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

In the reported case unilateral external ophthalmoplegia developed in a young woman, with concomitant pain and anaesthesia in the area innervated by the 1st branch of the trigeminal nerve. After ruling out an expanding lesion of the central nervous system, aneurysm, diabetes, myasthenia, multiple sclerosis and other diseases dexamethasone was administered in a total dose of 30 mg. Complete clinical remission was achieved. In the light of observations and a survey of the peritinent literature it has been accumed that the disease was caused by a non-specific process in the small vessels in the vicinity of the cavernous sinus.
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PMID:[Tolosa-Hunt syndrome (ophthalmoplegia dolorosa)]. 52 41

Myasthenia gravis developed in two children at 2 and 3 years of age respectively. Minimal improvement followed chronic oral administration of cholinesterase inhibitors. Patient 1 had ptosis and ophthalmoplegia but no clinical or electromyographic involvement of muscles of the extremities, although a quadriceps muscle biopsy revealed lymphorrhages. Patient 2 had progressive generalized myasthenia for 3 1/2 years. Both children were given a 3-month course of prednisone followed by thymectomy. They both are in remission, 12 and 8 months after thymectomy, with only minimal residual ocular weakness, but this weakness is much more responsive to anticholinesterase drugs than before thymectomy. Long-term administration of steroids, with the attendant complication of growth retardation, is avoided.
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PMID:Effective treatment of infantile myasthenia gravis by combined prednisone and thymectomy. 55 63

Rapid eye movements, having high velocity and low amplitude, are described in 11 patients with myasthenia gravis. These movements occur with various degrees of ophthalmoplegia. To distinguish them from the somewhat similar lid-twitch phenomenon, they are called quiver movements. We believed that their presence is pathognomonic of myasthenia and results from a differential involvement of the two myoneural mechanisms that are peculiar to the extraocular muscles.
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PMID:Rapid eye movements in myasthenia gravis. I. Clinical observations. 93 89

A frequent occurrence of ophthalmoplegia and muscle fatigability in mitochondrial myopathy (MAM) often makes its differential diagnosis from myasthenia rather difficult. Neuromuscular transmission was investigated in 9 patients with MAM, presenting marked fatigability. The aim of the study was to see whether there were any other causes of muscle fatigability in addition to the metabolic factors. Classical electrostimulation as well as the SFEMG, which is very sensitive in detecting neuromuscular transmission disorders, were used. The findings were far from uniform: we found normal neuromuscular transmission in 5 cases, in 3 patients we observed slight abnormalities of neuromuscular transmission, in 1 case neuromuscular transmission disturbances seemed to be of neurogenic origin. Our results allow an assumption that the causes of muscle fatigability in MAM are of a much more complex nature than it has been anticipated. They might depend not only on the metabolic disorders within the muscle fibre itself but also on the impaired function of the peripheral nerve or of the neuromuscular junction. All the mechanisms combined may also play a role, though in individual patients the contribution of particular factors responsible may vary.
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PMID:Muscular fatigability in mitochondrial myopathies. An electrophysiological study. 131 91

Clinical description of a 33 years-old woman, with hyperthyroidism, admitted to the emergency unit with external bilateral ophthalmoplegia and left mydriasis, unreactive to light. The external ophthalmoplegia is proven to be due to myasthenia. The left mydriatic pupil demonstrates features typical for Adie's tonic pupil. The association of a tonic pupil with an auto-immune disease is infrequent. The axiom that internal ophthalmoplegia in a patient with external ophthalmoplegia excludes myasthenia gravis should be reevaluated.
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PMID:Hyperthyroidism, ophthalmoplegia and unilateral mydriasis. 134 43

Patients (n = 41) with isolated weakness of the eyelids or extraocular muscles, who had been referred for single fiber electromyography (SFEMG), were followed up after 4 to 24 months, At follow-up the patients were classified as "definite ocular myasthenia gravis" (MG), "definite other diagnosis," or "no definite diagnosis" on the basis of the completed investigations and subsequent course. The original SFEMG findings in the frontalis muscle were then reviewed. The specificity and sensitivity of SFEMG for "definite ocular MG" could be maximized by using as criteria for abnormality greater than 8/20 pairs with jitter greater than 45 microseconds, or a mean jitter of 20 pairs of greater than 50 microseconds. Patients with abnormal SFEMG according to these criteria have MG, and are likely to require treatment in the immediate future. Patients who have normal SFEMG according to these criteria (and no other demonstrated disorder) may have MG, but it is so mild that they are unlikely to require treatment. Two patients whose final diagnosis was progressive external ophthalmoplegia had normal SFEMG according to these criteria.
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PMID:Single fiber EMG in the frontalis muscle in ocular myasthenia: specificity and sensitivity. 155 90

A 44 year old diabetic woman presented with diplopia and bilateral ptosis and mild exophthalmos. The patient was clinically euthyroid, the baseline thyroid function tests were normal, but the thyroid stimulating hormone response to thyrotrophin releasing hormone was flat. Computed tomographic scan and magnetic resonance imaging of the orbits showed left medial and inferior rectus muscle thickening, more prominent on the left side, consistent with Graves' disease. The tensilon stimulation test resulted in resolution of the ptosis and partial improvement of the ophthalmoplegia. The single fibre electromyography was consistent with a defect in neuromuscular transmission. However, forced duction test was normal and anti-acetylcholine receptor antibodies were undetectable. Significant improvement of the extraocular muscle function and resolution of the right ptosis had resulted from anticholinesterase therapy. These findings and the clinical response to therapy were consistent with concomitant euthyroid Graves' ophthalmopathy and ocular myasthenia gravis. Coexistent isolated ocular myasthenia gravis and Graves' ophthalmopathy is rare and should be considered in patients with findings of ocular myasthenia and extraocular muscle dysfunction.
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PMID:Concomitant euthyroid Graves' ophthalmopathy and isolated ocular myasthenia gravis. 209 29

The patients with pure ocular myasthenia presenting no signs of neuromuscular transmission defect on classical supramaximal repetitive stimulation were studied using SFMG method. Only those patients were selected to the study in whom the diagnosis of the ocular myasthenia was confirmed by the clinical criteria arranged so as to exclude ophthalmoplegia of the other nature. The series comprises 20 patients in whom the results of the supramaximal repetitive stimulation with the recording from the proximal muscles and with the evaluation of posttetanic phenomena were normal. SFEMG examination was performed in the clinically intact EDC muscle and in 17 patients an elongated jitter, sometimes with blocking was found. Among those three patients with normal results two were in full clinical remission, so there was only one patient presenting the symptoms of ocular myasthenia in whom the neuromuscular transmission defect was absent in the EDC. The results justify an admission that the neuromuscular block in ocular myasthenia is only apparently selective; in fact it concerns all the muscles with a different severity and may be detected if properly sensitive diagnostic methods are applied.
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PMID:Pseudoselectivity of the neuromuscular block in ocular myasthenia: a SFEMG study. 215 69

An investigation was made into the occurrence of muscular atrophy and muscular pathology in a series of 170 patients with myasthenia gravis. The results can be summarized as follows: (1) Of the 148 patients with generalized myasthenia gravis, 14 showed local muscular atrophies. Of 10 biopsies from atrophic muscles, eight showed neurogenic changes, with or without lymphocytic infiltrations. One biopsy showed lymphocytic infiltrations only, and one showed type II-fibre atrophy (Table 1). No relationship was demonstrable between the presence of clilnical muscular atrophy and age, sex, duration of the disease, severity of the disease, presence of a thymoma, or drug resistant ophthalmoplegia. (2) In this group of patients 61 biopsies were examined from 46 individuals; 40 of these biopsies were taken from the quadriceps muscle. A thymoma was present in 17 patients. Examination disclosed neurogenic changes in 17 biopsies, lymphocytic infiltrates in 21, and myositis in one biopsy (Table 2). A distinct correlation was established between the presence of a thymoma and lymphocytic infiltrates, but none was demonstrable between thymoma and neurogenic changes (Table 3). (3) An enzyme-histochemical study was carried out in 35 cases, including 12 with neurogenic changes. A normal differentiation of type I- and type II-fibres was observed in eight instances, type grouping of type II-fibres in three, and type II-fibre atrophy in two cases. (4) In 21 patients and 19 controls, the smallest mean diameter was determined in the quadriceps muscle. Both type I- and type II-fibres proved to have a smaller mean diameter in the female patients than in the controls. In the male patients this could not be proven. (5) Of the eight patients who had died without disorders of ventilation, 90 muscle specimens were examined postmortem. Four of these patients had a thymoma. Lymphocytic infiltrations, found in 32 biopsy specimens, were mostly observed in the presence of a thymoma. Neurogenic changes were apparently unrelated to the presence of a thymoma (Tables 5 and 6). The post mortem examination included the spinal cord in five, and peripheral nerves in three cases. No abnormalities were found. (6) The muscular atrophy found in patients with myasthenia is not a myopathy but an affection of the lower motor neurone. Neurogenic changes were regularly found in the muscles of patients with myasthenia, even without muscular atrophy. The finding of these changes is no reason to reject the diagnosis. It is postulated that denervation occurs at the neuromuscular junction as a result of permanent absence of acetylcholine.
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PMID:Neurogenic muscle involvement in myasthenia gravis. A clinical and histopathological study. 470 58

A clinical study of 50 Southern Chinese myasthenic children observed for periods of two to 18 years (six years on average) revealed manifestations different from those of caucasian patients. Onset was early, at an average of 4.8 years. 82 per cent had ocular myasthenia. Ophthalmoplegia followed ptosis between three months and 10 years later. Additional facial and isolated limb-muscle fatigability developed in only 6 per cent within three months to 3 1/2 years. Only 12 per cent developed generalized myasthenia. Although extension from ocular to the generalized form did not occur later than 20 months after onset, a deterioration in ocular symptoms, without extension into generalized myasthenia, occurred in nine of 18 children during adolescence. Ptosis and generalized myasthenia responded better to anticholinesterase and/or prednisone. Ophthalmoplegia was difficult to treat. The natural clinical course was benign. Spontaneous remission occurred in 62 per cent of cases, but 54.8 per cent of these relapsed, all confined to ocular muscles. Although there was no familial occurrence of myasthenia gravis, an association was found between myasthenia and thyroid disorders in some patients and their relatives. The association with HLA BW46 antigen was striking. Acetylcholine receptor antibodies were absent in the majority, but mildly elevated titres were found in three of five patients whose ocular symptoms deteriorated during adolescence, without extension into generalized myasthenia.
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PMID:Myasthenia gravis in Chinese children. 651 56


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