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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report a case of right midbrain and high pons haemorrhage with complete external ophthalmoplegia, except bilateral convergence, voluntary and automatic-reflex abduction of the left eye and adduction of this eye with vestibulo-ocular reflex ("one-and-a-half syndrome"). There is voluntary-automatic dissociation of the eyelids motricity and the vertical gaze. Still, there is sensitive-motor hemiplegia at left and asterixis.
Arq Neuropsiquiatr 1986 Dec
PMID:[Combined lateral gaze palsy with unilateral internuclear ophthalmoplegia in high mesencephalic-pontine hemorrhage. Report of a case]. 360 31

A retrospective analysis of 29 patients with Graves' ophthalmopathy treated from 1973-1986 was undertaken to evaluate the use of radiation therapy (RT). Most patients presented with advanced disease; 20 had been treated with steroids and 8 had undergone prior surgery with orbital decompression. Post-RT evaluation was performed by the patients' Radiation Oncologist, Ophthalmologist, and Endocrinologist with a median follow-up period of 45 months. The overall assessment of the patients' disease was judged as improved with respect to the majority of signs and symptoms in 48%. Soft tissue changes were relieved in 78% and proptosis reduced in 52%. Ophthalmoplegia responded poorly and was improved in only 24%. After RT, 26/29 (90%) of patients have had no further steroid requirements. Thirteen patients underwent surgery for residual signs/symptoms post radiation therapy, indicating that combined modality may be necessary in many patients with advanced disease.
Int J Radiat Oncol Biol Phys 1987 Dec
PMID:Radiation therapy for Graves' ophthalmopathy: a retrospective analysis. 367 18

We report a case of intracavernous carotid artery aneurysm presenting an unusual clinical course and suggest possible pathogenetic mechanisms to explain the clinical picture. We discuss the differential diagnosis from other disorders producing unilateral ophthalmoplegia.
Ital J Neurol Sci 1986 Dec
PMID:Unruptured intracavernous carotid artery aneurysm mimicking "painful ophthalmoplegia". Case report. 380 14

A 52-year-old woman with secondary amenorrhea presented with ophthalmoplegia, subarachnoidal bleeding. Pituitary function tests showed mild hyperprolactinemia and deficiencies of other functions of adenohypophysis. X-ray films of the skull showed enlarged sella turcica, and CT scan was interpreted as demonstrating pituitary tumour. Carotid arteriography led to diagnosis of intrasellar aneurysm of the right internal carotid, without any pituitary tumour. After embolisation of the aneurysm, followed, by a temporo-sylvian anastomosis, endocrine functions did not improve. The mechanism of hyperprolactinemia is discussed, probably due to pituitary ischemia. This case provides evidence of interest of further investigations before a transsphenoidal surgery in pituitary tumours, in particular if subarachnoidal bleeding occurs.
Rev Med Interne 1985 Dec
PMID:[Intrasellar carotid aneurysm simulating prolactin adenoma]. 383 39

We report a clinical case with chronic progressive external ophthalmoplegia, retinal pigmentation and left anterior hemiblock (Kearns-Sayre Syndrome) associated with mitral valve prolapse. The mitral valve prolapse is present in familiar neuromuscular diseases. We think that mitral valve prolapse is expression of diffuse metabolic process of muscular heart in Kearns-Sayre Syndrome.
G Ital Cardiol 1985 Dec
PMID:[Kearns-Sayre syndrome and mitral valve prolapse. Description of a clinical case]. 383 1

A treatment technique employing retrobulbar supervoltage radiotherapy (XRT) in Graves' ophthalmopathy is described. Twenty-eight patients have been treated and followed between 1980-1983. Twenty-six of 28 patients (93%) showed some response, with 19/28 (68%) having a good to excellent response. Four of 28 (14%) had recurrent symptoms post-XRT. Soft tissue signs and symptoms were relieved and disease progression arrested in the majority of patients, but proptosis and ophthalmoplegia responded poorly. High dose corticosteroids have generally been the alternative form of therapy for this disease. Post-XRT, 24/28 (86%) of patients have had no further steroid requirements, and been spared the associated complications. Treatment was well tolerated, with only five patients (18%) demonstrating a transient worsening of symptoms which settled entirely within 2 weeks. No long-term complications have been encountered. Eleven patients had post-XRT CT scans which were compared to pre-treatment scans and clinical response. Nine of 11 patients (82%) had CT changes in general agreement with the observed clinical response, but the degree of involvement seen on the initial CT scan was not predictive of response nor the risk of relapse. XRT with the described technique is felt to be an effective, safe, and practical approach to the disabling and disfiguring complications of severe Graves' ophthalmopathy.
Int J Radiat Oncol Biol Phys 1985 Dec
PMID:Supervoltage radiotherapy for Graves' ophthalmopathy: CCABC technique and results. 384 Jul 86

To find out whether the central structures governing conjugate eye movements are affected in ophthalmoplegia plus (OP), we conducted an electro-oculographic study in 6 patients suffering from OP with varying degrees of extraocular involvement and with mitochondrial changes in the skeletal musculature. All the patients presented alterations of the smooth pursuit and saccadic movements, of optokinetic nystagmus as well as of the vestibular reflexes and of visuovestibular interaction, thus confirming impairment of the central oculomotor structures and particularly of those of the archicerebellum and brainstem. These data suggest a supranuclear component in the pathogenesis of ophthalmoplegia and are in accord with the findings of brainstem spongiosis in OP, which in turn seem to express a multisystem pathological state of the mitochondria.
Ital J Neurol Sci 1985 Dec
PMID:Ophthalmoplegia plus, an electro-oculographic study. 387 36

To determine the frequency of biochemical vitamin E deficiency and of the clinical signs of the vitamin E deficiency neurologic syndrome in children with prolonged neonatal cholestatic disorders, we studied 46 children (aged 1 month to 17.0 years) with chronic forms of intrahepatic neonatal cholestasis and 47 children (aged 4 months to 8.0 years) with extrahepatic biliary atresia. Based on serum vitamin E concentrations and the ratios of serum vitamin E concentration to total serum lipid concentration, 64% of the intrahepatic and 77% of the extrahepatic cholestasis groups were vitamin E deficient. Prior to age 1 year, neurologic function was normal in all children. Between ages 1 and 3 years, neurologic abnormalities were present in approximately 50% of the vitamin E-deficient children; after age 3 years, neurologic abnormalities were present in all vitamin E-deficient children. Areflexia was the first abnormality to develop between ages 1 and 4 years; truncal and limb ataxia, peripheral neuropathy, and ophthalmoplegia developed between ages 3 and 6 years. Neurologic dysfunction progressed to a disabling combination of findings by ages 8 to 10 years in the majority of vitamin E-deficient children. Neurologic function was normal in the vitamin E-sufficient children. We conclude that vitamin E status should be evaluated in infants in whom cholestasis is diagnosed, and effective therapy should be initiated to prevent or treat vitamin E deficiency at an early age.
Am J Dis Child 1985 Dec
PMID:Frequency and clinical progression of the vitamin E deficiency neurologic disorder in children with prolonged neonatal cholestasis. 406 25

Eight unrelated children with centronuclear (myotubular) myopathy are described, ranging in age at the time of diagnosis from 5 days to 12 years. Six had an intrauterine onset and 5 were severely asphyxiated at birth. All had facial involvement and 6 had ophthalmoplegia. Detailed study of the parents in 7 of the families suggested an autosomal recessive inheritance or sporadic occurrence in 2 and X-linked inheritance in 5. Classification in this very variable disorder should be based on severity and mode of presentation together with the genetic pattern, allowing three subgroups to be defined: a severe neonatal X-linked recessive type, a less severe infantile or juvenile autosomal recessive type and a milder autosomal dominant type. For genetic counselling, available relatives should be examined for mild degrees of clinical involvement and morphological abnormalities on needle muscle biopsy.
Brain 1985 Dec
PMID:Congenital centronuclear (myotubular) myopathy. A clinical, pathological and genetic study in eight children. 407 80

Electrophysiological studies showed that a patient with Aland eye disease had no misrouting of the optic pathways which is always found in all forms of albinism as a consequence of the retino-geniculate anomaly. Also the spontaneous and optokinetic nystagmus did not resemble that of the large majority of human albinos. The marked asymmetry found in this patient seems to be typical for humans with a defective development of foveal binocular vision. These findings are in agreement with clinical, nystagmographic and EM findings that Aland eye disease is distinct from the Nettleship-Falls type of X-linked ocular albinism. Furthermore, Aland eye disease is different from X-chromosomal congenital stationary night blindness with myopia by the fact that the scotopic functions are only moderately affected and there is no restriction of the peripheral photopic visual fields. In addition, there is latent nystagmus of extraocular type that appears also in female carriers. There is no ophthalmoplegia, there is a progression of the myopia and the dyschromatopsia is of secondary type.
Clin Genet 1985 Dec
PMID:Aland eye disease: no albino misrouting. 407 63


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