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)

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.
 ...
PMID:[An autopsy case of progressive supranuclear palsy with central pontine myelinolysis]. 187 Feb 89

Nine cases of progressive supranuclear palsy are reported in this paper. There are 6 males and 3 females in this series. The average age at admission was 62 years. The clinical features of those cases are: (1) onset at the presenile with gradual progression, (2) supranuclear vertical ophthalmoplegia, especially downgaze paresis, (3) disarthria, (4) gait disturbances, (5) dystonia and rigidity of the limbs, (6) clumsiness and ataxia, (7) masked face, (8) bilateral pyramidal signs and (9) mental disturbances. The CT scan in this series showed dilatation of the ventricular system, enlargement of the Sylvius fissure and cortical sulci. There was also enlargement of quadrigeminal and cisterns in the CT scan. The CT diagnosis were cerebral atrophy in 9 cases and brainstem atrophy in 8 cases in this series.
 ...
PMID:[Progressive supranuclear palsy]. 263 3

A clinicopathological report is presented of a British male, aged 59 years, who died after an illness of 10 years, manifested by progressive respiratory failure, ptosis, and dysphagia. At no time was there evidence of ophthalmoplegia, Parkinsonism or dementia. At necropsy the main finding was of neurofibrillary tangles in the neurons of the pontine and medullary reticular formation, with particularly severe involvement of the nucleus ambiguus, dorsal motor nucleus of the vagus and nucleus tractus solitarius. Morphologically, by light and electron microscopy and immunostaining, the tangles were similar to those of other neurofibrillary degenerative diseases. Although similar in some respects to progressive supranuclear palsy and amyotrophic lateral sclerosis of the Guam type, the combination of clinical and neuropathological features suggest that this is a distinct disease entity.
 ...
PMID:Progressive medullary failure associated with neurofibrillary degeneration. 273 35

We report the clinical and neuropathological findings in an autopsy case of progressive dementia, Parkinsonism, pseudobulbar palsy and supranuclear ophthalmoplegia. Since 70 years old, this hypertensive patient developed forgetfulness, unsteady gait and festination. These symptoms rapidly worsened and he was admitted in October 1983, at age 71. He had severe dementia and showed stiff face. Voluntary vertical movement of the eyes was severely disturbed, but reflex vertical movement by the doll's head eye maneuver was not affected. Muscle tone in the limbs increased slightly, and deep tendon reflexes were hyperactive in jaw and the upper extremities. Babinski sign was negative bilaterally. Sensation and coordination remained normal. Although he was not paretic or ataxic, his movements were very slow. He could not stand by himself and easily fell backward. These clinical features resembled those of progressive supranuclear palsy except for severe dementia and rapid progression. Brain CT scan revealed marked dilatation of the lateral ventricles, prominent periventicular lucency and atrophy of brainstem and cerebral cortex. Treatment with levodopa slightly improved his movement, but difficulty in swallowing worsened increasingly. He died of hypoglycemic coma and aspiration pneumonia in September 1984, about two years after the initial symptoms. General autopsy showed severe pneumonia and atrophy of the liver. The brain weighed 1,210g. Atherosclerotic change in the cerebral arteries were mild. Coronal sections of the cerebral hemispheres revealed diffuse ischemic change and multiple small infarctions in the bilateral cerebral white matter. Cortical atrophy was observed in the cerebral hemispheres. The basal ganglia, thalamus, and pons showed status lacunaris. Atrophy of midbrain and depigmentation of the substantia nigra were observed macroscopically.
 ...
PMID:[Progressive supranuclear palsy accompanied with progressive subcortical vascular encephalopathy of Binswanger type--a case report]. 275 47

We report a patient affected by progressive supranuclear palsy who also presented with bilateral internuclear ophthalmoplegia. The computed tomographic (CT) scan showed the previously described radiological picture characteristic of the syndrome and two additional unusual findings. The significance of these findings is discussed and the importance of the CT scan in the diagnosis of progressive supranuclear palsy is emphasized.
 ...
PMID:Unusual CT findings in progressive supranuclear palsy. Case report. 315 92

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.
 ...
PMID:Progressive supranuclear palsy. 331 57

The clinical picture of progressive supranuclear palsy is relatively constant, including supranuclear ophthalmoplegia, pseudobulbar palsy, axial dystonia in extension, parkinsonian signs, postural instability and dementia. A case is reported, which is unusual in having flexor dystonia of the neck and marked signs of lower motor neuron involvement.
 ...
PMID:Progressive supranuclear palsy with lower motor neuron involvement. A case report. 361 95

Progressive limb dystonia contributed to disability in 8 of 30 patients with progressive supranuclear palsy (PSP). In five, it was present when the patients were on no medication. In four, it had been present before the distinctive ophthalmoplegia permitted a correct diagnosis. The severity of limb dystonia did not correlate with the severity of either ophthalmoplegia or neck dystonia. The importance of dystonia in the pathophysiology of PSP is emphasized, with regard to both the branchial dystonia that underlies several cardinal features of the disease, and to the frequent occurrence of limb dystonia as an early sign.
 ...
PMID:Limb dystonia in progressive supranuclear palsy. 362 56

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.
 ...
PMID:Bilateral intracerebral haemorrhage presenting with supranuclear ophthalmoplegia, bradykinesia and rigidity. 366 70

Progressive supranuclear palsy (PSP) is a distinct clinicopathologic entity characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, axial dystonia in extension, and subcortical dementia. Although relatively rare, PSP is disabling, thus rehabilitation techniques and management are indicated in nearly every case. This report describes the neurologic presentation, rehabilitation management, and outcome of treatment of a patient with PSP during a 12-month period. The patient required thorough neuromuscular, neuropsychological, speech, swallowing, vision, and social service evaluations prior to the implementation of a rehabilitation program. Therapeutic rehabilitation techniques focused on limb coordination activities, tilt board balancing, ambulation activities, and activities to improve route finding and visual scanning ability. Prism lenses were introduced to compensate for deficits in vertical eye movements. Treatment improved the patient's functional status. Later, as the patient's neurologic status deteriorated, it became necessary to educate the family and caretakers in the ongoing rehabilitation management of the patient.
 ...
PMID:Rehabilitation in progressive supranuclear palsy: case report. 372 94


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