Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0233565 (bradykinesia)
 2,352 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 72-year-old man developed supranuclear ophthalmoplegia, bradykinesia, rigidity, unsteady gait, dementia, dysphagia, retrocollis, grasp reflex and apraxia of eyelid opening. These findings were compatible with progressive supranuclear palsy (PSP). At the age of 66, he presented a peculiar phenomenon characterized by simultaneous tonic contraction of the orbicularis oris muscle (OOM) and the palatal muscles elicited by pronouncing "pa", which resulted in difficulty of voluntary opening of the mouth and the rhinopharynx. Therefore, the respiration air reciprocated between the lung and the closed mouth. The expiratory pressure puffed out the cheeks, while the lips remained tightly closed. While the respiratory movements and the pressure increased by degree, the OOM contracted more strongly in proportion to the pressure. Sixty to ninety seconds after the elicitation, the pressure overcame the contraction of the OOM and the course of the phenomenon was completed. The electromyograms showed that the OOM activity was prolonged after initial voluntary contraction, remaining thus after a tracheostomy for pneumonia at the age of 72, and that it increased in response to the pressure. Apraxia of eyelid opening, one of the other symptoms, resembled this phenomenon in terms of the aspect of difficulty of voluntary mouth opening. The "holding" phase of grasp reflex, yet another symptom, resembled it in the recruitment of the OOM activity. The phenomenon is not common in patients with PSP. However, we concluded that it may be included among the symptoms of PSP because it has similar characteristics to apraxia of eyelid opening and grasp reflex, which are not uncommon in patients with PSP.
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PMID:[A case of progressive supranuclear palsy presenting mouth opening difficulty with tonic contraction of the orbicularis oris muscle]. 130 27

Progressive supranuclear palsy (PSP) was first recognized as a distinct syndrome by Richardson, Steele and Olszewski roughly a quarter century ago. Subsequent clinical experience has corroborated and enlarged their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is a significant cause of parkinsonism and its etiology remains obscure. The case of a patient from Santa Catarina who presented definite clinical evidences of this syndrome is reported. This is the first description in this southern Brazilian State, where at least 50 more patients should exist, if we may extrapolate the prevalence rate of this condition in developed countries to this well developed area of Brazil. A review of the literature was undertaken with emphasis on recent clinical and therapeutic aspects of PSP.
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PMID:[Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of a case and review of the literature]. 130 17

Two siblings of a consanguineous marriage showed identical clinical features consisting of supranuclear vertical ophthalmoplegia, bradykinesia, rigidity, stooped posture, pseudobulbar palsy, and dementia, all beginning in the sixth decade. There was no history of encephalitis or of exposure to known chemicals. L-Dopa therapy was only partially and temporarily effective. Autopsy of one patient revealed extensive neurofibrillary degeneration with prominent involvement of the limbic system. There were no senile plaques or Lewy bodies. Under electron microscopy, there were paired helical filaments as well as 15-nm-wide straight tubules. The diffuse appearance of neurofibrillary tangles, predominant in the limbic system, and familial occurrence are unusual in progressive supranuclear palsy; these cases may, therefore, constitute a different, distinctive clinicopathologic disease.
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PMID:Progressive supranuclear palsy-like syndrome in two siblings of a consanguineous marriage. 157 23

A patient with diffuse Lewy body disease presented with supranuclear vertical and horizontal ophthalmoplegia, dementia, axial rigidity and falls, bradykinesia and pyramidal signs. This broadens the clinical presentation of this pathological diagnosis and re-emphasises the heterogeneity of patients diagnosed clinically as progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).
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PMID:Diffuse Lewy body disease presenting with a supranuclear gaze palsy. 185 Apr 51

An autopsy case of progressive supranuclear palsy (PSP) associated with central pontine myelinolysis (CPM) is reported. A 73-year-old male patient suffered from gait disturbance for about 5 years. The clinical features were characterized by gradual development of supranuclear ophthalmoplegia, tremor, bradykinesia, rigidity, neck dystonia, dementia and pseudobulbar palsy at the advanced stage of his illness. Treatment with levodopa did not improve his neurological signs and symptoms. PSP or multiple system atrophy was considered as a clinical diagnosis of the patient. He died of pneumonia, acute pancreatitis and liver dysfunction in November 1985. The main neuropathological findings were neuronal loss and gliosis with neurofibrillary tangles of globose type in the globus pallidus, subthalamic nucleus, substantia nigra and dentate nucleus, and at the base of the pons, bilateral and symmetrical demyelination was found. In addition, myelin staining revealed circumscribed pallor in the cerebral white matter. The histologic diagnosis was PSP associated with CPM. An association of PSP with CPM is rare in the elderly and possible etiologic factors of both diseases were discussed.
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PMID:[An autopsy case of progressive supranuclear palsy with central pontine myelinolysis]. 187 Feb 89

Progressive supranuclear palsy (PSP) was first recognized as a distinct morbid entity by Richardson, Steele and Olszewski a quarter century ago. Subsequent experience has confirmed and extended their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigidity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is an important cause of parkinsonism. Its etiology remains obscure. Familial concentrations have not been observed. Some cases exhibit no oculomotor dysfunction. Dementia is usually mild. Recent neuropsychological studies have defined features consistent with frontal lobe cortical dysfunction. Seizures and paroxysmal EEG activity may occur. CT and MRI scans show midbrain atrophy early and later atrophy of the pontine and midbrain tegmentum and the frontal and temporal lobes. PET scans have shown frontal hypometabolism and loss of striatal D-2 dopamine receptors. Postmortem studies have documented involvement of both dopaminergic and cholinergic systems. Treatment remains palliative and unsatisfactory.
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PMID:Progressive supranuclear palsy. 331 57

A 70 year old man recovered from a left putamenal haemorrhage in 1984 with a residual mild right hemiparesis. In 1985 he presented following the sudden onset of inability to stand. The clinical findings of supranuclear ophthalmoplegia, bradykinesia and rigidity resembled those of progressive supranuclear palsy. CT scan revealed a recent haemorrhage deep in the right hemisphere white matter in addition to a low density change in the left basal ganglia reflecting the site of previous haemorrhage. The patient's course was uncomplicated and the ophthalmoplegia resolved. Bradykinesia and rigidity persist. This case illustrates an unusual clinical presentation of bilateral intracerebral haemorrhage with supranuclear ophthalmoplegia, bradykinesia and rigidity.
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PMID:Bilateral intracerebral haemorrhage presenting with supranuclear ophthalmoplegia, bradykinesia and rigidity. 366 70

To assess the extent of overlap between clinically diagnosed patients with progressive supranuclear palsy (PSP) and corticobasal ganglionic degeneration (CBGD) we compared clinical scores for rigidity, bradykinesia, supranuclear gaze abnormalities, hemineglect and limb apraxia, postural instability, neck rigidity, and limb dystonia in 15 patients with a degenerative rigid-akinetic syndrome at presentation and at follow-up 3 to 120 months later. Only the presence of hemineglect, usually in combination with limb apraxia, was a reliable and early clinical factor for discriminating between these two conditions. These symptoms were present at admission in all 4 CBGD patients but not in any of the 11 PSP patients either at presentation or later during serial examinations. Though supranuclear ophthalmoplegia, neck rigidity, and postural instability were already observed in most CBGD patients at presentation, their scores remained low compared to those for PSP patients over the longterm. CT-scans and MRI were helpful in supporting clinically-based diagnoses made at presentation in that the vast majority of the PSP patients exhibited various degrees of midbrain atrophy and 50 percent of the CBGD patients exhibited asymmetric pericentral cortical atrophy.
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PMID:Progressive supranuclear palsy and corticobasal ganglionic degeneration: differentiation by clinical features and neuroimaging techniques. 796 99

Progressive supranuclear palsy (PSP) is a degenerative neurological disease not typically associated with a family history. Two siblings developed identical clinical features consisting of supranuclear vertical ophthalmoplegia, bradykinesia, rigidity, gait disturbance, and dementia. There was no history of encephalitis or of exposure to known chemicals. L-dopa and dopamine agonist therapy were minimally effective. Autopsy of 1 patient revealed the typical pathological findings of PSP: severe neuronal loss with neurofibrillary tangles (NFTs) in the substantia nigra, subthalamic nucleus, and locus ceruleus. Prominent neurofibrillary degeneration of the amygdaloid nucleus and hippocampus was also observed. Scattered neurofibrillary tangles were seen in the cerebral cortices. Cerebellar degeneration was characterized by a loss of neurons in the dentate nucleus associated with neurofibrillary tangles. Lewy bodies and cortical neuritic plaques were notably absent. The existence of a rare familial form of PSP is supported by these 2 siblings.
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PMID:Familial progressive supranuclear palsy. 883 6

Progressive supranuclear paly (PSP) was firstly reported by Steel in 1964. This condition was separated from Parkinsonism by both clinical symptoms and neuropathological findings. Recently, in an attempt to improve diagnostic accuracy to give appropriate informed concepts and to select correct cases for drug studies or other research purpose, diagnostic criteria for PSP have been developed. PSP begins in the presenile period and duration of illness is 5.9 years (1.2-10.3 years; Maher and Lees, 1986). Cardinal clinical symptoms of PSP are supranuclear gaze palsy, neck dystonia, parkinsonism, pseudobulbar palsy, gait imbalance with frequent falls and subcortical dementia. Supranuclear gaze palsy and bradykinesia are essential for diagnosis. MR-imaging of PSP shows dilatation of the third ventricle. Other laboratory examinations show no specific findings. Neuropathologically, marked dilation of the third ventricle and volume loss of periaqueductal area of the midbrain are noted in macroscopic view. Microscopical examination reveals neuronal loss and gliosis in the tegmentum, the tectum, periaqueductal gray, the dentate-rubro-pallido-luysial area, and the inferior olivary nucleus. Neuropathological hallmarks of PSP are neuronal loss, presence of the globose typed neurofibrillary degeneration, and glial tangles (so called tuft shaped astrocyte and coiled body). Atypical cases of PSP are reported. Such cases are reported as pure akinesia, PSP without ophthalmoplegia, dementia predominant PSP, pathologically diagnosed pallido-nigro-luysial atrophy (PNLA), pathologically diagnosed corticobasal degeneration which showed no laterality, and so on. Reported cases as pure akinesia was diagnosed as PSP or PNLA by neuropathological findings. Improvement of diagnostic accuracy in PSP is expected to ithrapeutic trials, to investigate the etiology, and to separate the other clinical entity from PSP.
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PMID:[Progressive supranuclear palsy]. 957 67


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