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

A case of Whipple's disease with central nervous system (CNS) involvement has been retrospectively diagnosed. At the age of 50 and after six years of recurrent bouts of pyrexia, anorexia and loss of weight, a man developed a subacute encephalitis. Two episodes of acute loss of vision were followed by changes in mental status, supranuclear ophthalmoplegia and oculo-facio-cervical myorhythmias which remained present until death, one year later. Combined light- and electron microscopic studies have demonstrated: No. 1 the presence in the CNS of nodules containing large amounts of Sieracki cells; No. 2 the existence of bacteria in various stages of degeneration in macrophages or in the neuropile. A review of CNS complications in Whipple's disease is made.
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PMID:Cerebral manifestations of Whipple's disease. 9 79

Paralyses of accommodation are safely diagnosticizable even in patients over 45 years of age provided there is no complete presbyopia. The identification can be a key to diagnosing the entire disease pattern. In one case a suspected Adie's syndrome with amblyopia and without any connection with internment and damage due to malnutrition was identified as ophthalmoplegia interna with partial atrophy of the n. opticus, most probably caused by malnutrition encephalopathy or encephalitis. In another patient, a bilateral isolated accommodation paralysis indicated damage of the accommodation center during concussion of the brain (possibly only a functional damage).
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PMID:[Unrecognized paralyses of accommodation and their importance in diagnostics and expertise (author's transl)]. 59 69

An internuclear ophthalmoplegia was diagnosed and recorded electro-oculographically in a patient with pontomedullary listeriosis. The etiological differential diagnosis in case of internuclear ophthalmoplegia is discussed. It is emphasized that listeria monocytogenes encephalitis should be considered because this pathology may be electively located in the pontomedullary area and because of the encreasing frequency of human listeriosis.
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PMID:[Internuclear ophthalmoplegia in infectious encephalitis: observation on a case of listeriosis]. 75 76

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 70 year-old woman presented with a progressive supranuclear ophthalmoplegia, with "apraxia" of eyelid opening, axial akinesia and dementia. CT scan showed a mild cortico-subcortical atrophy and there was a high level of immunoglobulins, with an oligoclonal pattern, without cell reaction in the CSF. The patient died two years after the onset. Post-mortem examination, limited to CNS, showed subacute encephalitis confined to the tectal, pretectal, subthalamic areas and to Ammon's horns. These changes and their location were strongly suggestive of polioencephalomyelitis with or without cancer, in which such a prevalent midbrain involvement has been exceptionally described.
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PMID:[Chronic encephalitis with mesencephalic predominance. A clinico-pathologic case]. 239 10

Two cases of combined HIV-CMV encephalitis are described. One presented with a sixth nerve palsy and a tetraparesis, the other with an internuclear ophthalmoplegia. Pathologically brain stem involvement was predominantly due to CMV.
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PMID:Combined HIV-CMV encephalitis presenting with brainstem signs. 255 24

Three patients with Fisher's syndrome had normal MRI scans of the brainstem 6 days, 7 days, and 5 weeks, respectively, after the onset of illness. This further supports a peripheral origin for the ataxia and ophthalmoplegia in this illness and distinguishes it from brainstem encephalitis.
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PMID:Three patients with Fisher's syndrome and normal MRI. 341 8

Ophthalmoplegia, ataxia, and areflexia are present in a distinctive syndrome called Fisher's syndrome. Eye-movement recordings were used to analyze ophthalmoplegia and other eye-movement abnormalities in three patients. Disorders of supranuclear ocular motor pathways (palsy of upward gaze and internuclear ophthalmoplegia) and peripheral cranial nerve palsies could account for the ophthalmoplegia. In one patient, computed tomography demonstrated a lucency in the midbrain that could be correlated with palsy of upward gaze and bilateral internuclear ophthalmoplegia. Eye-movement abnormalities characteristic of lesions of the midline structures of the cerebellum were documented in two patients. A review of reported cases of Fisher's syndrome and brain-stem encephalitis suggested that these syndromes comprise a spectrum of a reversible, parainfectious process affecting the peripheral cranial nerves and CNS.
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PMID:Eye-movement abnormalities in ophthalmoplegia, ataxia, and areflexia (Fisher's syndrome). 397 76

Fisher's syndrome was studied in six patients, one of whom was an 11-year-old girl, and the nosological position of this neuro-ophthalmological disorder was reviewed. The clinical features of the ophthalmoplegia in these cases were reported as secondary to a transient inflammatory lesion in the mid-brain; this was demonstrated in one of the cases by an enhancing area on CT scan. This mid-brain localization could explain not only the classical Fisher's triad but also many variations of the syndrome. The overlap of our cases of Fisher's syndrome with so-called brain-stem encephalitis of Bickerstaff has been emphasized. Many cases of Fisher's syndrome could therefore correspond to an inflammatory process at brain-stem level with or without nerve-root involvement. Clinical and neurophysiological findings show, however, that there is peripheral damage in some cases, so that a neutral attitude needs to be maintained on the nosological position of Fisher's syndrome.
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PMID:The nosological position of Fisher's syndrome (ophthalmoplegia, ataxia and areflexia). 618 75

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.
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PMID:[Steele-Richardson-Olszewski disease without ophthalmoplegia. 6 clinico-anatomic cases]. 661 48


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