Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four pathologically documented cases of progressive supranuclear palsy are reported. Two patients exhibited severe dementia and 2 parkinsonism; none had the classic ophthalmoplegia. On retrospective analysis, clues to the diagnosis included early prominent gait disturbance, apraxia of eyelid opening in 1 patient, lack of tremor, poor response to levodopa-carbidopa, and severe rigidity with a posture of neck extension terminally in 1 patient. The clinical presentation of progressive supranuclear palsy, therefore, is not as stereotyped as previously thought, and the diagnosis can be overlooked if one adheres rigidly to the classic diagnostic criteria.
 ...
PMID:Atypical presentation of progressive supranuclear palsy. 400 54

Ten autopsy cases of Progressive Supranuclear Palsy (PSP) are reported. Age at onset ranged from 16 to 67 years and the duration of illness 3 to 24 years. The clinical features were aggressive mental retardation in 4 cases with early onset, paroxysmal dysequilibrium, ophthalmoplegia, rigidity and akinesia, pseudobulbar palsy and variable degrees of dementia. Neuropathology showed widespread neurofibrillary degeneration associated with system-bound neuronal loss and gliosis in subcortical areas, particularly affecting the subthalamic nucleus, substantia nigra, brainstem tegmentum and dentate nuclei, with no or little involvement of the cerebral cortex. The distribution of the lesions and the ultrastructure of the neurofibrillary tangles made of 15 nm straight filaments (seen in one case) in PSP are different from postencephalitic parkinsonism, Guam Parkinson-dementia complex and brainstem affection in (pre)senile dementia. Post-mortem biochemical analysis of two brains disclosed severe reduction of tyrosine hydroxylase, the key synthetic enzyme of the catecholamine pathway, not only in the nigrostriatal system as seen in Parkinson's disease, but in most areas of the brain-stem and limbic system. The implication and possible pathogenic and therapeutic significance of these biochemical findings are discussed. The etiology of PSP and its nosological position within the degenerative extrapyramidal disorders remain unknown.
 ...
PMID:Progressive supranuclear palsy: clinico-pathological and biochemical studies. 610 28

Six clinico-pathological cases (4 males and 2 females) with a Parkinson-like syndrome which lasted an average of 5 1/2 years are reported. The average age at death was 73 years. Neuropathological features were similar to those described in post encephalitic Parkinson's syndrome and in Steele-Richardson-Olszewski disease. However, neurofibrillary tangles were less numerous; lesions in tectal, periaqueductal and reticular structures were less severe. Furthermore, the lesions seemed more pronounced in the substantia nigra, the globus pallidus and the nucleus subthalamicus, giving the appearance of a systematic pallido-luyso-nigral atrophy. None of these patients had a history of encephalitis. Clinical examination did not reveal the dystonic rigidity in extension of the neck nor the characteristic ophthalmoplegia of progressive supranuclear palsy. These 6 cases are compared to 10 cases of progressive supranuclear palsy typical both on clinical and pathological grounds. They could be considered as a particular form of Steele-Richardson-Olszewski disease without ophthalmoplegia.
 ...
PMID:[Steele-Richardson-Olszewski disease without ophthalmoplegia. 6 clinico-anatomic cases]. 661 48

Progressive supranuclear palsy has been recognized as a distinct nosological entity for about three decades now. Typically, this progressive neurological disease manifests itself late in the sixth decade with a terminal course of approximately four to six years. Well over one hundred cases have been described in the literature and the heterogeneous nature of progressive supranuclear palsy includes the characteristic vertical ophthalmoplegia, frequent falling and a profound nuchal rigidity. Other features are similar in many respects to those found in Parkinson's disease. The present article reviews the literature on progressive supranuclear palsy with particular reference to its clinical manifestations including the ophthalmoplegia, characteristic sleep disturbances and unique dementia. Also addressed, are neuropathological and epidemiological findings. Finally, conclusions and recommendations for further investigation are offered especially with regard to the neuropsychological nature of this neurological disorder.
 ...
PMID:Progressive supranuclear palsy. 676 62

In this report we are describing 3 further cases of progressive supranuclear palsy, all displaying the typical clinical features (first described by Steele, Richardson and Olszewski, 1964): Ophthalmoplegia (affecting chiefly vertical gaze), pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and upper trunk and dementia. Clinical symptoms started between 49 and 51 years of age with slow progression during 2 to 4 years. One patient died 2 years after the first clinical symptoms began. The purpose of this paper is, to describe a further group of 3 cases of progressive supranuclear palsy and to point out in detail the clinical symptoms, that all correspond to supranuclear localisation of this disease and to report about some differences in the development of the disease and the fully developed disorder. The use of treatment with Adamantin and Akineton was not (very) satisfying.
 ...
PMID:[The Steel Richardson Olszewski-syndrome. A report on 3 further cases (author's transl)]. 723 12

Progressive supranuclear palsy (PSP) is characterized by supranuclear ophthalmoplegia mainly affecting vertical gaze, nuchal dystonia in extension, pseudobulbar palsy, and mental changes. The literature on PSP has been neurologically oriented whereas the psychiatric aspects have been relatively neglected. A review of the literature shows that psychiatric disturbance in PSP is common but with no characteristic pattern. Cognitive impairment, nonspecific affective and behavioral disturbances are commonly found, whereas frank psychosis or bipolar disorder are rare. Misdiagnoses with psychiatric disorders are common and a heightened awareness of the condition is necessary for early diagnosis.
 ...
PMID:Psychiatric aspects of progressive supranuclear palsy. 778 84

A 63-year-old man was admitted to the hospital with a 1,5-year history of progressive dementia, supranuclear ophthalmoplegia, pseudobulbar palsy, rigidity and dystonia in the neck and the upper trunk. Magnetic resonance imagings showed severe atrophy of the frontal lobe and the brainstem. He was diagnosed as having progressive supranuclear palsy (PSP). Rigidity, nuchal dystonia, frequent micturition, and profuse sweating ameliorated after trazodone administration. Furthermore, additional administration of L-dopa and droxidopa improved his pseudobulbar palsy, akinesia, and lack of initiative. Single photon emission tomography using IMP after medication showed increased IMP-uptake in the frontal areas and the basal ganglia compared with that before medication. This patient illustrates a substantial role of impairments in the serotonin system in the production of some PSP symptoms.
 ...
PMID:[A case of progressive supranuclear palsy showing improvement of rigidity, nuchal dystonia and autonomic failure with trazodone]. 783 44

We studied cerebral blood flow and oxygen metabolism in 6 patients with pure akinesia (PA), 8 patients with progressive supranuclear palsy (PSP), 16 patients with Parkinson's disease (PD), and 10 normal control subjects using positron emission tomography (PET). Regions of interest were studied in the cerebral cortex (the frontal, temporal, parietal, and occipital lobe), thalamus, cerebellar hemispheres, cerebellar vermis, and brainstem. In patients with PA, regional cerebral blood flow (CBF) was significantly decreased in the frontal cortex, thalamus, and brainstem compared with normal control subjects. PSP patients showed significantly decreased CBF in the entire cerebral cortex, thalamus, cerebellar hemispheres, and brainstem and regional cerebral oxygen metabolic rate (CMRO2) in the frontal cortex, thalamus, and brainstem, whereas patients with PD were revealed to be normal in both CBF and CMRO2 values. In conclusion, a part of patients with PA may be in the early stage of PSP or may be atypical presentations of PSP whose symptom is only akinesia judging from the followings. 1) PET findings demonstrated a close similarity between PA and PSP; a pattern of CBF and CMRO2 decrement especially in the frontal cortex, thalamus and brainstem. 2) Patients with PA were shown to have similar pathological changes to PSP in recent studies. 3) Several authors reported that some PSP cases could show only akinesia initially, later developing dementia, supranuclear ophthalmoplegia, pseundobulbar palsy, and nuchal rigidity during their illnesses.
 ...
PMID:[Cerebral blood flow and oxygen metabolism in patients with pure akinesia and progressive supranuclear palsy]. 795 10

Eyelid motor abnormalities found in progressive supranuclear palsy are reviewed. Electrophysiological correlates of blepharospasm, levator inhibition (blepharokolysis) and supranuclear paralysis of lid closure are presented. Disorders of eyelid motility are not uncommon in progressive supranuclear palsy (PSP). They may be found in about one third of patients with this syndrome (Jackson et al., 1983; Golbe et al., 1989). This is not surprising since ocular and eyelid movements are highly coordinated, mainly in the vertical plane (Gordon, 1951; Kennard and Smith, 1963; Kennard and Glaser, 1964), and a supranuclear ophthalmoplegia with down gaze impairment is a cardinal feature of PSP (Steele et al., 1964). The spectrum of eyelid motor disorders described in PSP includes blinking abnormalities, lid retraction, blepharospasm, levator inhibition and supranuclear palsy of eye closure.
 ...
PMID:Eyelid motor abnormalities in progressive supranuclear palsy. 796 95

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.
 ...
PMID:Progressive supranuclear palsy and corticobasal ganglionic degeneration: differentiation by clinical features and neuroimaging techniques. 796 99


<< Previous 1 2 3 4 5 6 7 Next >>