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PubMed1995

A SPECT study of parkinsonism in Alzheimer's disease.

The authors examined the presence of significant regional cerebral blood flow (rCBF) differences between Alzheimer's disease (AD) patients with and without extrapyramidal signs (EPS). Nine patients with probable AD and EPS (resting tremor or rigidity and bradykinesia) and 9 AD patients without EPS, comparable in age, duration of illness, and global cognitive decline, were studied with [99mTc]HMPAO SPECT. Patients with AD and EPS showed significantly lower rCBF in the superior frontal, superior temporal, and parietal regions of the left hemisphere than AD patients without EPS. Rigidity and bradykinesia independently accounted for the decreased rCBF in these areas. These findings suggest that the presence of EPS in AD may result from dysfunction in specific brain regions.

Aged↗
PubMed1989

Cerebral sarcoidosis presenting as supranuclear gaze palsy with hypokinetic rigid syndrome.

A 31-year-old man with histologically documented pulmonary sarcoidosis developed a severe hypokinetic rigid syndrome with a supranuclear gaze palsy following recurrent lymphocytic meningitis and occlusive hydrocephalus. Magnetic resonance imaging (MRI) showed multiple hyperintense foci in the CNS, not detectable by computed tomography (CT). Long-term steroid therapy led to clinical complete remission.

Adolescent↗
PubMed2000

Curariform peripheral block of muscular tone selectively increases precentral N30 somatosensory evoked potentials component. A pharmacological study carried out on healthy subjects and parkinsonian syndromes.

In the present study we investigated whether the precentral component (N30) of short somatosensory evoked potentials (SEPs) to median nerve stimulation may be modified by peripheral neuromuscular blocking agent in patients affected by rigidity. We, therefore, recorded SEPs in nine Parkinson's disease (PD) patients and in seven psychotic patients affected by neuroleptic malignant syndrome (NMS), all showing severe rigidity. Each patient group was studied before and after the placebo, and before and after an atracurium besilate bolus of 0.05 mg/kg, in a single recording session. At the time of the test the PD patients had not taken any antiparkinsonian therapy for at least 48 h. The same recordings were also taken on nine neurologically normal subjects undergoing surgical procedures. Atracurium administration produced a remarkable amplitude increase of the major precentral component (N30) of SEPs. An atracurium-induced N30 amplitude increase was observed in both PD patients (from 2.41 to 4.07 microV) and NMS psychotic patients (from 2.03 to 3.97 microV), whereas there was a minor N30 amplitude increase in healthy subjects (from 3.53 to 4. 10 microV). The N30 latency was unaffected. Amplitude and latency of the major parietal SEPs component (N20) was unchanged in the three groups studied. Our results lead to the conclusion that a neuromuscular blocking agent is capable of increasing the N30 amplitude in patients affected by severe rigidity, exclusively reducing their muscular tone without interfering with the central dopaminergic system. Thus, a "peripheral gating" of sensory input to the supplementary motor area due to rigidity may play a relevant role in producing the N30 amplitude decrease described in patients affected by degenerative or pharmacologically induced parkinsonism. The reduction of rigidity could be the mechanism by which dopamine may increase the precentral N30 amplitude in parkinsonian syndromes.

Adult↗
PubMed2004

Behavioral disorder, dementia, ataxia, and rigidity in a large family with TATA box-binding protein mutation.

BACKGROUND: Spinocerebellar ataxia type 17 is an autosomal dominant cerebellar ataxia caused by a CAG repeat expansion in the TATA box-binding protein gene. Ataxia is typically the first sign whereas behavioral symptoms occur later. OBJECTIVE: To characterize the unusual phenotypic expression of a large spinocerebellar ataxia type 17 kindred. DESIGN: Clinical, neuropathological, and molecular genetic characterization of a 4-generation family with 16 affected patients. RESULTS: Behavioral symptoms and frontal impairment dominated the early stages preceding ataxia, rigidity, and dystonic movements. Neuropathological examination showed cortical, subcortical, and cerebellar atrophy. Purkinje cell loss and gliosis, pseudohypertrophic degeneration of the inferior olive, marked neuronal loss and gliosis in the caudate nucleus, and in the medial thalamic nuclei were salient features together with neuronal intranuclear inclusions stained with anti-TATA box-binding protein and antipolyglutamine antibodies. The disease was caused by a stable 52 CAG repeat expansion of the TATA box-binding protein gene, although there was apparent variability in the age of onset. CONCLUSION: The characteristics of this family broaden the clinical picture of spinocerebellar ataxia type 17: initial presenile dementia with behavioral symptoms should be added to ataxia, rigidity, and dystonic movements, which are more commonly encountered.

Adult↗
PubMed2001

Attention and cognition in bradykinetic-rigid syndromes: an event-related potential study.

Bradykinetic-rigid syndromes are often accompanied by cognitive impairment. Because prominent motor involvement in these disorders may interfere with neuropsychological testing, we used event-related potentials (ERPs) for the assessment of cognition and attention in 41 patients with various bradykinetic-rigid syndromes of less than 5 years duration: idiopathic Parkinson's disease corticobasal degeneration, Steele-Richardson-Olszewski syndrome (SRO), and multiple system atrophy. Patients were compared with matched normals. ERP abnormalities in the auditory "oddball" paradigm were found only in corticobasal degeneration and SRO. ERP abnormalities in selective attention tasks were present in all patient groups, changes in SRO being the most prevalent. Abnormalities in corticobasal degeneration were present under "less-attention-demanding" conditions and suggested involvement of posterior parts of the brain. Multiple system atrophy and idiopathic Parkinson's disease patient groups had minimal ERP abnormalities. However, reaction times in MSA were longer in all paradigms. The results of the study support the view that bradykinetic-rigid syndromes involve some attentional deficits, but also have distinct reaction time and ERP characteristics, which may be helpful in differential diagnosis.

Acoustic Stimulation↗
PubMedDate not supplied

[A comparison of the effects of haloperidol when administered systemically and injected into the striatum of rats].

The effects of chronic intraperitoneal (2 mg/kg) and bilaterally intrastriatal (5 micrograms) haloperidol were studied in albino male rats. Intraperitoneal injections resulted in inhibition of conditioned avoidance reflexes and catalepsy. After intrastriatal microinjections, the Parkinsonism-like status appeared along with akinesia and rigidity; that effect was recorded on day 7 of the experiment, which lasted up to the first few days of haloperidol withdrawal. The causes of distinctions between the effects of systemic and local intrastriatal haloperidol injections are discussed.

Animals↗
PubMed1977

Reciprocal continuous inhibition in rigidity of Parkinsonism.

A reciprocal continuous inhibition by flexor on extensor muscles was proved during Parkinsonian rigidity. Extensor motoneurone excitability was tested by Hoffmann's reflex and flexor activity was altered by lidocaine anaesthesia of the peroneal nerve (PN). During PN anaesthetic block, a gradual increase of Hoffmann's reflex was noted. It was statistically significant at the 15th minute after lidocaine injection, and was maximum at the 30th minute. The reciprocal inhibition was considerable in 16 patients with Parkinsonism, as recorded before treatment. In normal subjects, it was small and non-significant. After one month's treatment with L-dopa at the usual therapeutic dose, this reciprocal inhibition greatly decreased in the same patients. The origin of this reciprocal continuous inhibition is discussed.

Adult↗
PubMed1980

Neuroleptic malignant syndrome.

The neuroleptic malignant syndrome is an uncommon, severe illness that consists of fever, muscular rigidity, and stupor. Various neuroleptics have been associated with the disease. A detailed neurological, medical, and neuropathological evaluation of this case was performed. Presumably, the syndrome is secondary to biochemical dysfunction of the basal ganglia and possible of the hypothalmus.

Adult↗
PubMed2000

Tryptophan 512 is sensitive to conformational changes in the rigid relay loop of smooth muscle myosin during the MgATPase cycle.

To examine the structural basis of the intrinsic fluorescence changes that occur during the MgATPase cycle of myosin, we generated three mutants of smooth muscle myosin motor domain essential light chain (MDE) containing a single conserved tryptophan residue located at Trp-441 (W441-MDE), Trp-512 (W512-MDE), or Trp-597 (W597-MDE). Although W441- and W597-MDE were insensitive to nucleotide binding, the fluorescence intensity of W512-MDE increased in the presence of MgADP-berellium fluoride (BeF(X)) (31%), MgADP-AlF(4)(-) (31%), MgATP (36%), and MgADP (30%) compared with the nucleotide-free environment (rigor), which was similar to the results of wild type-MDE. Thus, Trp-512 may be the sole ATP-sensitive tryptophan residue in myosin. In addition, acrylamide quenching indicated that Trp-512 was more protected from solvent in the presence of MgATP or MgADP-AlF(4)(-) than in the presence of MgADP-BeF(X), MgADP, or in rigor. Furthermore, the degree of energy transfer from Trp-512 to 2'(3')-O-(N-methylanthraniloyl)-labeled nucleotides was greater in the presence of MgADP-BeF(X), MgATP, or MgADP-AlF(4)(-) than MgADP. We conclude that the conformation of the rigid relay loop containing Trp-512 is altered upon MgATP hydrolysis and during the transition from weak to strong actin binding, establishing a communication pathway from the active site to the actin-binding and converter/lever arm regions of myosin during muscle contraction.

Actins↗
PubMed2005

Parkinsonism in the course of HTLV-I-associated myelopathy.

Parkinsonian syndromes may represent a complication of viral infection. Human T cell lymphotropic virus I (HTLV-I) is a cause of a chronic myelopathy in which encephalic involvement has been also found. We report on the case of a 60-year-old man with HTLV-I-associated myelopathy, complicated with bradykinesia, resting tremor, and cogwheel rigidity. These findings suggest that parkinsonian features may represent a neurological disorder associated with HTLV-I infection.

Antiparkinson Agents↗
PubMed1986

Post-operative rigidity after fentanyl administration.

A case of thoraco-abdominal rigidity leading to respiratory failure is described in the post-operative period in an elderly patient who received a moderate dose of fentanyl. This was successfully reversed by naloxone. The mechanisms possibly implicated in this accident are discussed.

Aged↗
PubMed1993

Circadian secretion pattern of melatonin in de novo parkinsonian patients: evidence for phase-shifting properties of l-dopa.

Aim of this study was the characterization of the circadian melatonin profile in de novo Parkinson patients (N = 9, age 60.0 +/- 3.2 years, mean +/- SEM) and the comparison of these profiles with those of controls and Parkinson patients treated with l-dopa/decarboxylase inhibitor (l-dopa/DCI). We collected 14 venous blood samples during a period of 24 hours and measured the serum melatonin levels by a radioimmuno assay. De novo Parkinson patients displayed the nocturnal melatonin peak (acrophase) at the same time as controls and significantly later than l-dopa/DCI treated patients (1:54 +/- 15.6 min [average clock time +/- SEM in minutes] vs. 1:45 +/- 15.6 min vs. 0:13 +/- 40.8 min). The amount of secreted melatonin did not differ among the three groups. Stage and duration of Parkinson's disease did not correlate with the amount of secreted melatonin. Patients of the tremor subgroup, however, secreted more melatonin than patients presenting only with rigidity and akinesia. The phase advance in Parkinson patients treated with l-dopa/DCI is possibly due to a central nervous dopaminergic effect elicited by l-dopa administration and not inherent to Parkinson's disease per se.

Aged↗
PubMed2003

Gaucher's disease with Parkinson's disease: clinical and pathological aspects.

The association between type 1 Gaucher disease and PD has been reported in the literature. The clinical picture is characterized by the predominance of bilateral akinetic-rigid signs and poor response to levodopa therapy. The authors describe four patients (two siblings) with type 1 Gaucher disease presenting with the following signs of typical PD: asymmetric onset of rigidity, resting tremor, bradykinesia, and a favorable response to Parkinson therapies.

Adult↗
PubMed1997

High-frequency stimulation of the globus pallidus internalis in Parkinson's disease: a study of seven cases.

The effectiveness of ventroposterolateral pallidotomy in the treatment of akinesia and rigidity is not a new discovery and agrees with recent investigations into the pathogenesis of Parkinson's disease, which highlight the role played by the unbridled activity of the subthalamic nucleus (STN) and the consequent overactivity of the globus pallidus internalis (GPi). Because high-frequency stimulation can reversibly incapacitate a nerve structure, we applied stimulation to the same target. Seven patients suffering from severe Parkinson's disease (Stages III-V on the Hoehn and Yahr scale) and, particularly, bradykinesia, rigidity, and levodopa-induced dyskinesias underwent unilateral electrode implantation in the posteroventral GPi. Follow-up evaluation using the regular Unified Parkinson's Disease Rating Scale has been conducted for 1 year in all seven patients, 2 years in five of them, and 3 years in one. In all cases high-frequency stimulation has alleviated akinesia and rigidity and has generally improved gait and speech disturbances. In some cases tremor was attenuated. In a similar manner, the authors observed a marked diminution in levodopa-induced dyskinesias. This could be an excellent primary therapy for younger patients exhibiting severe bradykinesia, rigidity, and levodopa-induced dyskinesias, which would allow therapists to keep ventroposterolateral pallidotomy in reserve as a second weapon.

Adult↗
PubMed2004

Neonatal chest wall rigidity following the use of remifentanil for cesarean delivery in a patient with autoimmune hepatitis and thrombocytopenia.

Remifentanil is a useful adjunct in general anesthesia for high-risk obstetric patients. It provides effective blunting of the rapid hemodynamic changes that may be associated with airway manipulation and surgical stimulation. There have been no previous reports of opioid-related rigidity in the neonate delivered by a parturient receiving intraoperative remifentanil. We present a case of short-lived neonatal rigidity and respiratory depression following remifentanil administration during cesarean section to a parturient with autoimmune hepatitis complicated by cirrhosis, esophageal varices and thrombocytopenia.

Adult↗
PubMed1982

Jerking stiff-man syndrome.

A female patient had permanent axial muscular rigidity similar to the "stiff-man syndrome", together with axial myoclonus triggered by stretch reflexes and by supramaximal stimulation of the supraorbital nerve. The disorder responded to treatment with diazepam and baclofen. This disorder bore a marked similarity to the so-called "jerking stiff-man syndrome".

Adult↗