskinesias.

Antiparkinson Agents↗

Total biopterin levels in the ventricular CSF of patients with Parkinson's disease: a comparison between akineto-rigid and tremor types.

Total L-erythro-biopterin (T-BP) levels in the ventricular cerebrospinal fluid (CSF) were measured in 43 patients with Parkinson's disease (PD) and 12 age-matched neurological controls. In 5 of the PD patients and 1 control, lumbar CSF T-BP values were also measured. The mean ventricular CSF T-BP level in the PD patients, 15.6 +/- 0.5 pmol/ml (mean +/- SE), was significantly lower than that in the controls (21.3 +/- 1.4 pmol/ml, P less than 0.0001). The mean T-BP concentration in the ventricular CSF was 1.9 times higher than that in the lumbar CSF (P less than 0.0005), indicating a rostrocaudal gradient for the T-BP value in the CSF. When the PD patients were classified according to their predominant clinical features into 24 akineto-rigid (A-R) type and 19 tremor (T) type, there was a significant negative correlation between the T-BP levels and duration of illness only for the A-R type patients (rho = -0.605, P less than 0.005). No such significant correlation was found in the T type patients. These results may indicate a difference of pathophysiological changes in the brain between the 2 types of PD.

Biopterins↗

The optimal stimulation site for chronic stimulation of the subthalamic nucleus in Parkinson's disease.

To determine the optimal stimulation site within the subthalamic nucleus (STN), monopolar stimulation of four electrode contacts and the resulting effects on parkinsonian symptoms were evaluated in 10 consecutive patients. The UPDRS score for rigidity and akinesia improved significantly after stimulation at each of the contacts, compared to the pre-evaluation state (Fisher's test, p < 0.05). The most significant improvement was obtained after stimulation at contact-2 (rigidity: 74.4 +/- 20.4%, akinesia: 53.7 +/- 14.3%) (Fisher's test, p < 0.001). Contact-2 was located at the dorsal border of the STN at a mean distance of 0.3 +/- 0.7 mm. DBS at the dorsal border of the STN, where the stimulation affects the neurons as well as their axonal fibers, produces the greatest clinical improvement in parkinsonian symptoms.

Brain Mapping↗

[Alfentanil in routine clinical use. A study of 50 patients].

Alfentanil in combination with etomidate and N2O/O2 was given to 50 patients as single dosage (0.024 mg/kg b.w.) or with repeated injections for surgical interventions up to 90 minutes duration. In 68% of these cases sufficient analgesia was obtained. The most frequent side-effects were rigidity of the thorax (54%), quick, extensive changes in blood pressure (32%) and bradycardia (28%). The recovery phase was very short, postoperative sickness and vomiting were seen in 6% of all cases. Still, after repeated injections phases with prolonged sleep can appear. While using Alfentanil, exact monitoring is necessary, as quick and unexpected changes of blood pressure and pulse can appear.

Adolescent↗

Haemodynamic, metabolic and physical responses to a neuroleptanalgesic-glyceryl guaiacolate combination in the horse.

A commercial neuroleptanalgesic acepromazine-etorphine combination administered intramuscularly to four horses produced a severe tachycardia and an increase in muscular tone, together with hypoxaemia, hypercapnia, metabolic acidosis associated with an increase in the packed cell volume and hyperglycaemia. No electrolyte changes were found. After reversal of the action of etorphine with diprenorphine, there was a prolonged decrease in the calcium and phosphorus serum concentrations and decreases in the packed cell volume and the total protein serum concentration. In a second experiment on the same four horses, glyceryl guaiacolate (10 g/100 kg body weight intravenously) was given as soon as the horses were anaesthetized with acepromazine-etorphine. The muscular rigidity disappeared and the tachycardia was less evident. There was a more pronounced hypoxaemia but the changes in the other parameters were similar to those in the first experiment. It was concluded that the neuroleptanalgesic-glyceryl guaiacolate combination is not a safe anaesthetic procedure in horses.

Acepromazine↗

Movement disorders in childhood organic acidurias. Clinical, neuroimaging, and biochemical correlations.

Over the last 5 years the Pediatric Neurology service at King Faisal Specialist Hospital and Research Centre (KFSH&RC) has seen 131 infants and children with movement disorders. Forty-nine (37%) had identifiable biochemical defects, 25 of which were organic acidemias. Nineteen of 29 patients with dystonia had organic acidemias, primarily glutaric aciduria type 1 (7 patients), bilateral striatal necrosis (4 patients), and 3-methyl glutaconic aciduria (3 patients). All patients with parkinsonian rigidity (n = 11) had organic acidemias; again, the greatest number accounted for by glutaric aciduria type 1 (7 patients), who had both parkinsonian rigidity combined with dystonia. Myoclonus occurred in only 1 of 25 and chorea in 7 of 25 patients with organic acidemias. At the least all patients had bilateral lesions of putamen and head of caudate, seen best in MRI brain scans as increased T2 signal intensities with normal volume, and later with volume loss.

Acidosis↗

A case of progressive encephalomyelitis with rigidity and positive antiglutamic acid decarboxylase antibodies [corrected].

A 50 year old woman developed progressive encephalomyelitis with rigidity over a three year period. Her CSF contained oligoclonal bands and both her serum and CSF contained antibodies directed against GABA-ergic synapses (antiglutamic acid decarboxylase [corrected] antibodies). These antibodies have recently been described in cases of stiff man syndrome. Both disorders may be part of a clinical spectrum that has an underlying autoimmune basis.

Autoantibodies↗

Judgment of the visual vertical and horizontal in patients with Parkinsonism.

Judgment of the visual vertical and horizontal in the upright body position was abnormal in 19 (29 percent) of 66 patients with Parkinson's disease. The magnitude of errors correlated with the degree of rigidity and of tremor in the limbs, but not with bradykinesia or other clinical features. The results suggest that the effect on visual perception of the vertical and horizontal coordinates in patients with parkinsonism is brought about by specific changes in the basal ganglia and is not, as in the case of other visual-motor tasks, determined merely by the overall severity of cerebral pathology.

Adult↗

[Serotonin syndrome caused by tandospirone citrate alone].

We describe a case of serotonin syndrome provoked by tandospirone citrate alone, a serotonergic antianxiety agent. A 42-year-old alcoholic woman started to take tandospirone (30 mg/day in 3 divided doses) because of psychosomatic complaints. One month later, dellirium, marked rigidity and myoclonus in the neck and extremities, fever, hyperhidrosis and diarrhoea developed in succession. Tandospirone was discontinued, and all symptoms disappeared within the following 6 days. We diagnosed her condition as serotonin syndrome, although alcoholic withdrawal syndrome might have superimposed. Serotonin syndrome caused by tandospirone alone has not been reported, although it is widely used in Japan.

Adult↗

[Familial parkinsonism: different clinical phenotype between a mother and her daughter].

We reported a familial case of parkinsonism in which clinical features are different between a mother and her daughter. The mother developed rigidity and bradykinesia at age 76. Her symptoms were slowly progressive, and were responsive to levodopa. Thus, she was diagnosed as having Parkinson's disease. In contrast, her daughter developed postural instability as well as rigidity and bradykinesia at age 53 in rapid progression. In addition, she also developed pyramidal signs and autonomic failures, including orthostatic hypotension and urinary incontinence. The disease phenotype of the daughter resembled that of striatonigral degeneration (SND). However, PET studies showed normal binding of 11C-N-methyl-spiperone (NMSP) in the striatum, suggesting that the daughter was also affected with Parkinson's disease.

Aged↗

Riluzole prevents MPTP-induced parkinsonism in the rhesus monkey: a pilot study.

Previous studies have shown that riluzole (2-amino-6-trifluoromethoxy-benzothiazole), a drug which interferes with glutamate neurotransmission, has a neuroprotective action in rodent models of global and focal cerebral ischemia. In this pilot study, the protective and palliative effects of riluzole have been examined using an animal model of Parkinson's disease. Two monkeys were rendered hemiparkinsonian by one intracarotid injection of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), and motor signs were evaluated using clinical examination and electromyographic recordings. When riluzole (4 mg/kg) was administered before the injection of MPTP, parkinsonian motor symptoms, in particular bradykinesia and rigidity, were absent. When injected daily in one monkey which presented stable motor symptoms, bradykinesia and rigidity were significantly reduce d. Riluzole pretreatment induced a persistent increase in dopamine turnover when compared to MPTP alone. Thus, a possible neuroprotection and a facilitation of dopamine release may explain the behavioural effects reported with riluzole treatment. These preliminary results suggest that riluzole could possess neuroprotective and palliative effects in a primate model of Parkinson's disease.

Animals↗

Pramipexole ameliorates neurologic and psychiatric symptoms in a Westphal variant of Huntington's disease.

Levodopa is recommended as a therapeutic approach for patients with the hypokinetic-rigid, Westphal variant of Huntington's disease, but no guidelines are available for the case of nonrespondence to levodopa. In this study, however, we report a 34-year-old woman with this rare variant who showed a clear-cut improvement in motor function and depressive symptoms after treatment with pramipexole, a new dopamine agonist.

Adult↗

The neuroleptic malignant syndrome. A priority system.

The neuroleptic malignant syndrome (NMS) of hyperthermia, rigidity, hyperautonomicity and altered consciousness results from the use of neuroleptic agents. Although rare, it is important to recognize and treat the syndrome early because it is potentially lethal. It bears striking similarities to several other conditions, particularly malignant hyperthermia. NMS probably reflects a disorder of the hypothalamic and basal ganglia dopamine systems. No specific treatments are available, but dantrolene sodium, bromocriptine, amantadine and phenytoin have been used successfully.

Amantadine↗

Primary brainstem injury: benign course and improved survival.

Primary brainstem injury following head injury is a rare event. The victims often have features of supratentorial injury, and a primary isolated injury to the brainstem occurring due to shearing stresses or to injury from the tentorial edge is extremely rare. In the presence of supratentorial injury, these patients may have altered sensorium. Isolated brainstem injury may manifest itself as internuclear ophthalmoplegia, anisocoria, rigidity and cerebellar tremor. Such injuries are now being diagnosed more often due to improved imaging techniques. We treated nine such cases who had sustained primary brainstem injury in road traffic accidents, all but one of whom were subsequently independent. Primary brainstem injuries need not be associated with poor prognosis and mortality and may run a benign course with good quality of survival.

Adult↗