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Ventricular somatostatin-like immunoreactivity in patients with basal ganglia disease.

The concentrations of somatostatin-like immunoreactivity (SLI) in lateral ventricular fluid of patients with extrapyramidal motor disease were determined by specific radio-immunoassay. Mean SLI levels were significantly lower in patients with Parkinson's disease (mean +/- SEM); 42.9 +/- 2.9 fmol/ml) and in patients with dystonic syndromes (39.4 +/- 3.2) than in patients with benign essential tremor (65.3 +/- 9.7). The lowest levels were found in patients with athetosis (34.7 +/- 5.4). In parkinsonian patients somatostatin levels correlated with the degree of akinesia, rigidity and autonomic disturbances.

Adult↗

Fentanyl-induced rigidity during emergence from general anesthesia potentiated by venlafexine.

PURPOSE: To present and discuss a case of opioid-induced rigidity with low-dose fentanyl during recovery from anesthesia. CLINICAL FEATURES: A 41-yr-old woman underwent laparotomy for total abdominal hysterectomy and bilateral salpingo- oophorectomy under general anesthesia. She received a total of 500 micro g of fentanyl by iv intermittent boluses during the three-hour anesthetic. During emergence from anesthesia, while intubated, the patient presented with rigidity. No changes in ventilatory parameters were measured during the episode. The only notable predisposing factor was treatment with venlafexine, an antidepressant that modifies serotonin and norepinephrine levels. She was successfully treated with iv naloxone 20 micro g. The rest of the postoperative period was uneventful. CONCLUSION: We observed an atypical case of opioid-induced rigidity in contrast to the classical syndrome, which presents at induction with high-dose opioids. This syndrome has many clinical presentations with neurologic and ventilatory signs of varying intensity. Early recognition of the syndrome and adequate treatment is crucial. If treated adequately, opioid-induced rigidity is self-limited with few complications.

Adult↗

[The differentiated treatment of dystonia musculorum deformans in children].

Thirty-eight children with deforming muscular dystonia were treated. Eighteen of them suffered from rigid, seven from hyperkinetic, and 13 from, mixed forms of the disease. To patients with rigid condition DOPA-containing drugs (nakom) were administered in combination with cholinolytics, diphenin, midocalm. If the condition proved resistant to therapy, parlodel, remantadin, antidepressants were added. To patients with hyperkinetic forms haloperidol, clonazepam, dephenin, finlepsin were administered. In mixed forms combinations of nakom with haloperidol and clonazepam were prescribed. The best results were attained in therapy of rigid forms (77% of all rigid forms), particularly of DOPA-dependent forms and those with local involvement.

Adolescent↗

Prognostic implications of the motor symptoms of Parkinson's disease with respect to clinical, computertomographic and psychometric parameters.

Forty-four Parkinson patients (19 patients of the rigid-akinetic type, 13, of the rigid-akinetic-tremor type, and 12, of the tremor type) were included in a study in order to analyse correlations of the expression of the motor symptoms tremor, rigidity, akinesia, with other clinical parameters, computertomographic aspect of brain atrophy and psychometrically assessed cognitive parameters. Rigidity and akinesia are significantly positively correlated with the severity of motor dysability, stage of the disease, and brain atrophy, as is akinesia with a history of pharmacotoxic psychosis. Tremor is significantly negatively correlated with motor dysability, stage of the disease, and history of pharmacotoxic psychosis. Akinesia is correlated with visuomotor dysfunction (tested with Bender Gestalt Test) and rigidity with the depression score (Zung scale). The tremor type is favorable, the rigid-akinetic type unfavorable with respect to motor disability and psychosis.

Adult↗

Effects of lesions in some basal ganglia nuclei and efferent projections on tremorine-induced limb rigidity in rats.

A mechanical apparatus was used to measure in rats the effects of some unilateral basal ganglia lesions on the hind-limb rigidity produced by the cholinomimetic compound, tremorine. A globus pallidus lesion reduced the resting tone and greatly increased rigidity in the contralateral leg. Lesions in the entopeduncular, subthalamic, and accumbens nuclei had no effects on rigidity, although the entopeduncular nucleus lesion reduced resting limb tone. The role of the globus pallidus in rigidity is significant in relation to work in which striatal lesions abolished tremorine-induced rigidity. Some brain regions that receive basal ganglia efferent fibers received a lesion unilaterally. Lesions in the substantia nigra, red nucleus, or pedunculopontine nucleus had no effect on tremorine-induced rigidity. A habenular lesion significantly reduced rigidity in both hind legs. Very pronounced reductions in rigidity occurred after midbrain lesions involving the central periaqueductal grey region and the superior colliculus. The basal ganglia output pathways that may mediate tremorine rigidity are discussed, together with the possibility that the superior colliculus, which has many muscarinic receptors, may be influenced directly by tremorine.

Animals↗

Progressive encephalomyelitis with rigidity.

Two cases of encephalomyelitis are described in which the major clinical manifestation was muscular rigidity and stimulus-sensitive muscular spasms. It is suggested, from pathological evidence, that this rigidity was of spinal origin, and that this disorder is a rare but recognizable entity. Comparison is made with previously reported cases of rigidity of spinal origin, including encephalitis lethargica, and with 'subacute myoclonic spinal neuronitis' and the 'stiff man syndrome.'

Adult↗

Immunohistochemical detection of GABA in rat striatum by intraperitoneal injection of MPTP (1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine).

To study MPTP-induced muscular rigidity, we try to detect the changes of both dopamine (DA) and GABA within rat striatums by immunohistochemical means. A high dose (30 mg/kg) of MPTP i.p. injected into rats produces behavioral abnormalities (tremor and ataxia), and higher doses (greater than 60 mg/kg) develop an acutely muscular rigidity without producing a measurable histological change. GABA is induced in the striatum of 4 MPTP (30 mg/kg) i.p. treated-rats developed tremor and ataxia. But the animals recovered to an apparently normal state and are not showed GABA-immunoreactivity. Dense GABA-immunoreactivity is observed in the striatum developed muscular rigidity, when the animals are injected with 60 mg/kg MPTP i.p.. At this time, their striatums show slight decrease of DA-immunoreactivity in medium sized-spiny neurons. The results give some insight as to how DA and GABA function within the striatum with respect to the development of neuronal abnormalities. It is also suggested by our behavioral and immunohistochemical studies that effects induced by the depletion of DA within the striatum may be mediated through the inhibition of the striato-nigral GABAergic pathway, which in turn may lead to an activation of the nigrothalamic or nigro-collicular GABAergic pathway. This study indicates that the role of striatal GABAergic transmission is important in the development of muscular rigidity. The activity within the striatum can be followed with immunohistochemical technique for GABA morphologically.

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

[Rigidity abolished by intravenous dantrolene in a patient with Parkinson's disease under spinal anesthesia].

Spinal anesthesia was given to a patient with right femur fracture and Parkinson's disease (PD). Although sufficient analgesia was obtained up to L1 level after spinal anesthesia, the muscular rigidity remained. Furthermore, tremor of the upper extremities continued. After administering dantrolene sodium (DT) intravenously, these untoward features were abolished. These findings suggest that DT abolishes rigidity and tremor in PD, and is useful for the management of anesthesia for a patient with PD.

Aged↗

Differences in the reduced 18F-Dopa uptakes of the caudate and the putamen in Parkinson's disease: correlations with the three main symptoms.

It has been reported that the F-Dopa (FD) uptake in patients with idiopathic Parkinson's disease (PD) decreased significantly in the caudate and putamen when compared to controls. The FD uptake severely decreased in the putamen, while it was relatively spared in the caudate nucleus. We also previously reported that atypical parkinsonism with no or little tremor showed a homogeneously reduced FD uptake in both the caudate and the putamen. In this study we evaluated the caudate and the putaminal FD uptakes in relation to the three main symptoms in PD. The FD uptake was measured by PET with 6-L-[18F]fluorodopa in 17 patients with PD. The caudate and the putaminal FD uptake ratios to the cerebellum at 120 min were evaluated. The caudate and the putaminal FD uptake ratios in the patients with PD decreased as their clinical stages advanced. These decreases also correlated with the degree of rigidity and bradykinesia. However, such decreases did not correlate with the degree of tremor. The caudate-putamen index (CPI)(%), which was calculated by a formula based on the difference in the uptakes of the caudate and putamen divided by the caudate uptake, indicated 11.6 +/- 3.6, 16.5 +/- 5.5 and 18.3 +/- 4.1 in the group of no, mild and moderate tremor, respectively, and increased as the degree of tremor advanced. The CPI in the group of moderate tremor significantly increased from that in the group of no tremor (P < 0.04). However, the CPI did not correlate with the clinical stage, the degree of rigidity or the degree of bradykinesia. The FD/PET study therefore effectively demonstrated the severity of the clinical symptoms of rigidity and bradykinesia in patients with PD in correlation with a decrease in the FD uptakes in the caudate and the putamen, and it also demonstrated that the severity of tremor might have a different mechanism from that of such other symptoms as rigidity and bradykinesia.

Adult↗

Pharmacological characteristics of tremor, rigidity and hypokinesia induced by reserpine in rat.

The experiments characterized the dose- and time-dependence of parkinsonian motor signs induced by reserpine in rats and a standardized system of manipulation of animals, evaluation of symptoms and analysis of data was devised. The assay procedure yielded no more than 0.5, 4.5 and 0.0% false positives with the evaluation of tremor, rigidity and hypokinesia, respectively. A dose-dependent and often complete blockade of all three signs was obtained with L-DOPA plus carbidopa (10:1) as well as with other classes of pharmacological agents that are used in the treatment of Parkinson's disease, i.e. direct or indirect dopamine (DA) agonists (amantadine, pergolide, lisuride) and inhibitors of monoamine oxidase (MAO) (clorgyline, pargyline, deprenyl, tranylcypromine). The inhibitor of the uptake of DA, nomifensine, and anticholinergics, 5-hydroxytryptamine (5-HT) antagonists, histamine antagonists and tricyclic antidepressants exerted little or no effect. The effects of putative agonists and antagonists at alpha 1- and alpha 2-adrenoceptors were also examined. Yohimbine blocked tremor and rigidity, but not hypokinesia, at 0.66 and 0.28 mg/kg, respectively. It is suggested that alpha-adrenergic mechanisms and, in particular, alpha 2-adrenoceptors, may be involved in reserpine-induced tremor and rigidity. Noradrenergic and dopaminergic systems can conceivably interact to progressively generate these different motor signs.

Animals↗

Rigid spine syndrome with respiratory failure.

The pathogenesis and therapy of respiratory failure in the rigid spine syndrome are discussed in two cases who improved with respiratory assistance. In both cases, the partial pressures of oxygen and carbon dioxide were reversed in arterial blood gas analysis and %VC was less than 30%. Remission from respiratory failure has been obtained by the use of a ventilator during the night. The cause of the respiratory failure in both cases was severe restrictive respiratory dysfunction due to extreme flattening of the chest and fixation of the thorax during respiration as a result of contracture of costovertebral joints. All the previously reported cases of the rigid spine syndrome with respiratory failure died. Appropriate use of the ventilator can improve the prognosis.

Adolescent↗

Time course of postanoxic akinetic-rigid and dystonic syndromes.

Twelve previously normal patients with anoxic brain damage due to various causes are described. The mean age at anoxia was 27.4 years. Six patients, in their fourth decade or older, developed an early akinetic-rigid syndrome; the mean interval between anoxia and akinetic-rigid syndrome was 3.1 months (range, less than 1 week to 12 months). Four of these six patients also developed dystonia, usually after some months. Six other patients, all children or young adults, developed a pure dystonic syndrome; the mean interval between anoxia and dystonia was 9.7 months (range, 1 week to 36 months). Dystonia was generalized and progressed over a mean period of 21.9 months (range, 3 to 96 months). Severe bulbar involvement was common. Age determined whether an akinetic-rigid or a dystonic syndrome developed in our patients and those reported in the literature. Many, but not all, patients had lesions on brain imaging (or at autopsy). In those with visible lesions in the basal ganglia, those with dystonia tended to have damage in putamen, while those with an akinetic-rigid syndrome tended to have damage in the globus pallidus. There were exceptions.

Adolescent↗

Progressive encephalomyelitis with rigidity responsive to plasmapheresis and immunosuppression.

The stiff-man syndrome is reported in a 55-year-old woman who also had signs of severe bulbar musculature hypercontraction. These clinical features correspond to the progressive encephalomyelitis with rigidity syndrome. Diazepam was minimally beneficial, but after treatment with plasmapheresis and corticosteroids, she was still asymptomatic when seen 5 years later.

Adrenal Cortex Hormones↗

Subthalamic prelemniscal radiation stimulation for the treatment of Parkinson's disease: electrophysiological characterization of the area.

Previous reports have provided evidence of a reticulo-thalamic system, extending from the mesencephalic reticular formation (MRF) to the ventrolateral thalamus (VL), involved in the production of tremor. In humans, a funnel of fibers in the posterior subthalamus named the prelemniscal radiations (Raprl) has been described as an exquisite target to treat tremor in cases of Parkinson's disease. In the present study, a group of 14 patients suffering from Parkinson's disease, with prominent unilateral tremor and rigidity, were implanted with tetrapolar depth brain stimulation (DBS) electrodes in Raprl to perform chronic electrical stimulation (ES) for the treatment of patient symptoms. Electrodes were left externalized to corroborate their placement throughout MRI studies and also to perform the following electrophysiological battery: (a) recording of somatosensory-evoked responses (SEP) through different electrode contacts and scalp by means of a paradigm to study the attention process; (b) evoking scalp EEG responses by stimulation with low (3 cps, 6 cps) and high (60-120 cps) frequencies with stimuli delivered through different electrode contacts, and (c) studying recovery cycle (RC) potentials in the Raprl while the upper MRF was being stimulated and, conversely, the RC in MRF while Raprl was being stimulated, before and after subacute Raprl stimulation. Thereafter, the electrodes were internalized and connected to a pulse generator (IPG) to carry on chronic ES, while the effects of stimulation were determined through a quantitative evaluation that measured phasic and tonic muscular activity with EMG recordings during different motor tasks. Results indicate the following: (a) that late, but not early, SEP components were recorded in Raprl and modulated in different attentive conditions; (b) that bilateral recruiting responses and spike and wave complexes were elicited by Raprl through low-frequency stimulation, while bilateral positive DC shifts induced by high-frequency stimulation were recorded, similar to those obtained in animals from MRF, and (c) that Raprl-ES induced RC inhibition at Raprl, but Raprl ES did not change MRF-RC. Long-term Raprl-ES induced a significant decrease in tremor and rigidity. It was concluded that Raprl represents a subthalamic circuit electrophysiologically related to MRF in the genesis of tremor and rigidity and in the process of selective attention. Raprl-ES induced a significant improvement in tremor and rigidity by causing inhibition of the stimulated area.

Action Potentials↗