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Juvenile Huntington disease in the Netherlands.

Juvenile Huntington disease (JHD) patients are distinguished from adult patients by an age at onset of less than 20 years. Investigating patients in our own database, we examined the proposition derived from studies in world literature that JHD should not be viewed as a separate clinical entity but rather as a manifestation of the rigid variant of the disease. Of 53 patients with JHD recorded in the Leiden Roster for Huntington Disease, relationships between sex, age at onset, duration of illness, maternal or paternal inheritance, motor symptom, first clinical features, and characteristics during the disease course, were obtained from the patients' files, and investigated. Although chorea is present in JHD, patients more often developed rigidity. Paternal inheritance, early dementia, epilepsy/myoclonus, and tremor during the disease course are confined for the most part to the rigid cases. A shorter duration of illness was evident in male patients with rigid JHD who inherited the disease from their father and developed their first disease feature at a younger age. The recognition of JHD as a distinct clinical entity does not appear to be warranted. Therefore, we propose, in accordance with other investigators, that rigid JHD be considered a clinical variant with special features.

Adolescent↗

Intracranial pressure increases during alfentanil-induced rigidity.

Intracranial pressure (ICP) was measured during alfentanil-induced rigidity in rats. Ten rats had arterial, central venous (CVP), and subdural cannulae inserted under halothane anesthesia. The animals were mechanically ventilated to achieve normocarbia (PCO2 = 42 +/- 1 mmHg, mean +/- SE). Following instrumentation, halothane was discontinued and alfentanil (125 mu/kg) administered iv during emergence from halothane anesthesia. In the five rats that developed somatic rigidity, ICP and CVP increased significantly above baseline (delta ICP 7.5 +/- 1.0 mmHg, delta CVP 5.9 +/- 1.3 mmHg). These variables returned to baseline when rigidity was abolished with metocurine. In five rats that did not become rigid, ICP and CVP did not change following alfentanil. These observations suggest that rigidity should be prevented when alfentanil, and, presumably, other opiates, are used in the anesthetic management of patients with ICP problems.

Alfentanil↗

Chest wall rigidity during fentanyl- and midazolam-fentanyl induction: ventilatory and haemodynamic effects.

In a double-blind randomised study, we examined if pretreatment with small doses of midazolam, given before anaesthesia induction with fentanyl, influences the occurrence of fentanyl-induced thoracic rigidity (FITR). At the same time, the effect of rigidity on the cardiovascular and respiratory system was assessed. Sixteen patients undergoing coronary artery bypass surgery were divided into two groups. The midazolam group (M) received 0.075 mg/kg midazolam i.v. and the placebo group (P) NaCl 0.9% 3 min before the start of fentanyl induction. During the induction period, FITR was assessed clinically on a 3-point scale. Haemodynamic and respiratory variables were collected before anaesthesia induction, at the end of the fentanyl infusion and 3 min after intubation. The incidence of FITR was high in both groups: 63% in Group M and 75% in Group P (n.s.); however, its severity was less in Group M. The appearance of rigidity affected the cardiovascular and the respiratory system: central venous and pulmonary capillary wedge pressures showed a sharp increase in patients with FITR accompanied by CO2 retention, due to an inability to ventilate these patients adequately. We conclude that small doses of midazolam do not prevent, but may attenuate, FITR and that the appearance of rigidity causes alterations of haemodynamic and respiratory variables during induction.

Clinical Trials as Topic↗

Gamma Knife thalamotomy and pallidotomy in patients with movement disorders: preliminary results.

The Leksell Gamma Knife is a useful and safe method to perform thalamotomy and pallidotomy in selected older patients with Parkinson's disease and related movement disorders. In this preliminary report, 2 of 3 patients with severe intention tremor were relieved of their symptoms by thalamotomy, as were 4 of 7 patients with Parkinson's tremor. Four of 8 patients had significant improvement of contralateral rigidity following pallidotomy.

Aged↗

The evaluation of anti-parkinson drugs on reserpine-induced rigidity in rats.

The effect of anti-parkinson drugs on reserpine-induced rigidity was examined using a technique which measured rigidity by hind limb palpation. Centrally acting dopaminergic and anti-cholinergic drugs were able to antagonize reserpine-induced rigidity, whereas peripherally acting drugs had no effect. Results are discussed in terms of possible modes of action. Relative potencies of the drugs studied in this model were well correlated with the dose ranges of clinically established anti-parkinson drugs. It is concluded that measurement of reserpine-induced rigidity by hind limb palpation is a rapid and sensitive technique to evaluate potential anti-parkinson drugs.

Amantadine↗

[Progressive supranuclear palsy].

Progressive supranuclear palsy, first described as clinical entity by Steele, Richardson and Olszewski, is a degenerative disorder of the central nervous system. Besides progressive supranuclear oculomotor disturbances, other characteristic signs are pseudobulbar paresis, axial rigidity, gait disturbances and subcortical dementia. Misinterpretation in the early stage as Parkinson's disease is frequently seen. A causal therapy is still missing.

Aged↗

Clinical and positron emission tomographic studies in the 'extrapyramidal syndrome' of dementia of the Alzheimer type.

Extrapyramidal signs, particularly rigidity and tremor, have been reported in a proportion of patients with dementia of the Alzheimer type. To test the hypothesis that these extrapyramidal signs are similar clinically and neurochemically to the extrapyramidal signs of Parkinson's disease, a group of 20 patients satisfying clinical criteria for probable Alzheimer's disease were studied and assessed clinically for the presence of rigidity, tremor, and bradykinesia. In those patients with extrapyramidal signs, qualitative differences were observed between the signs in these patients and in subjects with Parkinson's disease. Fifteen of 20 patients underwent fluoro-18-dopa scans, which showed no significant difference in fluoro-18-dopa uptake into the caudate and putamen between normal subjects and the rigid and nonrigid patients with Alzheimer's disease, in contrast to the marked reduction in fluoro-18-dopa uptake into the putamen that is observed in Parkinson's disease. This provides clinical and in vivo neurochemical support for the hypothesis that extranigral factors may be involved in the pathogenesis of rigidity in Alzheimer's disease.

Adult↗

Huntington's disease: survival of large striatal neurons in the rigid variant.

A morphometric method was employed to investigate the relationship of rigidity and hyperkinesia to the degree of striatal and nigral nerve cell loss in one patient with the rigid variant of Huntington's disease and four patients with hyperkinetic chorea. Both striatal neuron populations, small and medium-sized neurons and large neurons, were affected in the patients with hyperkinetic chorea, whereas the large neurons were preserved in the patient with the rigid variant. The substantia nigra was slightly involved in each patient. The findings suggest that the rigid variant of Huntington's disease may reflect the unbalanced activity of residual, mostly large, striatal neurons, inhibiting the substantia nigra.

Aged↗

Depression in classic versus akinetic-rigid Parkinson's disease.

We examined the prevalence of major depression and dysthymia in 78 patients with the classic variant of Parkinson's disease (PD) (that is, tremor plus rigidity and/or bradykinesia), and in 34 patients with the akinetic-rigid variant. Although the prevalence of dysthymia was similar in both groups (classic PD, 31%; and akinetic-rigid PD, 32%), patients with akinetic-rigid PD had a significantly higher prevalence of major depression (38% versus 15%, respectively; p < 0.01). A stepwise regression analysis demonstrated that bradykinesia was the extrapyramidal sign with the highest correlation with Hamilton depression scale scores. Our findings demonstrate a significant association between major depression and the akinetic-rigid type of PD.

Aged↗

An in vivo alpha-2 assay reversal of opioid-induced muscular rigidity and neuroleptic-induced ptosis.

A method is described to detect selective alpha-2 adrenergic agonists in vivo. Palpebral ptosis is induced in rats by the neuroleptic agent haloperidol (Hal), or by tetrabenazine (TBZ) methanesulfonate. Twenty minutes later, test compounds are injected, and ptosis is scored. In a separate test, muscular rigidity is induced by the opioid, fentanyl, and subsequently, test compounds are assessed for their ability to reverse muscular rigidity. Results indicate that only alpha-2 agonists reliably reverse neuroleptic-induced and TBZ-induced ptosis, as well as opioid-induced rigidity. An alpha-1 antagonist reversed only rigidity, whereas, alpha-2 antagonists and beta-agonists were generally ineffective in all tests. Therefore, the ability to reverse neuroleptic and TBZ-induced ptosis along with the ability to reverse opioid-induced muscular rigidity is a characteristic unique to alpha-2 agonists.

Adrenergic alpha-Agonists↗

The frequency of physical signs usually attributed to meningeal irritation in elderly patients.

Nuchal rigidity, which may be a sign of meningitis, was found in 35 per cent of geriatric patients on acute-care and rehabilitation wards and in 13 per cent of younger patients on an acute-care ward. It was significantly associated with cerebrovascular disease, confusion, abnormal plantar responses, and primitive reflexes. Elderly patients who have nuchal rigidity with no history of neurologic or cognitive disorders should be investigated for meningitis.

Adolescent↗

Mephenesin as a relaxing agent in the treatment of tetanus; clinical experience in 12 cases.

Maintenance of a patent airway in cases of tetanus is best accomplished by means of tracheotomy performed early in the course of the disease. This eliminates the possibility of upper respiratory obstruction and facilitates the elimination of irritating and obstructing secretions from the lower respiratory passages. In many cases the amount of sedation required is decidedly reduced following tracheotomy. The combination of phenobarbital and Tolserol(R) is very satisfactory in controlling the muscular rigidity and spasm in cases of moderately severe tetanus. In severe cases, response is not always satisfactory. Severe respiratory depression was observed in only one case in which phenobarbital and Tolserol(R) were used.

Mephenesin↗

[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↗

Can the frontal assessment battery (FAB) differentiate bradykinetic rigid syndromes? Relation of the FAB to formal neuropsychological testing.

The frontal assessment battery (FAB) is a bedside test of executive function. It takes less than 10 minutes to administer and a low score indicates executive dysfunction. To determine whether the FAB could detect the more severe subcortical dementia that is a feature of PSP and differentiate it from other bradykinetic rigid syndromes, we studied 17 patients with progressive supranuclear palsy (PSP); 11 with multiple system atrophy (MSA) and 12 with Parkinson's disease (PD). We compared FAB scores with the results of more detailed tests of executive and general cognitive function.FAB scores were significantly lower in PSP than in MSA or PD (p=0.02 and p<0.001) and were also found to be significantly lower in MSA than in PD (p=0.047). We divided the study group into those with an FAB score <15 and those with an FAB score>/=5, regardless of the clinical diagnosis. While 82% of the PSP group had FAB scores of <15, such scores were recorded in only 36% of the MSA and 8% of the PD groups. The lexical fluency and motor series subscores of the FAB discriminated 70% of the PSP, MSA and PD patients. The FAB scores correlated with tests of executive function, as well as with scores on the Mattis Dementia Rating Scale, the Mini Mental State Examination and other tests of general cognitive function. A stepwise regression analysis revealed that across the groups, among the variables that correlated with FAB scores, alternating semantic fluency accounted for 80% of FAB variance.These results suggest that the FAB is a valid and easily applicable bedside test to discriminate executive dysfunction in these three frequently confused bradykinetic rigid syndromes.

Aged↗

Quantifying rigidity with a new computerized elbow device.

A new computerized method is described for measuring parkinsonian rigidity with an elbow device. We present data from 127 subjects (103 controls and 24 parkinsonian patients) to show the clinical utility of the method. The instrumental quantification of rigidity correlates highly with clinical ratings of parkinsonian rigidity. The test-retest repeatability was excellent. In parkinsonian patients versus normal controls, significantly higher values of measured rigidity were observed. Moreover, activation procedure significantly increases rigidity only in parkinsonian patients. Activated rigidity in control subjects is lower than basal values. The procedure was sensitive to increased rigidity in parkinsonian patients during 3 days' drug holiday. Contrary to previous reports, levodopa therapy reduced both basal and activated rigidity in parkinsonian patients. This method is relatively simple and takes only 15 min to complete.

Adult↗

Eosinophilia-myalgia syndrome in association with L-tryptophan ingestion.

The association between the recently described eosinophilia-myalgia syndrome and L-tryptophan is now well established. We describe a patient with eosinophilia-myalgia syndrome who developed incapacitating myalgias and peripheral eosinophilia responsive only to high dose corticosteroids. When massive upper gastrointestinal hemorrhage developed while receiving corticosteroid therapy, surgery was complicated by striking abdominal wall rigidness. A discussion of this case and of eosinophilia-myalgia syndrome is presented.

Abdomen↗